VIEWS: 5 PAGES: 14 POSTED ON: 7/7/2011
Abstracts 183. Jahrestagung der Schweizerischen Neurologischen Gesellschaft SNG 183e réunion annuelle de la Société Suisse de Neurologie SSN (181e réunion) Abstracts Aarau, 5.–7. November 2009 Freie Mitteilungen FM 2 Multicentric tumor manifestations of traventricular). The patterns showed individual high grade gliomas: independent proliferation and location specific time gaps to metachronous Donnerstag, 5.11.2009, 17.30–18.30 Uhr or hallmark of extensive disease? manifestation. Calculated from the time of first tumor di- FM 1 Motor imagery in older adults, a new M. Hefti1, G. von Campe2, C. Schneider4, agnosis, the median survival was longer for pa- way to assess higher-level gait and balance H. Landolt3, U. Roelcke3 tients with metachronous MC lesions (353 days, disorders 1 Universitätsklinikum Schleswig-Holstein p <0.05) compared to patients with synchronous G. Allali1, F. Assal1, C. Annweiler2, Campus Kiel, Kiel, Germany MC lesions (110 days) or patients without mul- S. Bridenbaugh3, F. R. Herrmann4, 2 Universitätsklinik für Neurochirurgie, Graz, ticentricity (234 days). Patients with metachro- R. W. Kressig3, O. Beauchet2 Austria nous lesions showed similar survival (72 days) 3 Kantonsspital, Aarau, Switzerland as patients with synchronous MC lesions (110 1 Department of Neurology, Geneva, 4 Kantonsspital, Winterthur, Switzerland days) once they developed MC disease. Switzerland Conclusion: The topographical patterns and 2 Department of Internal Medicine Objective: Improvements in microneurosurgical temporal characteristics of MC disease suggest and Geriatrics, Angers, France techniques, radiotherapy and chemotherapy for that all manifestations share common mecha- 3 Department of Geriatrics, Basel, Switzerland high grade gliomas result in better local tumor nisms such as an active migratory process. Our 4 Department of Rehabilitation and Geriatrics, control and longer progression free survival. Multi- data therefore do not support the concept of Geneva, Switzerland centric (MC) lesions located distant from the ini- an independent MC development of multiple tial resection area, contribute to treatment failure Objectives: To measure and compare the time need- gliomas. in a growing number of patients. These MC le- ed to complete the Timed Up & Go test (TUG), sions may develop within the course of the disease the time needed to imagine performing the same (metachronous) or may already be present at the test (iTUG) in a sample of young and older adults, FM 3 Stress hormones to predict time of first tumor manifestation (synchronous). and to examine whether there was an association cerebrovascular Re-events in TIA patients To look for mechanisms and regular patterns be- between the Timed Up & Go test results (TUG and hind MC glioma manifestations and to investigate M. Katan1, F. Fluri2, N. Morgenthaler3, iTUG), age and cognitive decline. whether they are “a second primary tumor” or N. Nigro1, Ph. Schütz1, S. Neidert1, Methods: This study included 162 subjects (38 the result of continuous diffuse glioblastoma cell F. Jax4, S. Meckel4, A. Gass4, R. Bingisser5, healthy young adults, mean age 25.7 ± 2.3 years, invasion, we retrospectively analyzed the initial L. Kappos2, A. J. Steck2, S. Engelter2, 73.7% women and 124 older inpatients, mean and all follow up MR studies of our high grade B. Müller6, M. Christ-Crain1 age 85.3 ± 6.5 years, 76.6% women). The mean glioma (HGG) patients. ± SD of TUG and iTUG, age and the Mini Men- 1 Department of Endocrinology University tal State Examination (MMSE) score were used Patients and methods: MR studies of 247 con- Hospital Basel, Basel, Switzerland as outcomes. The height, the number of drugs secutive patients treated for HGG at a single in- 2 Department of Neurology, University Hospital taken per day, the use of psychoactive drugs, the stitution were analysed. MC tumor manifesta- Basel, Basel, Switzerland use of a walking aid and a history of falls in the tion was defined as more than one gadolinium 3 Research Department, Brahms AG, previous year were used as covariables. enhancing lesion within the brain on MRI with- Hennigsdorf/Berlin, Germany Results: Older adults performed the TUG out a connecting signal alteration in T2 sequenc- 4 Department of Neuroradiology, University slower than young adults (P <0.001), as the es and/or without a connecting hypointense Hospital Basel, Basel, Switzerland imagined TUG (P <0.001). Multivariate linear mass in T1 sequences. The minimal distance to 5 Department of Internal Medicine University regressions showed that age was associated with define two solitary lesions was set at >10 mm. Hospital Basel, Basel, Switzerland an increase in time of TUG (P <0.001) and with The MR studies were retrospectively analyzed 6 Department of Internal Medicine, a decrease in time of iTUG (P <0.001). There for patterns in MC tumor manifestation and pro- Kantonsspital Aarau, Aarau, Switzerland was an increase in time of TUG (P <0.001) for gression. Topographical specifications and delay in manifestation were used to explain possible Background: Transient-ischemic-attack (TIA) is a subjects who used a walking aid and who fell in pathways of development. Kaplan Meyer sur- strong predictor of subsequent stroke. It has been the previous year. vival graphs for metachronous and synchronous shown that copeptin, a hypothalamic-stress-hor- Conclusion: These results provide the first MC disease were calculated. mone, is an accurate prognostic marker in acute evidence that iTUG is clinically feasible among Results: According to specifications 40 pa- stroke. This study assessed prognostic reliability frail older adults and may quickly contribute to tients showed MC tumor manifestations in their of two distinct stress hormones, copeptin and assess higher-level changes in control of gait and MR studies on admission or during treatment cortisol, for the risk-stratification of re-events in balance in older adults. of their disease. 24 patients showed MC tumor TIA-patients. manifestation at the time of admission. 16 of Methods: We conducted a prospective study the cases developed MC manifestation within a in patients admitted to the emergency depart- follow up period of 3 to 57 months. The location ment with acute TIA. Clinical risk scoring using of all lesions could be categorized into three dis- the ABCD2-score was determined and both hor- tinct patterns (white matter, subependymal, in- mones were measured on admission. The pri- S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 305 Abstracts mary endpoint was a cerebrovascular re-event (48%), or cured (16%), with a significant pre- sclerosis was diagnosed on the basis of three clini- within 90 days. The secondary composite end ponderance of women (73% versus 50%, p = cal relapses, progression of the MRI lesion load point consisted of stroke, death, or presence of 0.047). There was no difference regarding out- and oligoclonal bands in the cerebrospinal fluid a high-risk stroke mechanism requiring specific comes between the three treatment groups in (CSF) . From 2003, he was treated with in- early intervention (i.e. 50%> stenosis in the rel- men and women. The most robust difference terferon-b1b, mitoxantrone (40 mg/m2 body sur- evant vessel or a cardioembolic source). was achieved with CBT, which was associated face) and azathioprine, before monotherapy with Results: We included 107 consecutive TIA- with a significantly higher rate of recovery (23% natalizumab was begun in 2007 in the presence patients. Re-events occurred in 10 patients vs 9%), and improvement (53% vs 42%) (p = of normal blood cell counts. At this time, he had (9%). The composite endpoint occurred in 33 0.024), and with a significant gender difference experienced 16 multiple sclerosis relapses in total, patients (31%). Copeptin levels were higher in (p = 0.01), being more effective only in women and the expanded disability status score (EDSS) patients with a re-event compared to patients (p = 0.004, men: p = 0.69). All treatments signifi- was 3.5 . During treatment with natalizumab, without re-event (p = 0.02). In patients fulfilling cantly improved pain intensity (VAS: p = 0.01, no further relapses occurred, the EDSS improved the combined endpoint (n = 33), copeptin levels p = 0.003 and p = 0.000 and McGill total: p = to 3.0, and the one-year follow-up MRI in 2008 were higher compared to patients without (p = 0.004, p = 0.122 and p = 0.014 for infiltration, showed a stable lesion load. 0.03). There was no difference in cortisol levels medication and physiotherapy, respectively) In May 2009, the patient developed fever and between these groups (p = 0.53, respectively p = without significant differences between the meningism. MRI showed a new T2-hyperintense 0.78). Copeptin revealed a higher area under the groups. Also, working ability improved overall lesion in the left occipital lobe (fig. 1a). CSF showed receiver-operating-characteristics curve (AUC) (p = 0.023) in the infiltration and physiotherapy pleocytosis of 106 cells/µl, mostly lymphocytes, to predict re-events compared to the ABCD2 groups, but not in the medication group. CBT and no JC virus (JCV) DNA, but HSV-2 DNA (1037 score (AUC of 0.73 versus 0.43; p <0.01). The had a favorable influence independent of the copies/ml). HIV-screening was negative. A relapse combination of the clinical score and the bio- primary treatment group (p = 0.003). of HSV-2 meningoencephalitis was suspected and marker showed an even higher overall prog- Conclusion: Intensive therapy in late whip- treated with valaciclovir. Three weeks later, HSV- nostic accuracy with an AUC of 0.77(p = 0.002). lash syndrome can achieve improvement in two 2 and JCV DNA were undetectable in the CSF, Conclusion: Measurement of plasma co- thirds of patients which is maintained beyond 6 and control MRI showed no progression (fig. 1b). peptin, but not cortisol levels in TIA-patients months in half of them. Overall, women showed Natalizumab treatment was continued under val- provides additional prognostic information be- a higher rate of improvement. Additional CBT acyclovir prophylaxis (500 mg/day). In July 2009, yond the ABCD2-clinical-risk-score alone. Rou- was the most effective treatment modality in- follow-up MRI revealed a progression of the oc- tine Copeptin measurement could be an addi- dependent of the concomitant therapies, with a cipital lesion, which was asymptomatic except for tional tool for risk-stratification after TIA. significant effect on outcome, including working increased light sensitivity (fig. 1c). Lumbar punc- capacity. ture revealed 7 cells/µl, no HSV-2 DNA, but of JCV in three laboratories (87–297 copies/ml), proving FM 4 Late Whiplash – a Study comparing PML. Plasmapheresis was performed with three Randomized Treatments FM 5 Relapse of herpes simplex type 2 menin- exchanges of the double plasma volume each, and goencephalitis and progressive multifocal mefloquine was initiated (250 mg at day 1, 2, 3, 8, U. Pato , G. Di Stefano , N. Fravi , M. Arnold , 1 1 1 1 leukoencephalopathy in a patient treated with and every 7th day thereafter) . M. Curatolo1, B. P. Radanov1, M. Sturzenegger1 natalizumab for multiple sclerosis The CSF cell count and the detection of HSV-2 1 Universitätsklinik Berne, Berne, Switzerland suggest that, in May, the patient had suffered a Ilijas Jelcic, Christian Baumann, relapse of HSV-2 meningoencephalitis. However, Background: To compare four different treatment Michael Weller, Michael Linnebank the newly evolved lesion might already have been strategies in patients suffering from chronic whip- Corresponding author: due to PML accompanied by a negative CSF result lash injury (symptoms persisting for more than PD Dr. Michael Linnebank, for JCV. It remains speculative whether HSV-2 6 months after injury, Grade I or II, Quebec Task University Hospital Zurich, Dept. Neurology, meningoencephalitis or other conditions like the Force classification). Subdivision of Neuroimmunology, preceding immunosuppressive therapy might Methods: Patients fulfilling the inclusion Frauenklinikstrasse 26, CH-8091 Zurich, have disposed this individual to PML, which is a criteria were randomly assigned to one of the Switzerland. E-mail: Michael.Linnebank@usz.ch. rare, but serious adverse event during natalizu- following treatment groups: 1) local anesthetic Phone: 0041 44 2551111 mab treatment [4, 5]. A history of viral meningo- infiltration, 2) physiotherapy, and 3) medica- Fax: 0041 44 2554507 encephalitis may be considered as a contraindica- tion. Group allocation was stratified according A now 41-year-old, male Caucasian patient ex- tion for natalizumab treatment. to gender, age, and education. Additionally, pa- tients of each treatment group were randomized perienced herpes simplex virus type 2 (HSV-2) 1:1 to cognitive behavioral therapy (CBT) or no meningoencephalitis in 2000. He recovered com- References CBT. Patients were assessed at baseline, after pletely, but the MRI then also revealed T2 lesions suggestive of inflammatory disease as an inciden- 1 Polman CH, Reingold SC, Edan G, et al. an 8 week treatment period, and then 3 and 6 tal finding. In 2002, relapsing-remitting multiple Diagnostic criteria for multiple sclerosis: months later, as follows: general physical and neurological examination, cervical spine MRI Figure (at baseline), evaluation of psychosocial factors Axial MRI from 2009, 05/07 (1a, T2-FLAIR), 05/22 (1b, T2-FLAIR) and 07/22 (1c, T2; axial FLAIR not done). and litigation status (at baseline), subjective outcome rating (cured, improved, unchanged, worse), standardized pain assessment question- naire (Mc Gill Pain Questionnaire), visual ana- logue scale (VAS), and self-assessment scores (well-being scale, self-rated cognitive ability, ac- tivities of daily living). Main outcome measures were: subjective outcome rating, pain intensity, and working ability. Results: Of 91 enrolled patients 73 completed the study and 62% were women. After treat- ment (irrespective of the treatment group), 47 patients (64%) were subjectively improved 1a 1b 1c S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 306 Abstracts 2005 revisions to the “McDonald Criteria”. tralateral changes were observed in the striatal to further examinations. The mammography Ann Neurol 2005;58:840–6. indirect pathway neither with enkephalin nor showed microcalcifications in both mammary 2 Kurtzke JF. Rating neurologic impairment with D2R gene expression. We also did not find glands, but biopsy showed no evidence of a ma- in multiple sclerosis: an expanded dis- any significant contralateral changes with TH, lignant tumor; PET scan of the whole body was ability status scale (EDSS). Neurology glutamatergic receptors or vGluT1. normal. In the clinical examination, livedo ra- 1983;33:1444–52. Conclusion: Our results suggest a role of the cemosa and diffuse subcutaneous oedema were 3 Brickelmaier M, Lugovskoy A, Kartikeyan striatal direct pathway on the healthy side to noticeable. Skin biopsy attested typical changes R, et al. Identification and characterization compensate the ipsilateral dopaminergic lesion attributed to lupus erythematosus but the vas- of mefloquine efficacy against JC virus and improve motor symptoms. This finding may culitis parameters, including testing for ANA and in vitro. Antimicrob Agents Chemother be important to understand the course of PD in antiphospholipid-antibodies, were negative. 2009;53:1840–9. humans. The direct pathway could play a role in Considering a non classifiable collagenosis, 4 Biogen. Tysabri Update. http://phx. compensatory mechanisms of the less affected we started a high dose steroid pulse therapy with corporate-ir.net/External.File?item=UGFyZ side slowing down the clinical progression of 500 mg methylprednisolone per day for 5 days. W50SUQ9MTEwODd8Q2hpbGRJRD0tMXxU the disease. The neurocognitive deficits declined. eXBlPTM=&t=1 Conclusion: Collagenosis should be consid- (accessed on 3th August 2009). ered in a patient with acute focal neurological 5 Yousry TA, Major EO, Ryschkewitsch deficit and additional symptoms, even if vascu- Postersession, C, et al. Evaluation of patients treated litis parameters show negative results. Freitag, 6.11.2009, 13.00–14.00 Uhr with natalizumab for progressive multifo- cal leukoencephalopathy. N Engl J Med Gruppe 1 2006;354:924–33. 02 A subtype of Creutzfeldt-Jakob disease 01 A case report: Cerebral manifestation presenting with higher order visual of non classifiable collagenosis disturbance including Balint syndrome FM 6 Parkinson’s disease: adaptive gene B. Erdèlyi1, A. Felbecker1, O. Coco1, C. Häubling1, F. Donati2, M. Braunschweig3, expression changes in the healthy striatum B. Tettenborn1 J. Haybaeck4, A. Aguzzi4, J. Rutishauser5, C. Capper-Loup1, D. Rebell1, A. Kaelin-Lang1 H. Pihan2 1 Dep. of Neurology, St. Gall, Switzerland 1 Department of neurology, University Hospital, 2 Dep. of Neurology, St. Gall, Switzerland 1 Universitätsklinik für Allgemeine Innere Medi- Inselspital, Berne, Switzerland 3 Dep. of Neurology, St. Gall, Switzerland zin, Inselspital Berne, 3010 Berne, 4 Dep. of Neurology, St. Gall, Switzerland Switzerland Introduction: A unique feature of Parkinson›s dis- 2 Spitalzentrum Biel, Abteilung Neurologie, ease (PD) is its asymmetry. In human, PD symp- A 65-year-old-woman was admitted to our hospi- 2501 Biel/Bienne, Switzerland toms only appear after a very large striatal dopa- tal with acute focal neurological symptoms with 3 Spitalzentrum Biel, Abteilung Radiologie, mine depletion. Several ipsilateral compensatory left sided hemiparesis, hemianopsia to the right, 2501 Biel/Bienne, Switzerland mechanisms could counteract dopamine denerva- dysarthria and an NIHSS of 13 points. In the am- 4 Institut für Neuropathologie, Departement tion on the same side. However it is not yet known bulance during transfer to the hospital, focal epi- Pathologie, UniversitätsSpital Zurich, whether compensatory mechanisms of the less af- leptic seizures of the left arm were observed for 8091 Zurich, Switzerland fected side could also play a role in the process. the first time. 5 Spitalzentrum Biel, Abteilung Medizin, Our hypothesis in this study is that the healthy Perfusion-CT showed decreased perfusion in 2501 Biel/Bienne, Switzerland side of a unilateral PD rat model shows adaptive the territory of the right arteria cerebri posterior behaviorally important gene expression changes and part of the dorsal territory of the right arteria Introduction: Creutzfeldt Jakob disease (CJD) is a in direct and indirect pathways. cerebri media. Native CT-scan showed no early prion disease typically presenting with progres- Methods: We analyzed the rat spontaneous signs of ischemia and bleeding, so the patient sive dementia accompanied by other neurological locomotor behaviour four weeks after a unilat- was administered intravenous thrombolysis. features such as cerebellar ataxia and myoclonus. eral 6-hydroxydopamine (6-OHDA) lesion and The focal neurological signs disappeared after In 1929, Heidenhain described a variant of the dis- then studied the contralateral striatal expression thrombolysis, the NIHSS showed 0 points. Re- ease characterized by an initially predominating of dynorphin (DYN) and D1 dopamine recep- sidual neurocognitive deficits with anosognosia, visual disorder and a rapid progression. We report tors (D1R) mRNA for the direct pathway and short term memory problems and disorientation the findings in a 64-years-old man admitted for in- enkephalin and D2 dopamine receptors (D2R) were still present. vestigation of progressive visual disturbances. Five mRNA for the indirect pathway. We also ana- MRI-DWI showed no ischemia, only diffuse months prior to admission, unspecific behaviour lyzed contralateral expression of several striatal hyperintensity of the parietal lobe, the brain- changes such as listlessness, insomnia, decline of and cortical glutamatergic markers, as well as stem and the temporal lobe with normal ADC. interests and a slightly clumsy gait had started nigral tyrosine hydroxylase (TH), to see whether The status epilepticus could be interrupted to develop and had been attributed to a possible contralateral changes in PD were due either to by administering Lorazepam. Anticonvulsive episode of depression. cortico- or nigrostriatal connections. The stria- treatment was started intravenously with Leve- Results: Clinical evaluation at admission re- tum was divided into two parts, medial (limbic) tiracetam and changed to Carbamazepine. vealed a right-homonymous hemianopsia and a and lateral (motor). Medical history showed a 6 month history color vision disturbance. The patient presented Results: Mean velocity was decreased in of depression and B-symptoms with weight loss difficulties in performing visual guided grasping 6-OHDA rats compared to other experimental and inappetence. (optic ataxia) and could not name the elements groups, although not significantly. However, Lab results showed pancytopenia, high CRP of the Poppelreuter figure (simultaneous agno- we found a positive and significant correlation and increased erythrocyte sedimentation rate. sia). Identification of single objects, spontaneous between D1R mRNA expression in the lateral Cerebrospinal fluid revealed pleocytosis with 13 grasping as well as coordination of non visual part of the contralateral healthy striatum and cells/µl and elevated lactate (3.5 mmol/l) and guided movements were intact. Tendon reflexes mean velocity both measured four weeks after protein (0.64 g/l) levels. Cytology, viral, bacte- were increased on the left side. surgery, in 6-OHDA rats only. Moreover we ob- rial and parasitic antibodies and PCR showed Diffusion MRI demonstrated a gyriform sig- served a significant increase in DYN and GAD normal results. Paraneoplastic antibodies were nal predominantly in the left-parietooccipital mRNA in the lateral part of the contralateral absent. cortex. The EEG revealed 1-2 Hz periodic theta/ parkinsonian striatum in absence of changes in Differential diagnosis of paraneoplastic syn- delta activity with recurrent sharp wave com- other direct pathway genes. In contrast no con- drome or cerebral vasculitis/collagenosis led plexes (PSWCs) strongly left-lateralized in the S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 307 Abstracts parietooccipital cortex. Cerebrospinal fluid con- too showed DWI hyperintensities and reduced little activity. The natural CD95 ligand (CD95L) is tained increased levels of CSF 14-3-3 and S-100 ADC although less marked (fig. 2). a cytokine which needs to trimerize to mediate a protein. NCSE was refractory to extensive anti-con- cell death signal. Mega-Fas-Ligand, now referred Further neuropsychological evaluation vulsive therapy. The patient died 4 days later to as APO010, is a synthetic hexameric CD95 ago- was limited by a rapid progressive decline in from multi-organ failure. nist that exhibits strong antitumor activity in vari- visual and cognitive function culminating in Conclusions: SE may damage the brain by ex- ous tumor models. cortical blindness. Later, myoclonus and ataxia citotoxicity resulting from glutamate spillover Methods: The cytolytic activity of APO010 emerged. The patient died 5 weeks after admis- and an increase of intracellular calcium. Mem- was assessed by crystal violet assays in the gli- sion in a mutistic and blind state. brane peroxidation and energy exhaustion with oma cell lines LNT-229, U87MG and LN-308. At autopsy the diagnosis of a spongiform breakdown of electrolyte and water homeostasis Induction of apoptosis in these cell lines and 9 encephalopathy was confirmed. Pathological eventually lead to acute cell death. These mech- primary glioblastoma cell cultures from freshly structural changes including accumulation of anisms may be visualised by MRI, especially by resected human tumors was quantified by stain- proteinase-K resistant material were detected means of DWI which may show laminar corti- ing with annexin V and flow cytometry. The in both occipital cortices while frontal and tem- cal cytotoxic swelling. Proposed mechanisms of activation of caspases 8 and 3 and cleavage of poral areas were relatively spared. CCD were glutamatergic propagation of hyper- PARP was assessed by immunoblot. The activa- Conclusion: The Heidenhain variant of spo- excitation through the corticopontocerebellar tion of caspase 3 was assessed additionally by radic CJD may clinically develop from unspecific tracts and/or GABAergic disinhibition of these DEVD-amc cleavage assay. LNT-229 cells were changes in comportment. Following thorough tracts after lesions of the controlling inhibitory co-treated with temozolomide and APO010, behavioural neurological assessment, the ten- interneurons. Our findings based on imaging the fraction of surviving cells was assessed fol- tative diagnosis can be further consolidated by techniques like DWI may help to visualize the lowing annexin V staining and flow cytometry. a combination of electrophysiological, neurora- pathophysiological and neuroanatomical under- Synergy of temozolomide and APO010 was cal- diological and laboratory findings. standing of CCD during SE. culated using the fractional product method. For in vivo experiments, 100.000 U87MG cells Figure 1 were implanted orthotopically in the right stria- 03 Acute crossed cerebellar diaschisis tum of nude mice. Locoregional treatment with (CCD) as a network phenomenon in status APO010 was performed on days 7 and 14 after epilepticus demonstrated by DWI tumor inoculation. Systemic treatment was per- formed on three days per week by intraperito- S. Rüegg1, N. Rastalsky2, S. Wetzel2, A. Gass2 neal injection of APO010 until the mice had to 1 Division of clinical Neurophysiology, Depart- be sacrificed. Histologic analysis included H&E ment of Neurology, University Hospital Basel, and TUNEL staining and immunohistochemical Basel, Switzerland assessment of cleaved caspase 3. 2 Division of Neuroradiology, Department Results: Here, we studied the effects of of Radiology, University Hospital Basel, APO010 in human glioma models in vitro and Basel, Switzerland in vivo. Compared with a cross-linked soluble CD95L or a CD95-agonistic antibody, APO010 Rationale: The term “diaschisis” refers to the phe- exhibited superior activity in glioma cell lines ex- nomenon of transient/permanent loss of function Figure 2 pressing CD95 and triggered caspase-dependent in a brain region distant from the area of the corti- cell death. APO010 reduced glioma cell viability cal/subcortical primary brain lesion. Crossed cer- in synergy when combined with temozolomide ebellar diaschisis (CCD) was observed in several (TMZ). The locoregional but not the systemic supratentorial brain pathologies. CCD is rare in administration of APO010 induced glioma cell adults with chronic epilepsy; only two case reports death in vivo and prolonged the survival of tu- deal with CCD after status epilepticus (SE). Dif- mor-bearing mice by induction of apoptosis on fusion weighted imaging (DWI) allows to detect tumor cells but not in normal brain. acute tissue changes revealing energy metabolism Conclusion: A further exploration of APO010 compromise secondary to ictal activity. We report as novel anti-glioma agent is warranted. for the first time CCD findings during SE by means of DWI, although CCD was clinically not detect- able because of non-convulsive SE (NCSE). 05 Autoimmune myasthenia gravis Case: A 74-year-old woman was found unre- after sternal fracture sponsive and she had a generalized-tonic-clonic seizure on emergency admission. Examination J. A. Petersen1, M. Linnebank1 revealed unresponsiveness and right, but not 04 APO010, a synthetic hexameric CD95 1 University Hospital, Zurich, Switzerland left, arm movements to painful stimuli. She had ligand, induces human glioma cell death in fixed left-upward gaze and right-sided pupillary Clinical case: We report a 54-year-old woman who vitro and in vivo dilatation. She was transferred to the intensive developed blurry vision two months after suffering care unit for monitoring and therapy G. Eisele1, P. Roth1, W. Wick2, M. Weller1 a sternal fracture due to a car accident. Thereafter, Results: Her EEG showed right-hemispheric she complained fluctuating generalized weakness. 1 University Hospital Zurich, Department of focal NCSE (fig. 1). CSF was normal. MRI re- Five years later, the diagnosis of myasthenia gravis Neurology, Zurich, Switzerland vealed widespread cortical DWI hyperintensi- made by detection of significant concentrations of 2 University Hospital Heidelberg, Department of ties and corresponding apparent diffusion coef- acetylcholine receptor antibodies in the blood, and Neuro-oncology, Heidelberg, Germany ficient (ADC) reductions in the right hemisphere the patient came to our hospital. Treatment with including thalamus, hippocampus, head of the Introduction: Death receptor targeting has emerged pyridostigmine normalized the muscular strength. caudate, and parts of the occipital areas together as one of the promising novel approaches of can- Discussion: Speculatively, the damage of with pronounced cortical swelling of affected cer therapy. The activation of one such prototypic atrophic thymic remnants due to a sternal frac- areas. This pattern of DWI abnormality and nor- death receptor, CD95 (Fas/APO-1), has remained ture might induce an autoimmune response mal MR-angiography excluded brain ischemia. controversial because CD95 agonistic molecules to thymic acetylcholine receptor molecules or Contralaterally, the left cerebellar hemisphere have exhibited either too strong toxicity or too might increase an existing subclinical antibody S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 308 Abstracts response. Similar mechanisms have been pro- 07 Comparative judgement of time: Background/aim: Small GTPases of the Rho family, posed for the occurrence of autoimmune myas- the impact of spatial neglect and caloric such as Rac1 and Cdc42, are key regulators of actin thenia gravis after cardiac surgery. The knowl- stimulation cytoskeleton that have been known to mediate edge about such putative associations may be cell death after ischemic stroke. The goal of this E. Huberle1, P. Brugger1 helpful for diagnostic procedures and may be study was to determine changes in localisation of relevant from a medicolegal perspective. 1 UniversitätsSpital Zurich, Zurich, Switzerland Rac1 and Cdc42 in the rat brain after ischemia. Methods: Transient focal ischemia was in- Temporal information has become an important duced by occluding the middle cerebral artery element for the majority of people in the 21st cen- 06 Cervical muscle area measurements in for 90 minutes (damage mainly in the striatum). tury. Recent observations indicated that cultures whiplash patients – acute, 3 and 6 months Rats were sacrificed 24h after reperfusion and with a left-to-right written language map the past follow-up brains were used for immunohistochemistry to the left side of space and the future to the right analysis. Double immunostaining of Rac1 and E. U. Ulbrich1, R. A. Aeberhard1, S. W. Wetli1, (Santiago et al., 2007). In contrast to patients with Cdc42 with TUNEL (apoptotic cells) and a neu- A. B. Busato1, C. B. Boesch1, S. A. Anderson1, neglect, healthy individuals show a perceptual ronal marker NeuN was used to identify the cel- H. Z. Zimmermann1, P. H. Heini1, J. H. Hodler1, bias towards the left side (“Pseudoneglect”; Bow- lular localization in the ischemic side. P. V. Vock1, M. S. Sturzenegger2 ers & Heilman, 1980), which also exists for the Results: Rac1 was upregulated in the peri- processing of time. In particular, the first presenta- 1 Department of Diagnostic, Interventional infarct region. Co-immunostaining studies tion of two light-flashes with identical durations is and Pediatric Radiology, Inselspital, Berne, showed that Rac1+ve cells were also TUNEL+ve typically perceived longer (Rose et al., 1995). The Switzerland and NeuN+ve. Expression of Rac1 in core area present study investigated temporal information 2 Institute for Evaluative Research in Orthope- of infarct was not observed. Cdc42 was slightly processing in patients with neglect. In addition, dic Surgery, University of Berne, Berne, reduced in the ischemic region, and only a small we manipulated the processing of time in healthy Switzerland group of Cdc42+ve cells were TUNEL+ve. In ad- observers by applying caloric stimulation, which 3 Department of Emergency, Inselspital, Berne, dition, most of the Cdc42+ve cells were not la- has been shown to produce a rightward shift in Switzerland belled with NeuN. the sense of neglect-like behaviour (Karnath et 4 Department of Orthopedics, Inselspital, Berne, Conclusion: These results suggest that Rac1 al., 2003). Switzerland and Cdc42 may play differential role in isch- A train of three identical auditory stimu- 5 Department of Neurology, Inselspital, Berne, emia-related apoptotic pathway. li, separated by intervals of different lengths, Switzerland was delivered to patients with neglect as well 6 University of Berne, Berne, Switzerland as healthy participants. Intervals were of the 09 Epic dreaming: clinical and Introduction: The role of the cervical spine muscles following durations (first/second; in msec): 1) polysomnographic findings in three patients in whiplash injury is disputed. We hypothesized 1500/1500, 2) 1400/1600, 3) 1300/1700, 4) to find cervical muscle atrophy in the long-term, 1200/1800, 5) 1600/1400, 6) 1700/1300, 7) F. Siclari1, C. Bassetti1 probably related to pain or inactivity. 1800/1200. Participants were required to per- 1 University Hospital Zurich, Zurich, Switzerland Methods: 90 symptomatic patients (48 fe- form a comparative judgement of the interval males) were recruited from our emergency durations. The healthy participants were test- Introduction: Epic dreaming (ED) is a disorder of department and examined within 48 hours, ed under caloric stimulation, for which warm unknown origin that has been reported in two and 3, and 6 months after a motor vehicle ac- (44 °C) or cold (30 °C) water was injected in the full-length articles and two abstracts. It is char- cident. MRI cross-sectional muscle area (CSA) left and right auditory canal. acterized by dreams that are experienced as ex- measurements were performed bilaterally of Unlike healthy individuals, the patients with cessive and continuous throughout the night, re- the cervical extensor muscles using axial STIR neglect showed a preference to perceive the sec- sulting in daytime fatigue. We report the clinical (Short Tau inversion Recovery) sequences on ond interval longer than the first one. In accord, and polysomnographic features of three patients level C2 (deep and total dorsal cervical extensor neglect-like behaviour could be produced under with ED. muscles), C4 (sternocleidomastoid muscles) and bithermal caloric stimulation of the right but not Methods: Three patients with ED were identi- C5 (deep and total dorsal cervical extensor mus- the left ear in the healthy participants. fied out of over 1000 consecutive patients seen cles) using Picture Archiving and Communica- The present findings indicate a spatio-tem- in our outpatient sleep clinic. Assessment in- tion System (PACS). Two blinded trained raters poral interaction for comparative judgements of cluded a detailed neurological examination and independently performed the measurements. temporal information in patients with neglect polysomnography (PSG). CSAs were correlated with clinical outcomes and healthy individuals. In accord with earlier Results: Patient 1: A 20-year-old woman re- (EuroQuol, Whiplash Disability Score, neck pain work, they strengthen the notion of a general ported a three-year history of excessive neutral- intensity [VAS], cervical spine mobility). magnitude system (Walsh, 2003; Conson et al., content dreaming. Dreams had long, complicat- Results: There was a high agreement be- 2008) and support the idea of a common neural ed plots and involved multiple dream characters tween the two raters (intra class correlation: basis for the representation of space, quantity that she could interpret from different perspec- 0.815–0.980). There were no significant changes and time in the parietal lobes (Hubbard et al., tives. Patient 2: A-24-year-old woman had of muscle CSAs at the three levels over time. 2005). been experiencing exhausting nightmares for Women consistently had smaller CSAs than two years, in which she would witness violent men. There were no consistent significant cor- scenes, such stabbing or fighting, as an observer. relations of CSA values with the clinical scores 08 Differential localization of RhoGTPases Patient 3: A 69-year-old man related a 5-year at all time points except with the BMI. after focal cerebral ischemia in rats history of excessive dreaming involving his past Conclusion: Our results do not support a activity as a cook, in which he would mentally A. Hodor1, B. Gao1, O. O. Ogunshola2, major role of cervical muscles in the genesis of rehearse the sequence of steps involved in the C. Grimm3, E. Cam1, C. L. Bassetti1 symptoms after whiplash injury. preparation of a meal. 1 Department of Neurology, University Hospital Patients 1 and 2 had a history of depression Zurich, Zurich, Switzerland and cardiac arrhythmias, while patient 3 was 2 Institute of Veterinary Physiology, known for recurrent vertigo, essential tremor University of Zurich, Zurich, Switzerland and prostate cancer. Neurological examination 3 Lab of Retinal Cell Biology, Department of was normal in patient 1, revealed attention defi- Ophthalmology, University of Zurich, Zurich, cits in patient 2 and short term memory loss in Switzerland patient 3. PSG documented sleep fragmentation S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 309 Abstracts in patients 2 and 3 (due to bruxism and PMLS Postersession, emia has been associated with various neurologi- respectively). All patients had persistent epic Freitag, 6.11.2009, 13.00–14.00 Uhr cal disorders including Alzheimer’s disease (AD). dreaming on follow up interviews after 0.5–2 One central phenomenon of AD is the formation Gruppe 2 years. of amyloid plaques. These plaques are composed Conclusion: ED appears to be a heterogenous of fibrillar amyloid-beta peptides (Abeta peptides), 11 Hemispheric lateralization of the disorder, in which sleep fragmentation might other proteins and transition metal ions like Zn(II) corticostriatal glutamatergic system play a major role. Our observations support and Cu(II). Levels of extracellular zinc and copper in the rat the existence of at least two subtypes, includ- are normally low. ing “story epic dreaming” and “non-story epic C. Capper-Loup1, D. Rebell1, A. Kaelin-Lang1 The impact of homocysteine, zinc and cop- dreaming”. per on Abeta peptide aggregation was analyzed 1 Department of neurology, University Hospital, using a microtiter plate-based Thioflavin T ag- Inselspital, Berne, Switzerland gregation assay. 10 Evolution of sleep spindles after Introduction: Inhibitory gamma-aminobutyric acid The presence of Hcy did not relevantly paramedian thalamic stroke (GABA)-ergic medium spiny projection neurons change the aggregation behaviour of Abeta pep- in the striatum receive glutamatergic afferents tides. When observed in the absence of Hcy, the R. P. Poryazova1, R. K. Khatami1, E. W. Werth1, from the cortex. N-methyl-D-aspartate (NMDA) transition metals Zn(II) and Cu(II) inhibited the P. B. Brugger1, R. H. Huber2, C. B. Bassetti1 receptors are implicated in many neurological aggregation of Abeta peptides. Co-incubation 1 University Hospital Zurich, Zurich, Switzerland disorders, including Parkinson’s disease (PD). with Hcy reduced the effects of Zn(II) and Cu(II). 2 University Children’s Hospital Zurich, Zurich, Recently, we described a specific lateralization of This effect depended on the molar ratio between Switzerland the striatal direct pathway, with a predominance metal ions and Hcy. With Hcy concentrations of the left striatum, related to non-motor func- corresponding to pathophysiologically occur- Introduction: Thalamus is a key structure in the tion. The hypothesis of the present study is that ring hyperhomocysteinemia, Zn(II) and Cu(II) generation of sleep spindles. Sleep spindles have the lateralization of striatal direct pathway is due in physiological concentrations were no longer been associated with cognition and learning. Re- to a lateralization of glutamatergic corticostriatal able to reverse aggregation behaviour. Other covery in sleep spindles after hemispheric stroke projection. ions studied (Na, K, Ca, Fe, Mg) did not change has been reported and a role in plastic recovery Methods: In healthy rats, we investigated the Abeta peptide aggregation. changes has been suggested. We aimed at inves- striatal gene expression of three different NMDA By binding Zn(II) and Cu(II), Hcy can pro- tigating the evolution of sleep spindles after para- receptors subunits (NR1, NR2A and NR2B). We mote the formation of Abeta peptide fibrils. This median thalamic stroke and at comparing them to also analyzed the vesicular glutamate transport- observation may be an explanation of the associ- healthy controls. er 1 (vGluT1), a presynaptic component of the ation between hyperhomocysteinemia and AD. Patients and methods: Eight patients, two with corticostriatal projection in the limbic cingulate bilateral paramedian thalamic stroke (PMTS) cortex (Cg), the medial agranular cortex (AGm), and six with unilateral PMTS and six age and an association premotor cortex, and the primary 13 Imaging blood-brain barrier transporters gender matched controls underwent high-den- motor cortex (M1). in low-grade glioma patients with positron sity EEG (hd-EEG) examination with 128 elec- Results: We found a significantly higher ex- emission tomography, tyrosine and choline trodes during sleep. Seven patients were studied pression of NR2A mRNA in the medial sector twice and six patients three times: in the acute U. Roelcke1, M. Bruehlmeier1, M. Hefti1, of the left striatum. No difference between the phase after stroke, 3 and 6 months later. Sleep Th. Hundsberger2, E. Nitzsche1 right and left striatum was detected either with spindles power was averaged over all electrodes NR1 or NR2B mRNA. In the cingulate cortex, 1 Cantonal Hospital, Aarau, Switzerland and analyzed as previously reported. vGluT1 mRNA expression was also higher in the 2 Cantonal Hospital, St. Gall, Switzerland Results: There were two patients, one with left hemisphere. We did not observe any side unilateral and one with bilateral PMTS with no Introduction: Amino acids are actively transported difference neither in AGm nor in M1. peak in the spindle frequency range. The patient across the blood-brain barrier (BBB). Increased Conclusion: Our study demonstrates a lateral- with bilateral PMTS was studied three times and transport in low-grade gliomas (LGG, WHO II) ization of both the striatal glutamatergic recep- in none of the tests a spindle peak was detected. gives rise to detect these tumors with positron tors and the cortical presynaptic glutamatergic In all control subjects a spindle peak was de- emission tomography (PET) and amino acid ana- system, probably related to limbic function, with tected. Repeated measures ANOVA showed no logues, e.g. F18-fluoro-ethyl-tyrosine (FET). In a a predominance on the left side. We suggest that difference in the patients group over time (p = clinical setting FET PET may be applied to define this lateralization concerns the direct pathway. 0.65, F2 = 0.303). T-tests showed no difference targets for stereotactic biopsy, and for follow-up Lateralization of the corticostriatal projection of in the spindle peak power between patients and of LGG during treatment. However, up to 20% of the direct pathway could be implicated in the control subjects either. LGG are FET-negative. Here we assessed whether pathophysiology of asymmetric diseases like PD. Conclusion: Sleep spindles, if decreased, do FET-negative tumors can be visualized with PET Further studies are required to relate this finding not recover after PMTS. Complete loss of sleep and radiolabeled choline. Choline is involved in to behaviour. spindles was observed only in two patients with intracellular signalling associated with prolifera- PMTS, one with unilateral and one with bilateral tion, and is incorporated into membrane lipids of lesions. This suggests that a unilateral thalamic dividing cells. 12 Homocysteine alters the effects of Zinc lesion is enough for the complete loss of sleep Methods: We analyzed the uptake of F18-flu- and Copper on recombinant amyloid-beta spindles, depending on its specific location. oro-choline (CHO) in six patients with FET-neg- peptide aggregation Support: The study is supported by the Zu- ative LGG (three fibrillary astrocytomas, three rich Center for Integrative Human Physiology S. Keskitalo1, M. Farkas1, M. Hanenberg2, oligodendroglial tumors, age 42–60 yr). The di- (ZIHP). M. Linnebank1 agnosis of LGG was confirmed histopathologi- cally in all patients. None of the tumors showed 1 University Hospital Zurich, Department of gadolinium enhancement on MR. Uptake of FET Neurology, Zurich, Switzerland and CHO was quantified by normalizing the tu- 2 University of Zurich, Division of Psychiatry mor tracer uptake to the mean of the cerebellar Research, Zurich, Switzerland uptake. Values >1.0 indicate increased transport Homocysteine (Hcy) is a nonessential amino acid relative to the normal brain. formed as an intermediate during the metabolism Results: The mean FET tumor uptake was of methionine to cysteine. Hyperhomocystein- 0.95 (range 0.91–1.00). By visual inspection S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 310 Abstracts none of the six tumors showed increased CHO 15 Is lymphocytosis a predictor for good uptake. In accordance the mean CHO tumor up- response to Natalizumab treatment in take was 0.89 (0.73–1.15). Multiple Sclerosis? Conclusions: In this series imaging with CHO S. L. Lienhard1, G. S. Schwegler1 was not superior to FET in detecting LGG. Our data indicate that a subset of LGG has a very 1 Neurologische Klinik, Kantonsspital Aarau, low metabolic demand for both substrates, and Aarau, Switzerland suggest that neither amino acid nor choline Introduction: Treatment with Natalizumab (Nb), a pathways are significantly upregulated inde- monoclonal antibody blocking the migration of T pendently. and B cells into the CNS, leads to an elevation of lymphocytes, monocytes, eosinophiles and baso- philes in peripheral blood in many patients. The 14 Impaired digital dexterity in Parkinson’s mechanisms underlying these findings are not disease is associated with higher-order praxis completely understood, but several hypotheses function exist: It’s thought to be a sequestration in periph- T. Vanbellingen1, M. Bellion1, P. Temperli1, eral blood by inhibition of migration into the tis- F. Baronti1, S. Bohlhalter2 sue, a reaction to latent virus activation or even a particular Nb induced activation. We analyzed 1 Klinik Bethesda, Tschugg, Switzerland if a lymphocytosis (LS) has a predictive value for 2 Inselspital, Berne, Switzerland a good response to Nb in patients with Multiple Background: A controversial concept suggests that Sclerosis (MS). impaired digital dexterity in PD may be explained Methods: This is a retrospective study. Inclu- by limb kinetic apraxia (LKA) as it is little respon- sion criteria were treatment with Nb for at least sive to dopaminergic treatment. The goal of the one year, two differential blood examinations present study was to explore the relationship of under treatment and a baseline and follow up digital dexterity (as measured by coin rotation, MRI after one year. We statistically analyzed the CR) with praxis function (assessed by the test of sensitivity, specificity, positive predictive value upper limb apraxia, TULIA) and parkinsonian and negative predictive value for good response symptoms (MDS-UPDRS). to Nb using the parameters absolute LS, relative Methods: Ten PD patients (3 women, mean LS and relative granulocyte count <50% sepa- age 63.4 ) with wearing off fluctuations were as- rate and in combination. sessed on both arms separately. Testing was done Results: We will present preliminary results. in OFF and ON state as defined by MDS-UPDRS The interpretation of our findings is hampered (average decrease of scores 66%). In the CR task by a small sample size and difficulties to establish patients were requested to rotate a 50 Rappen coin reliable criteria for insufficient response in Nb as fast as possible between their thumb, index and treated patients. ties (image 2) which were partially ameliorated middle finger. CR performance was analyzed by Conclusion: It is desirable to have reliable lab- 2½ weeks later after antiepileptic therapy with measuring the frequency during three 10 second oratory markers for insufficient response to Nb levetiracetam, phenytoin, topiramate and pre- periods. Gesture production was assessed by the treatment in MS patients. LS probably is such a gabalin. The patient remained seizure-free and TULIA, balanced for predominant finger and hand reliable marker. To clarify this issue further stud- EEG normalized. postures. Both CR and TULIA were assessed by a ies with higher sample size are needed. Follow-up MRI three months showed a fur- blinded rater based on video recordings. Within- ther resolution of abnormalities but remaining subject analyses were done by Pearson’s correla- tissue damage. tion between summed scores of measurements 16 Multifocal reversible MRI Abnormalities Etiology of seizures remained unclear. The and by paired t-tests for comparisons between OFF in prolonged Status epilepticus patient had mild preexisting cognitive impair- and ON state. ment. There was no evidence of encephalitis, J. Bontadelli1, M. Tröger1 Results: CR scores significantly correlated with meningitis, vasculitis or brain tumor. TULIA scores (r = 0.63 p = 0.05) and not with 1 Kantonsspital Aarau Neurologie, Aarau, Conclusions: Prolonged status epilepticus can MDS-UPDRS (r = –0.32 p = 0.37), irrespective of Switzerland cause pronounced damage in involved brain the motor state, although only 4 patients scored structures. Corresponding abnormalities in im- Introduction: Refractory status epilepticus is a dev- slightly below the cut off for apraxia. The correla- aging are variable. Our patient showed wide- astating disorder leading to variable damage of the tion of CR and TULIA scores was even stronger, spread abnormalities of MRI imaging and a very brain. Various patterns of MRI abnormalities have if considered in OFF only (r = 0.77 p = 0.01). CR striking reversibility of most changes. been reported but there are quite few follow up and finger gestures did not change between OFF Further systematic studies are needed for a investigations published. and ON state (p = 0.56 and p = 0.35, respectively), better understanding of pathogenetic and prog- Methods: Single patient MRI series and case while hand gestures improved in ON (p <0.001). nostic relevance of these findings report. Conclusions: The significant association of Results: A 20-year-old woman presented to CR with TULIA but not MDS-UPDRS indicates our department after two generalized epilep- that impaired dexterity in PD may be indeed 17 Olfactory function in patients with tic seizures. MRI showed left temporal T2 hy- apraxic rather than bradykinetic in nature. Fur- dopamine and/or hypocretin deficit and in perintense and T1 hypointense abnormalities thermore, digital dexterity did not improve with patients with excessive daytime sleepiness (Image 1). dopaminergic treatment supporting the notion After therapy with levetiracetam. the pa- E. G. Ghielmini1, R. P. Poryazova1, C. B. Bassetti1 of LKA underlying dexterous difficulties in PD. tient initially recovered. Ten days later the 1 University Hospital Zurich, Zurich, Switzerland patient developed fluctuating aphasia, somno- lence and vomitus and the diagnosis of non- Introduction: Hyposmia is common in patients with convulsive status epilepticus was confirmed by Parkinson’s disease (PD). Olfactory deficits were electroencephalography (EEG). Follow-up MRI recently reported also in patients with narcolepsy demonstrated a profound increase of abnormali- and linked to hypocretin deficit/neuronal degen- S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 311 Abstracts eration. The role of daytime sleepiness, dopamine In addition, Hcy incubation led to a reduc- score is 20/30) paraphasias and neologisms ap- and hypocretin on olfactory function is poorly tion of oxidized glutathione of more than 50 % peared. known. of the control value while it did not influence the Conclusion: The association between narco- Aim: to examine olfactory function in pa- levels of total glutathione. This effect was seen lepsy, PD and AD has not been reported in the tients with narcolepsy, restless legs syndrome in transfected as well as in wildtype HEK cells. literature and while probably fortuitous suggests (RLS), PD, excessive daytime sleepiness (EDS) There are many candidates for speculative a need for systematic study on the long term and healthy controls. interactions between Hcy metabolism and AD evolution of patients with hypocretin-deficient Patients and methods: We studied 14 patients pathogenesis. Our results support the hypothesis narcolepsy. with PD, 11 patients with narcolepsy, 10 patients that Hcy leads to an up-regulation of gamma- with RLS, 8 patients with EDS and 15 controls. secretase activity. However, effects of Hcy on Olfactory threshold, discrimination and identifi- the redox system may further contribute to an 20 Psychopathologische Befunde bei cation were assessed using standardized “Sniffin’ influence of Hcy on neurodegeneration. Patienten mit idiopathischem Restless-legs- sticks” test. Sleepiness was assessed using Ep- Syndrom worth sleepiness scale (ESS), Karolinska (KSS) A. Seiz1, U. Kallweit1, E. Werth1, U. Ehlert2, and Stanford sleepiness scale (SSS). 19 Parkinson’s disease and Alzheimer’s C. L. Bassetti1 Results: One-way ANOVA showed a signifi- dementia in patients with narcolepsy cant difference between groups for threshold 1 Neurologische Klinik, Universitätsspital N. T. Economou1, M. Raimondi1, J. Ghika2, (p <0.001, F4 = 7.1), discrimination (p <0.001, F4 Zürich, Zürich, Switzerland F. Bornatico-Valsangiacomo1, C. L. Bassetti1 = 13.5) and identification (p <0.001, F4 =16.2). 2 Psychologisches Institut, Universität Zürich, Post-hoc t-tests revealed that patients with PD 1 Neurology Department, Neurocenter of the Zürich, Switzerland performed significantly worse for all three ol- Southern Switzerland, Lugano, Switzerland Hintergrund: Das Restless-legs-Syndrom (RLS) ist factory parameters than all other groups. Only 2 Neurology Department, University of Lau- eine häufige neurologische Erkrankung, die oft- for threshold PD patients were not significantly sanne, Lausanne, Switzerland mals mit psychischen Störungen assoziiert ist. different from patients with EDS (p = 0.066). Background: The etiology of narcolepsy is un- Ergebnisse aus Studien belegen ein überdurch- Olfactory functions were similar in patients known. An autoimmune but also a neurodegen- schnittlich häufiges gemeinsames Auftreten von with narcolepsy, EDS, RLS when compared to erative process involving the hypocretin (orexin) RLS und Angst- und depressiven Störungen, aber healthy controls. neurons of the hypothalamus are currently dis- auch somatoformen Störungen sowie eine Auf- Conclusion: We confirm the presence of olfac- cussed. Hypocretin deficiency is known to occur merksamkeits-/Hyperaktivitätsstörung (ADHS). tory deficits in PD but not in narcolepsy, who as in Parkinson’s disease (PD) and other neurode- Ziel: Ziel der Studie war es, psychopatholo- patients with RLS and EDS- performed similarly generative disorders. gische Befunde auf Symptom- sowie Diagnose- to healthy controls. Aim: To report two patients with a long his- ebene bei RLS-Patienten aufzuzeigen. Der Fokus tory of narcolepsy with cataplexy, in which Par- liegt dabei auf den Störungsbildern der depres- kinson Disease (PD) and Alzheimer’s dementia siven, Angst-, und somatoformen Störungen 18 On the interaction of homocysteine (AD) subsequently developed. sowie der ADHS. metabolism and Alzheimer’s disease: Patients and methods: Two male patients un- Methoden: 49 Patienten mit der Diagnose ei- in vitro and in vivo experiments derwent an assessment which comprised a clini- nes idiopathischen RLS wurden prospektiv mit- M. Farkas1, S. Keskitalo1, L. Kulic1, M. Weller1, cal and neurological evaluation with detailed tels des diagnostischen Expertensystems DIA-X M. Linnebank1 sleep history; both patients received the diag- bezüglich der Häufigkeit psychischer Störungen nosis of narcolepsy on clinical and laboratory in den letzten 12 Monaten befragt sowie anhand 1 University Hospital Zurich, Department of basis (CSF Hypocretin-1 levels, PSG/MSLT in- diverser Fragebogen zur psychischen und physi- Neurology, Zurich, Switzerland vestigations). schen Befindlichkeit untersucht. Sporadic Alzheimer’s disease (AD) is the most Results: The first patient, a 67-year-old male, Ergebnisse: Die Resultate des diagnostischen common cause of dementia. Increased homocys- suffers from narcolepsy with cataplexy since the Expertensystems DIA-X ergaben auf Störungs- teine (Hcy) plasma and CSF levels are a risk factor age of 17. He has a typical clinical narcoleptic gruppenebene bei insgesamt 18,3% der un- for AD. Although the association between hyper- tetrade and typical neurophysiological findings. tersuchten RLS-Patienten eine somatoforme homocysteinemia and AD is well established, the The HLA typing is positive (HLA DQB1*0602) Störung, bei 16,3% eine depressive Störung, underlying pathophysiology is unexplained. In the and CSF hypocretin-1 is undetectable. At the bei 12,2% eine Angststörung, bei 4,1% eine particular, it is not known whether Hcy itself or age of 60 he developed resting tremor, micro- posttraumatische Belastungsstörung sowie bei other components of the homocysteine metabo- graphia and right bradykinesia; the brain MRI 2,0% eine Essstörung. Es zeigten sich keine sig- lism, e.g., S-adenosylmethionine (SAM), folate was normal. The motor symptoms responded nifikanten soziodemographischen Unterschiede or vitamin B12, play a role in the development to L-DOPA and the diagnosis of PD was made. zwischen Probanden mit psychischen Störun- of AD. The objective of this study was to define Narcoleptic symptoms, especially cataplexia, gen und Probanden ohne psychische Störungen. the major player of Hcy metabolism in respect to also improved with L-DOPA treatment. RLS-Patienten mit auffälligen depressi- relevance for AD. The second patient, a 69-year-old male, suf- ven Symptomen (gemäss HADS-D/D) zeigten We manipulated different parts of Hcy me- fers from narcolepsy with cataplexy since ado- ein signifikant schwereres RLS, eine höhere tabolism in human embryonic kidney (HEK) cell lescence. The diagnosis was made only at the Erschöpfung sowie eine grössere psychische lines by adding Hcy or SAM to the cell culture age of 62. He has a typical clinical narcoleptic Beeinträchtigung der Lebensqualität. Dieser media and by dietary changes in transgenic mice tetrade and typical neurophysiological findings. Gruppenunterschied konnte bei Probanden mit over-expressing the amyloid-precursor protein The HLA typing is positive (HLA DQB1*0602) Angstsymptomen (gemäss HADS-D/A) nicht (APP) and analyzed AD-specific parameters e.g., the and CSF hypocretin-1 is undetectable. At festgestellt werden. APP-processing, gene expression patterns and the age of 65 he presented early signs of demen- Schlussfolgerung: Psychische Störungen, ins- markers of oxidative stress. Our cell culture re- tia, characterized by spatial disorientation, short besondere Depression, Angst- und somatoforme sults suggested that Hcy influences processing term memory deficit, reduced attention and re- Störungen sind eine häufige Ko-Morbidität bei and expression of APP in HEK cells, i.e., in a duced verbal fluency. Brain MRI evidenced dif- RLS und sollten vermehrt bei der Diagnostik dose-dependent manner, Hcy concentrations of fused cortical atrophy without focal lesions. The und Therapie der Erkrankung berücksichtigt 1, 2.5, 5 and 10 mM, 4 h incubation, lead to diagnosis of Alzheimer disease, probably familiar werden. a significant decrease of both C-terminal APP type (his mother had the same clinical picture) fragments. was made. As AD progressed (his actual MMSE S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 312 Abstracts Postersession, Gasper, 2002). In this study, we investigated immunoglobuline (IVIG) therapy is often only of Freitag, 6.11.2009, 13.00–14.00 Uhr whether inducing positive or negative emotions short duration. while bisecting lines may have opposite effects on Methods: Case study of a single female patient Gruppe 2 the direction of participants’ displacements. with myasthenia gravis case. Methods: Participants (40 healthy right- Results: We report a female patient, who pre- 21 Recurrent tumefactive demyelination handed adults) were required to mark the mid- sented for the first time with bulbar symptoms without evidence for multiple sclerosis or point of each of 24 horizontal lines (line length at our hospital at the age of 43 years (2005). The brain tumor 26 cm) with a sharp pencil held in the dominant diagnosis of an anti-MuSK positive seronega- A. Häne1, M. Bargetzi1, E. Hewer1, M. Bruehl- right hand (one line per sheet). While executing tive Myasthenia gravis was made and therapy meier1, A. Khamis1, U. Roelcke1 this task, subjects were asked to try to remem- with low dose steroids and azathioprin initi- ber and contemplate once a positive (during ated. Under IVIG therapy the patient improved 1 Cantonal Hospital, Aarau, Switzerland 12 lines) and once a negative autobiographical to a remission. During the following years the Introduction: The differential diagnosis of single event (during 12 lines; order of emotion coun- disease generalised with recurrent neurological space occupying lesions comprises multiple scle- terbalanced). The number of leftward vs right- impairments, severe loss of weight and dyspnea, rosis (MS) in younger patients, and primary or ward deviations were recorded in each run, and despite accompanying therapy with inhibitors secondary brain tumor at any age. We present an average deviation from the midline over all of cholinesterase, azathioprin and low dose ste- a 70-year-old man with recurrent tumefactive 24 lines was obtained for each participant, again roids and repeated cycles of IVIG. demyelination (TD), which is considered to re- for each run. The clinical course was complicated by mul- sult from demyelination. Some authors suggest Results: ANOVA of the number of leftward vs tiple embolic events that led to the diagnosis of that TD may evolve into MS or primary CNS lym- rightward displacements with induced emotion an antiphospholipid antibody syndrome. phoma. (positive or negative) as the repeated measure So therapy was switched to Rituximab. A Methods: This patient developed four single revealed significantly more leftward displace- first cycle was given in 10/2008 under which re- tumor-like, gadolinium enhancing lesions at dif- ment under negative emotion, and no system- mission could be achieved. A consolidating cycle ferent sites of the brain over three years. There atic directional bias under positive emotion (see was given in 5/2009 and the patient is still in was no preceding viral infection or vaccination. figure). remission. The accompanying immunosuppres- From the initial appearance on MRI CNS lym- Conclusion: Over both emotion conditions, sive therapy as well as the therapy with cholin- phoma was suggested. However, biopsy at first there were more left-sided than right-sided dis- esterase inhibitor could be tapered. presentation revealed perivascular lymphocytic placements (“pseudoneglect”). This result con- Conclusion: Our patient responds well to the cuffs and macrophages. Over two years three firms previous findings of a leftward bias in nor- treatment with Rituximab. Therefore a therapy CSF analyses showed mild lymphocytic pleocy- mal participants when bisecting horizontal lines with Rituximab should be considered in pa- tosis, elevated protein, but no oligoclonal bands. (Jewell, 2000). Pseudoneglect was significant tients, who appear to be refractory to a therapy FDG PET was negative at two, and positive at only for the run during which subjects contem- under other immunosuppressive therapy. one manifestation. 14 months after onset of TD plated the negative autobiographical event. This the patient also developed myelodysplastic syn- observation is in line with “valence theories” of drome (MDS) with isolated genomic deletion of emotional hemispheric processing, which pos- 24 Restless-legs-Syndrom (RLS) oder «RLS- 5q-. In addition renal cancer was diagnosed 11 tulate a right hemisphere mediation of negative like»? – Psychobiologische Untersuchungen months later. emotions. The reason(s) why positive emotions bei Patienten mit idiopathischem RLS Results: All cerebral lesions responded within could not overcome pseudoneglect remain to be S. Trulec1, U. Kallweit1, E. Werth1, U. Ehlert2, 2 to 3 months to dexamethasone. The patient elucidated in future research. C. L. Bassetti1 died after 33 months due to metastatic renal cancer. The final histopathological diagnosis Figure 1 Neurologische Klinik, Universitätsspital obtained from autopsy of the brain was identi- Zürich, Zürich, Switzerland cal to the initial diagnosis from the biopsy. No Number of Leftward / Rightward deviations 2 Psychologisches Institut, Universität Zürich, tissue changes consistent with brain tumor – in depending on the type of induced emotion Zürich, Switzerland particular lymphoma – were found. Hintergrund: Die Diagnose Restless-legs-Syndrom Conclusions: Age, clinical course with re- (RLS) wird primär anhand klinischer Diagnose- current single lesions and negative oligoclonal kriterien gestellt. Supportive Kriterien wie das bands suggest that TD must be considered dif- Auftreten von periodischen Beinbewegungen im ferently from MS. A relationship between TD Schlaf («PLMS») unterstützen die Diagnose. Bei and MDS or malignant cancer in our patient RLS-Patienten besteht oftmals eine psychiatri- remains speculative but may have an immu- sche Ko-Morbidität. Einzelne Studie weisen für nological dysbalance as cause. Treatment other diese Gruppe von RLS-Patienten auf starke than steroids is not established for TD. Ausprägungen bezüglich subjektiv eingeschätz- ter Variablen (Schweregrad des RLS, Müdigkeit, Beeinträchtigung der Lebensqualität, Somatisie- 22 Remembering negative events induces rungstendenzen) und schwache Ausprägungen leftward deviation in a line bisection task 23 Remission of an anti-MuSK positive bezüglich objektiv messbarer Variablen (z.B. seronegative Myasthenia gravis under C. T. Tamagni , S. C. Croce , V. W. Wettstein , 1 1 1 PLMS) hin («RLS-like«). Rituximab therapy P. B. Brugger1 Ziel: Ziel der Studie war es, RLS-Patienten T. Betz1, M. Tröger1 ohne und mit psychischen Störungen hinsicht- 1 Neuropsychology Unit, Department of lich der unterschiedlichen Ausprägungen psy- Neurology, University Hospital Zurich, Zurich, 1 Kantonsspital Aarau, Neurology, Aarau, chobiologischer Faktoren zu untersuchen. Switzerland Switzerland Methoden: In dieser prospektiven Studie Introduction: Previous studies suggest a relation Introduction: It is well known that patients with wurden 49 «de novo»-Patienten mit der Diag- between emotion and spatial attention. More anti-MuSK positive seronegative Myasthenia gra- nose eines idiopathischen RLS mit dem diagnos- specifically, personality traits like optimism or vis often do not respond sufficently to a therapy tischem Expertensystem DIA-X in die Gruppen positive mood were found to be associated with with inhibitors of cholinesterase and immunosup- «ohne psychische Störungen» (N = 30) und «mit a rightward orientation preference (Drake, 1992; pressive therapy. Remission under intravenous psychischen Störungen» (N = 19) kategorisiert. S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 313 Abstracts Der Vergleich der beiden Gruppen bezüglich 26 Sepsis induces long-term verbal memory 27 Serum Copeptin levels correlate with psychobiologischer Faktoren erfolgte anhand deficits and left-sided hippocampal volume disability in Multiple Sclerosis verschiedener Fragebogen (RLS-DI, IRLS, RLS- reduction in quantitative voxel-based brain M. Katan1, J. Kuhle1, N. Morgenthaler2, 6, SOMS, BSI, FSS, SF-12 und Schlaffragebo- MRI M. Christ-Crain3, L. Kappos1 gen) wie auch mittels Aktimetrie (PLMS) und A. S. Semmler1, K. F. Fliessbach2, J. W. Weide2, Blutanalysen. 1 University Hospital, Neurology, Basel, M. T. H. Heneka2 Ergebnisse: Die Resultate ergaben für die Switzerland Gruppe «ohne psychische Störungen» signi- 1 University Zurich, Zurich, Switzerland 2 Research Department, BRAHMS AG, fikant mehr PLMS. Für die Gruppe «mit psy- 2 University Bonn, Bonn, Germany Hennigsdorf/Berlin, Germany chischen Störungen» zeigten sich stärkere Aus- 3 University Hospital, Endocrinology, Basel, Background: Until recently, critical care practitio- prägungen bezüglich Schweregrad des RLS, Switzerland ners have focused on the survival of patients and Müdigkeit, Beeinträchtigung der Lebensqualität not on long-term outcomes. However, current Background: The degree of activation of the hy- und Somatisierungstendenzen. Es wurden kei- research indicates that critical illness can lead to pothalomo-pituitary-axis (HPA) has been help- ne signifikanten Gruppenunterschiede bezüg- permanent cognitive impairment. Cognitive im- ful in assessing disease severity and prognosis in lich klinischer Hauptkriterien, positiver Fami- pairment may be particularly common in survi- several diseases but the measurement of circulat- lienanamnese, Altersverlauf, Schlafstörungen vors of sepsis. Long-term follow-up examination ing Corticotrophin-releasing hormone (CRH) and und der Blutanalysen (inkl. Eisenstatus) gefun- in rodents indicated that experimental induction arginine-vasopressin (AVP) levels, the main secre- den. of sepsis induces permanent behavioural deficits tagogues of the HPA-axis, is challenging. Copeptin Schlussfolgerung: Das Auftreten von perio- and neuroanatomical changes, such as reduced is released in an equimolar ratio to AVP from the dischen Beinbewegungen ist ein potentieller density of hippocampal neurons and reduced cor- same precursor peptide and is more stable in the Marker zur Unterscheidung von RLS und «RLS- tical cholinergic innervations. circulation and easier to determine. We investi- like». Patients and methods: In this mono-center, gated if copeptin levels correlate with disability retrospective study we recruited 51 patients be- in multiple sclerosis and if it predicts conversion tween 18 and 75 years old for testing of cognitive to clinically definite multiple sclerosis (CDMS) in 25 Reversible cerebral vasoconstriction functions and quantitative voxel-based brain patients with a first demyelinating event (CIS). syndrom as a cause for atypical subarachnoid MRI 9 to 18 months after treatment on an inten- Methods: We measured Copeptin levels in the hemorrhage – two case reports sive care unit (ICU). During acute disease 25 pa- serum of 28 patients with a CIS (EDSS 0–2.5: D. Müntener1, L. Remonda1, H. Hungerbühler1 tients were diagnosed with sepsis. The remaining normal to minimal disability) and 15 MS pa- 26 patients were treated for non-inflammatory tients with clinically confirmed diagnosis (CD- 1 Kantonsspital Aarau, Aarau, Switzerland diseases and served as control group. Cognitive MS) and EDSS scores of >/= 3.5 (at least moder- Introduction: Reversible cerebral vasoconstriction testing was performed by NeuroCogFx, Trail ate disability) using a highly sensitive sandwich syndrome (RCVS) is a disorder characterised by making Test-A and B (TMT-A, TMT-B). Test immunoassay. sudden-onset and servere headache with or with- results were compared with specific normative Results: Median Copeptin levels in MS pa- out additional neurologic symptoms and signs. Im- data and between groups. Magnetic resonance tients were significantly higher compared to aging techniques show multifocal and reversible imaging measurements were performed using a patients with a CIS (4.4 pmol/l (IQR 3.1–8.6 vasoconstriction of cerebral arteries. The etiology 1.5-T scanner with sagittal T1-weighted image pmol/l) versus 3.3 pmol/l (IQR 2.4–4.2 pmol/l), is frequently idiopathic. RCVS can cause ischemic sequences yielding 200 slices and a voxel size p = 0.035). Time of conversion to CDMS was not strokes, parenchymal hemorrhages and nonaneu- of 0.9 x 0.9 x 0.9 mm, followed by statistical mirrored by Copetin levels (Converters, n = 14: rysmal cortical subarachnoid hemorrhages (SAH). parametric mapping. 3.2 pmol/l (2.6–3.8), No-Converters, n = 14: 3.2 Methods/Results: We describe two patients Results: Both, sepsis and other ICU survivors pmol/l (2.3–4.4)). with idiopathic RCVS presenting with thunder- show significant deficits in almost all cognitive Conclusion: The raised Copeptin levels ob- clap-headaches and additional neurologic symp- domains. To test for sepsis-specific effects, we served in patients with established MS and dis- toms. In both cases magnetic resonance imaging analysed the different patient groups separately ability may reflect, disease activity related re- (MRI) delineated a small cortical SAH. Transcra- (sepsis-survivors, survivors of severe sepsis, and lease of inflammatory mediators, or a secondary nial duplex sonography (TCD) and digital sub- non-septic ICU-survivors). Survivors of severe response to disability, or both. Further studies traction angiography (DSA) revealed segmen- sepsis showed verbal memory deficits in com- comparing MS patients with different levels of tal stenoses of multiple cerebral arteries with parison to non-septic ICU-survivors (p = 0.01), disability progression must show if copeptin lev- complete resolution after a few weeks. There and in comparison to survivors of sepsis for trend els are a useful – maybe even prognostic marker was no evidence for a different etiology of SAH (0.07). This was accompanied by a volume re- for disability and disease severity in MS or vasospasms such as intracranial aneurysms, duction of the left-sided hippocampal and para- other vascular malformations, cerebral venous hippocampal areas (p <0.05) in sepsis survivors 28 Sleep disturbance impedes stroke thrombosis or drugs. in quantitative MRI brain volumetry. recovery in the rat Conclusions: RCVS can cause SAH, which, Discussion: In this study we confirmed that in contrast to aneurysmal SAH, have a cortical ICU treatment leads to long-term cognitive im- C. Zunzunegui1, B. Gao1, E. Cam1, A. Hodor1, localisation. Vasoconstriction is likely a cause pairment in various cognitive domains. More C. L. Bassetti1 and not a result of the hemorrhage, although importantly, it shows that survivors of severe 1 Department of Neurology, University Hospital the pathophysiology is not clearly understood. sepsis show an impairment of verbal memory of Zurich, Zurich, Switzerland associated with a reduction of left sided hippo- campal volume. These findings argue for sepsis- Introduction: Sleep disturbance (SDis) represents specific effects of cognitive impairment after ICU a risk factor for stroke recovery. We have previ- treatment and demonstrate that survivors of se- ously showed that SDis over 3 days aggravates vere sepsis are at particular risk for long-term brain damage in the rat. The aim of this study is cognitive impairment after full recovery. to further investigate the effects of SDis on the function recovery. Methods: 17 male Sprague Dawley rats were subjected to ischemic surgery by occlusion of the distal branches of Middle Cerebral Artery (MCAo). 12 hours after ischemia, one group of S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 314 Abstracts rats (n = 9) was subjected to SDis (12 h of sleep of nocturnal breathing and leg movements). Postersession, deprivation over 3 days) by gentle handling and However, REM sleep behavior disorder (RBD) Freitag, 6.11.2009, 13.00–14.00 Uhr another group (n = 8) left undisturbed after was found in six PD patients with SW (in all 4 Gruppe 4 ischemia to serve as control. Single Pellet Reach- with de novo SW). ing test (SPR) was used to assess motor function. Conclusion: SW in PD appears to be associ- 31 Suppression of gesture pantomiming by Results: After two-three weeks of training, ated with depression, night-time hallucinations inhibitory theta-burst rTMS over left inferior the success of SPR reached about 50% (nor- and RBD. No differences were found, however, frontal lobe malized as 100% for baseline). It dramatically in polysomnographic parameters between PD S. Bohlhalter1, M. Bertschi1, T. Vanbellingen2, dropped to 4% of baseline after MCAo in both patients with and without SW. P. Wurtz3, T. Nyffeler1, C. W. Hess1, R. Müri1 groups. SPR continually recovered in the con- trol group, reaching 50.36% ± 23.95 at the end 1 Department of Neurology, Inselspital, Berne, of 4 weeks. In contrast, it remained at 24.70% 30 Soluble CD70: a novel immunotherapeutic Switzerland ± 24.34 in the SDis group. Repeated measures agent for experimental glioblastoma 2 Klinik Bethesda, Neurorehabilitation Centre, ANOVA indicated a significant difference (p = Tschugg, Switzerland J. Miller2, G. Eisele1, G. Tabatabai1, 0.001) in group*time interaction (F [6, 90] = 3 Perception and Eye Movement Laboratory, S. Aulwurm3, G. von Kuerthy1, L. Stitz4, 4.470). Post hoc independent t-tests showed Departments of Neurology and Clinical Re- P. Roth1, M. Weller1 that group difference was significant at day 14 search, Inselspital, Berne, Switzerland (Control 44.54 ± 16.52 vs SDis 19.36 ± 25.5, 1 University Hospital Zurich, Department Background: The traditional view of a predominant p = 0.031), 21 (Control 55.3 ± 25.57 vs SDis of Neurology, Zurich, Switzerland inferior parietal control of gesture production has 21.05 ± 20.89, p = 0.008) and 28 (Control 50.36 2 Department of Neurosurgery, Indiana Univer- been recently challenged by neuroimaging stud- ± 23.95 vs SDis 24.70 ± 24.34, p = 0.045), but sity, Indianapolis, United States ies demonstrating that gesture pantomime may not at days 1, 4 and 7. 3 Department of General Neurology, Hertie In- critically depend on inferior frontal function. The Conclusion: Sleep disturbance over 3 days has stitute for Clinical Brain Research, Tuebingen, aim of the present work was therefore to investi- a significant detrimental effect on long term re- Germany gate the effect of transient disruption by repetitive covery of motor function in the rat. 4 Institute of Immunology, Friedrich Loeffler transcranial magnetic stimulation (rTMS) over Institute, Tuebingen, Germany these brain sites on pantomime and imitation of Purpose of the study: Expression of CD70 on the sur- gestures. 29 Sleepwalking in patients with Parkinson’s Methods: In 10 right handed healthy subjects face of glioma cells was shown to induce a potent disease (5 women, aged 25–39) an inhibitory continu- anti-tumor immune response in a murine glioma N. D. Di Fabio1, R. P. Poryazova1, M. O. Ober- model in vivo. There is evidence that also the ous theta-burst protocol of rTMS (cTBS) was holzer1, C. B. Baumann1, C. B. Bassetti1 soluble form of CD70 is active in vivo. A soluble applied over left inferior parietal lobule (IPL) co-stimulatory ligand is attractive as it might have and inferior frontal gyrus (IFG) versus baseline 1 University Hospital Zurich, Zurich, Switzerland a wider distribution into the tissue surrounding (BSL).We employed a repeated measures design Introduction: Sleepwalking (SW) corresponds to a the tumor in vivo. with 1 week intervals between the 3 sessions in complex sleep-associated behavior, characterized Methods: The murine glioma cell line SMA- balanced order. Gesturing was assessed off-line by locomotion, mental confusion, and amnesia. In 560 was engineered to produce a soluble from within 15 minutes after cTBS using a recently a single center series of 165 consecutive patients of CD70 (sCD70). The expression of sCD70 was developed test of upper limb apraxia (TULIA), with Parkinson’s disease (PD), we found de novo confirmed by immunoblot. Markers of immune which includes a pantomime and imitation SW in 6 subjects (4%, Arch Neurol 2008). In a cells were assessed by flow cytometry. The pro- subtests. Based on its highly sensitive scoring questionnaire survey supported by the patient’s liferation of immune cells was investigated in a method the TULIA test shows no ceiling effect organization Parkinson Switzerland, 36 (9%) out 3H-Thymidine assay. The secretion of IL-2 and (cut off for apraxia <194, maximum score 240). of 417 patients with SW were identified. The clini- IFN-gamma was assessed by ELISA. SMA-560 Performance was assessed by a blinded rater cal and polysomnographic characteristics of PD cells were implanted stereotactically in the right based on video recordings. patients with SW are essentially unknown. striatum of syngeneic VM/Dk mice. The infiltra- Results: The findings revealed that cTBS sig- Aim: To assess the polysomnographic char- tion of immune cells in gliomas was assessed by nificantly affected total TULIA scores (F = 9.350, acteristics of SW in PD patients. immunohistochemistry. CD8+ or NK cells were p = 0.02, repeated measures ANOVA), which is Patients and methods: Out of 417 patients depleted by the injection of appropriate antibod- mainly explained by the effect on the panto- participating in the survey, 8 PD patients with ies intraperitoneally. mime subtest (F = 11.082, p = 0.009), as imita- SW (in four of them since childhood) and 6 PD Results: In vitro expression of sCD70 en- tion scores remained unchanged (F = 1.887, p = patients without SW were assessed. A detailed hances proliferation of syngeneic splenocytes 0.2). Furthermore, significant differences were clinical examination (including UPDRS III and and their secretion of IL-2 and IFN-gamma. found between left IFG and IPL stimulation for mini mental state examination), standardized In syngeneic VM/Dk mice bearing intracranial total scores (225.6 ± 7.8 and 231.0 ± 6.4, p = questionnaires (including validated sleep ques- gliomas, sCD70 enhances the survival rate. The 0.02, post hoc paired t-test) and particularly tionnaires, the Beck depression and anxiety infiltration of CD8+ cells is enhanced compared pantomime subscores (111.7 ± 4.8 and 115.0 inventory) and a conventional overnight poly- to controls. Depletion of CD8+ cells abrogates ± 3.9, p = 0.004). When contrasted with BSL, a somnography were performed. the survival advantage. decrease of total scores at the level of a statistical Results: Compared to PD patients without Conclusion: These data suggest that sCD70 trend was found for cTBS of IFG (225.6 ± 7.8 SW, those with SW had a similar disease dura- is a potent stimulator of anti-glioma immune and 228.6 ± 5.5, p = 0.09) or a just significant tion and motor symptom severity (UPDRS III), response with cytotoxic CD8+ T-cells playing a increase for cTBS of IPL (231.0 ± 6.4 and 228.6 but higher scores on the Beck Depression Inven- major role. ± 5.5, p = 0.05). tory (p <0.001). Mini mental state examination Conclusion: The suppression of gesture pan- scores did not differ between the two groups. tomiming by cTBS over left IFG points to an in- Night-time hallucinations were reported in 7 out ferior frontal rather than inferior parietal gesture of 8 patients with SW, but only in 1 PD patient representation. Furthermore, the findings sug- without SW. gests that inhibition of IPL favours the inferior There were no differences in polysomno- frontal function during gesture pantomime in- graphic sleep parameters (sleep latency, sleep dicating that such intrahemispherical facilitation efficiency, sleep stages percentages, and indices may have clinical implications. S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 315 Abstracts 32 The NADPH oxidase Nox-1 is a mediator Introduction: Amyotrophic lateral sclerosis not be an exclusion criteria for thrombolytic of brain injury in experimental stroke (ALS) is characterized by progressive neuronal therapy after stroke. degeneration. This study aimed at analyzing T. Kahles1, A. Kohnen1, S. Heumueller1, whether genetic variants of homocysteine me- A. Rappert1, I. Bechmann1, S. Liebner1, tabolism are associated with the development 35 Varicella zoster virus – hide and seek in an I. M. Wittko1, K.-H. Krause2, T. Neumann- of ALS. immunocompetent patient Haefelin1, H. Steinmetz1, R. P. Brandes1 Subjects and Methods: 162 patients diagnosed A. Becker, S. Biethahn, U. W. Buettner 1 JW Goethe University, Frankfurt, Germany with ALS by modified Airlie House El Escorial 2 Centre Medical Universitaire, Geneva, criteria and 162 healthy subjects were enrolled. Kantonsspital Aarau, Aarau Switzerland Switzerland Genomic DNA from peripheral leucocytes was Introduction: Encephalitis and angiitis are possible, used for genotyping of seven genetic variants Cerebral ischemia and reperfusion is associated but uncommon complications of varicella-zoster- of Hcy metabolism by allele specific PCR with with the generation of reactive oxygen species virus (VZV) infection, usually proceeded by a rash subsequent restriction analysis. In binominal (ROS). ROS are considered to contribute to the [1, 2]. Cerebro-spinal fluid (CSF)-PCR is an im- regression analysis, the association between the brain damage process. Mitochondria and the clas- portant diagnostic tool to confirm the diagnosis. genetic variants and ALS was analyzed with age sic leukocyte-type NADPH oxidase are thought We present a patient with a history suggestive of and gender as covariables and threshold = 0.05. to be the significant sources of ROS in this sce- VZV-angiitis and -encephalitis without a rash and Results: The frequency of the genotypes of nario. Although the NADPH oxidase Nox-1 also negative PCR. the variant MTHFR c.677C>T (p.A222V) was produces ROS, little is known regarding its role Case report: The immunocompetent patient different between patients and controls, i.e., the for cerebral ROS formation and ischemia/reper- presented with a history of headache, tempera- T-allele was more frequent in the patients than fusion-induced brain injury. We studied this as- ture up to 38 °C, ataxia with falls and a progres- in controls: patients, CC/CT/TT: 0.40/0.48/0.13; pect in Nox-1 knockout-mice and their wildtype sive confusional state with reduced vigilance controls, CC/CT/TT: 0.50/0.44/0.06 (Wald = littermates. for three weeks. The CSF was abnormal with 5.39; p = 0.020). After isolation of cells from the murine 42 cells (mononuclear) and a protein of 2 g/l. Discussion: In this study we found the ther- brain, we demonstrated the expression of Nox- A broad search for pathogenic agents revealed molabile variant of MTHFR as possible risk factor 1 in astrocytes, neurons as well as endothelial positive values for VZV IgM in Serum, CSF-PCR for ALS. This polymorphism is known to lower cells using RT-PCR. Transient focal cerebral isch- was negative. On MRI, multiple small ischemias MTHFR activity and to contribute to elevated emia was induced by the middle cerebral artery were present. On MR-angiography, convention- plasma Hcy levels, which have been observed filament occlusion followed by reperfusion. Le- al angiography and neurosonology multiple ste- in ALS patients. These results suggest that the sion volume was assessed using TTC staining. noses of the basal arteries were found. T-allele of MTHFR c.677C>T may contribute a Furthermore, we examined the integrity of the A therapy with acyclovir was initiated, four risk of ALS, which should be tested in additional blood-brain-barrier in vivo with sodium fluores- days later the patient was orientated, awake, study samples. cein (SF) and Evans blue (EB) as permeability GCS 15. However, after discussion of the PCR markers. and IgM-results concerning VZV, a non-specific Vascular architecture was similar between polyclonal stimulation was assumed, and acy- 34 Thrombolytic therapy in stroke patients WT and KO animals as were filament-induced clovir was stopped. In the course of another older than 85 years changes in cerebral blood flow in this model of three days the patient deteriorated again, this transient focal cerebral ischemia. Lesion volume A. Häne1, H. Hungerbühler1, G. Schwegler1 time also developing a slight hemiparesis on the after one hour of ischemia and 23 hours of re- left side. Acyclovir was initiated again. Over a 1 Kantonsspital Aarau, Aarau, Switzerland perfusion however was significantly reduced in period of 10 days the patient recovered, was brains of KO as compared to those of WT mice Introduction: There is only few data about the out- alert, orientated, able to walk with slight ataxia, (WT 77 mm3 ± 10, KO 43 mm3 ± 10 mean ± sem; come of very old patients being treated by throm- the hemiparesis wore off. Furthermore, the ste- p <0.05). In line with this observation, the clini- bolysis after stroke. noses decreased. The neurosonologic examina- cal brain injury score was significantly lower in Methods: We retrospectively analyzed the tion before discharge was normal. Additionally, KO than in WT mice (modified Bederson score, outcome of patients aged over 85 years, who no further ischemic lesions occurred on MRI. WT: 1.5, KO: 0.9; p <0.05). Early BBB damage underwent intravenous (IV) or intraarterial Discussion: Most likely this patient suffered was significantly smaller in brains of KO as com- (IA) thrombolysis in our stroke center between from VZV-angiitis and encephalitis, despite the pared to those of WT littermates (SF: WT 149 ng February 2004 and November 2008. lack of a rash or a positive PCR-result. How- ± 19, KO 72 ng ± 17; p <0.05). Accordingly, brain Results: Of 34 included patients, 71% (n = ever, a rash has been described to occur in only edema formation was attenuated in KO mice 24) were aged 85 to 90 years and 29% (n = 10) about 60% of patients , and the sensitivity (WT: 57 mm3 ± 7, KO: 35 mm3 ± 5; p <0.05). were older than 90 years. 44% had an occlusion of VZV-PCR varies between 50 and 90% in the These data demonstrate that the Nox-1- of the middle cerebral artery (M1 segment 21%, literature. containing NADPH oxidase is expressed in the M2 segment 23%). 82% of the patients were Conclusion: In a patient with typical clinical murine brain and contributes to the pathophysi- treated by IV thrombolysis, 15% by IA throm- features of a VZV-angiitis and -enzephalitis, the ology of stroke. Inhibition of the NADPH oxidase bolysis. One patient underwent a combined IV lack of a rash or a negative PCR does not neces- may serve as a new approach to reduce brain and IA thrombolysis. Favourable outcome was sarily rule out the diagnosis. injury after stroke. defined as modified Ranking Scale (mRS) <=2 or mRS 3 for the subgroup of patients with References historical prestroke mRS 3. Three months after 33 The thermolabile variant of stroke 29% of the patients reached a favourable 1 The neurotropic herpes viruses: herpes sim- 5,10-methylenetetrahydrofolate reductase as outcome. 21% had a mRS of 4 or 5 and 26% plex and varicella-zoster. I. Steiner, possible risk factor for amyotrophic lateral died. In the subgroup of patients older than 90 PGE Kennedy, AR Pachner. sclerosis: a case-control study years outcome was favourable in 40%. Lancet Neurology. 2007;6:1015–28. Conclusion: As expected, mortality is high- 2 The varicella zoster virus vasculopathies: B. I. Ineichen1, P. K. Kühnlein2, M. F. Farkas1, er and the outcome of patients aged over 85 Clinical, CSF, imaging and virologic features. S. K. Keskitalo1, A. S. Semmler1, M. W. Weller1, years after thrombolysis is not as beneficial as in MA Nagel et al. Neurology. 2008;70:853–60. A. L. Ludolph2, H. J. Jung1, M. L. Linnebank1 younger patients. Nonetheless, our data suggest 1 University Zurich, Zurich, Switzerland that some very old patients might benefit from 2 University Ulm, Ulm, Germany thrombolysis. Therefore very high age should S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 316 Abstracts 36 The increased number of regenerated craniectomy. It consists of neurologic symptoms group. However, the decrease in ESS scores was axons after thyroid hormone treatment of and deficits like controlateral weakness. higher in the treatment group (–2.3 ± 2.3 vs 0.7 transected sciatic nerve is accompanied We report the case of a 37-year-old man ± 1.8, p = 0.005). Objective estimation in the by improvement of muscle reinnervation who had suffered severe traumatic brain injury, MWT confirmed the amelioration of EDS only needing a large craniectomy because of right in the treatment group. Psychomotor vigilance P. A. Panaite, T. Kuntzer, I. Barakat-Walter hemispheric oedema. He presented with discrete test results were similar in both groups. Laboratory of Neurology Research, Neurology left hemiparesis and neglect; his gait was safe. Conclusion: Modafinil appears to be effective Service, CHUV, and Department of Cell Biology During the following weeks, left-sided weak- in the treatment of posttraumatic EDS and fa- and Morphology, University of Lausanne, ness became more important with plegia of the tigue. Lausanne, Switzerland hand and increasing difficulties to walk. A CT revealed no new lesion and EEG showed no epi- Injuries of peripheral nerves are common in clini- leptic activity. 39 State space mapping of sleep and cal practice, and axonopathy is the usual mecha- Following cranioplasty, his condition im- wakefulness in Parkinson patients and nism explaining loss of motor function and muscle proved markedly: within a few days, hemiple- healthy controls atrophy. Since reinnervation is essential for the re- gia nearly fully recovered and neglect improved turn of nerve function, the main goal of peripheral Johannes Sarnthein1, Esther Werth2, considerably. nerve repair is to induce as many axons as possible Christian R Baumann2 Grant and Norcross (Ann Surg 110, 1939) to regenerate, enter the distal stump and proceed first described the syndrome of the trephined. 1 Neurochirurgische Klinik, UniversitätsSpital, to reinnervate the end organs. The physiopathology remains unclear: direct Zurich, Switzerland In our previous works, we have already influence of the atmospheric pressure, altered 2 Neurologische Klinik, UniversitätsSpital, shown that the administration of thyroid hor- CFS circulation, decreased cerebral perfusion, Zurich, Switzerland mone (T3) in either silicon or biodegradable metabolic changes, have been proposed to ex- nerve guides significantly increased the num- Background: Sleep is commonly scored in stages, plain the syndrome. ber and the myelination of regenerated axons but the succession of behavioural states is only In our patient, the very large defect and (J Neurotrauma. 2007;24:567–77). Therefore, poorly understood. For a better description of prolonged upright body position have probably it is important to investigate whether the in- sleep-wake dynamics, we have adapted state space contributed to the occurrence of the syndrome. crease in number of regenerated axons after analysis (SSA, originally applied in rodents) to T3-treatment of transected rat sciatic nerve is analyze sleep in patients with Parkinson’s disease accompanied by an improvement in hind limb (PD) and healthy controls. 38 Modafinil ameliorates posttraumatic muscle reinnervation, as assessed by changes Methods: We obtained polysomnographic re- sleepiness and fatigue: A double-blind, in morphology and number of neuromuscular cordings from 7 PD patients and 7 age-matched randomized, placebo-controlled pilot study junctions (NMJ). healthy controls. 30-s epochs were scored vi- NMJ were examined in PBS-control and in Philippe Kaiser, MSc, Philipp Valko, MD, sually. EEG data were Fourier-transformed to T3-treated rats on serial longitudinal hind limb Esther Werth, PhD, Janine Thomann, MSc, obtain the power spectral density for each epoch. muscles (gastrocnemius and plantar) stained Judith Meier, Claudio L. Bassetti, MD, Thereafter, we calculated 3 ratios of frequency with rhodamine-conjugated a-bungarotoxin Christian R. Baumann, MD bands. While these ratios aim at capturing specif- and anti-neurofilament antibody: At 4 weeks ic spectral features, their final nature is heuristic. Department of Neurology, University Hospital of sciatic nerve regeneration, only rare regen- After data processing via principal component Zurich, Switzerland erated axons were observed in PBS-control analysis, smoothed time series were plotted as rats, and all end-plates were still denervated. Background: Excessive daytime sleepiness (EDS) clusters and trajectories in a three-dimensional In T3-treated rats, few reinnervated NMJ can and fatigue are frequent complaints following state space. The congruence between visual scor- be observed. Fourteen weeks following nerve traumatic brain injury (TBI). In our prospective ing and the classification based on EEG spectra regeneration, the histological analysis revealed study on sleep-wake-disturbances 6 months after was quantified with discriminant analysis (DA). the presence of a higher number of regenerated trauma, we found EDS in 38%, and fatigue in We assessed ratios with best cluster discrimi- axons in T3-treated rats than in control rats. The 17% of 65 consecutive TBI patients. Given the nation (optimized for maximal surface of the morphometric measurement and the statistical high frequency of TBI in the population and the triangle spanned between the centroids of the analysis revealed a significant increase in the enormous socioeconomic impact of vigilance im- DA) and congruence. size and shape complexity of NMJ in T3-treated pairment in TBI patients, treatment strategies are Results: Power spectra revealed only small rats as compared to PBS-controls. Moreover, urgently warranted but do not exists until now. differences between PD patients and controls. the density of acetylcholine receptors on post- We aimed at studying the effect of daily modafinil Congruent scoring was obtained for a median of synaptic membrane was significantly higher in on posttraumatic EDS and fatigue. 90% of epochs in PD patients, and 93% in con- T3-treated rats. Methods: We ran a double-blind, randomized, trols. The median surface of the triangle between These results suggest that the T3-treatment placebo-controlled pilot study in 20 TBI patients centroids was 49 units for PD patients, and 180 stimulates not only the number of regenerated with posttraumatic EDS or fatigue. Fatigue was units for controls. In all subjects, trajectory ve- axons but also improves hind limb muscle rein- diagnosed with the fatigue severity scale (FSS), locity within clusters was low for deep and REM nervation. and EDS with the Epworth sleepiness scale sleep, indicating that EEG spectra of subsequent This work was supported by a special grant (ESS). The Beck Depression and Anxiety Inven- epochs in these sleep states show low variability. from the SUVA. tory was used to assess depression and anxiety Highest velocities were found for wakefulness symptoms. At baseline and six weeks following and epochs adjacent to state transitions. treatment initiation (100–200 mg modafinil vs Conclusion: SSA can be applied to sleep re- 37 Syndrome of the Trephined placebo), we applied these questionnaires and cordings of both healthy controls and PD pa- A. Tanga, B. Leemann, A. Schnider performed actigraphy, conventional nocturnal tients. Embedding of further polysomnographic polysomnography, maintenance of wakefulness parameters such as submental muscle tone may Hôpital Cantonal de Genève, tests (MWT), and psychomotor vigilance tests. improve discriminatory power. The further de- Service de Neurorééducation Results: Demographic, psychiatric and sleep velopment of this method may improve our un- After a large craniectomy, a cranioplasty is not baseline characteristics did not differ between derstanding of regulation of sleep and wakeful- only cosmetic but also prevents or improves the the two groups (each: n = 10). After 6 weeks ness in health and disease. syndrome of the trephined. This syndrome is a of treatment, there was no significant effect on possible delayed complication of decompressive fatigue (as assessed by the FSS) in the modafinil S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 317 Abstracts 40 Population-based study of acute phase 41 Restless legs syndrome in pregnancy: 42 Experience of Moyamoya angiopathy blood pressure after TIA and stroke in relation A prospective, systematic study in Zurich to pre-morbid measurements, underlying A. Hübner1, A. Krafft2, S. Gadient1, E. Werth1, Y. Yonekawa, M. Hug, M. Wiesli, M. Fujioka, pathology and long-term outcome R. Zimmermann2, C. L. Bassetti1 J. Fandino U. Fischer1, L. Bull2, L. Silver2, Z. Metha2, 1 University Hospital Zurich, Department of University of Zurich, Kinderspital Zurich, P. M. Rothwell2 Neurology, Zurich, Switzerland Klinik im Park Zurich, Kantonsspital Aarau 1 Department of Neurology, Inselspital, Univer- 2 University Hospital Zurich, Obstetrics, Zurich, Introduction: Moyamoya angiopathy MMA (Moya- sity Hospital Berne, Berne, Switzerland Switzerland moya disease, Moyamoya syndrome and Moya- 2 Stroke Prevention Research Unit, John Rad- Introduction: Three retrospective studies suggested moya moid [unilateral pathology]) has been cliffe Hospital, University Department of Clini- a high frequency (11–26%) of restless legs syn- considered to be prevalent in Japan and Asian cal Neurology, Oxford, United Kingdom drome (RLS) in pregnancy. None of these studies countries. Now we know its existence also in Eu- Background: Blood pressure (BP) is elevated in used standardized methods for assessment of RLS ropean countries. Since 1993 we have experienced many patients with acute stroke, but the mecha- and sleep. Characteristics and determinants of RLS 68 cases submitted to multiple microvascular ex- nism is uncertain. Moreover, the extent of “post- during pregnancy are poorly known. tracranial – intracranial EC-IC bypass revascular- stroke” hypertension has not previously been re- Methods: Women during (from the 1st tri- ization procedures. We present the management lated to detailed records of pre-morbid BP, and so mester) and after pregnancy (8 weeks post- of patients with MMA and the postoperative re- it is uncertain how many patients have exhibited partum) are prospectively studied. Assessment sults based on our experience. a tendency to similar or higher BPs in the past in includes 1) interview about RLS-symptoms Method: Definitive diagnosis according to di- the absence of stroke. In the first ever popula- and sleep habits/disturbances: 2) standardized agnostic criteria can be done now with magnetic tion-based study of pre- and post-stroke BPs, we questionnaires (incl. the international RLS-scale resonance angiography MRA along with clinical compared acute post-stroke BPs with all recorded [IRLSS], Epworth sleepiness scale [ESS], and symptomatology. Digital subtraction angiogra- pre-stroke measurements. Pittsburgh Sleep Quality Questionnaire [PSQI]); phy DSA is still important to know the delicate Methods: We collected all acute phase post- 3) blood tests (incl. hemoglobin, estrogen, C- angiopathology and for the planning of surgical event systolic (SBP) and diastolic (DBP) read- reactive protein and ferritin), 4) leg actigraphy revascularization procedure. H215O PET is car- ings in all patients with an acute stroke or TIA (3rd trimester and post-partum). ried out for measurement of regional cerebral in the Oxford Vascular Study and related these Results: So far 498 women were screened. blood flow rCBF and hemodynamic reserves. to clinical characteristics, stroke subtype and all RLS was diagnosed in 52 women (10.4%), 38 Non-invasive Doppler-sonography is important recorded pre-stroke measurements by access to were included in the study. 37% of them had for regular following-up to know the hemody- life-long general practice patient records. We a positive family history for RLS, 34% reported namic situation. also assessed the association between early post- onset of RLS-symptoms before the 20th week, Multiple bypass procedures of direct vascu- stroke BP and risk of recurrent vascular events 18% suffered from RLS before pregnancy, 45% larization have been performed as therapy of during long-term follow-up. had RLS-symptoms daily, 42% had an IRLSS choice for augmentation of CBF. Results: 18,035 premorbid BP measurements >20, and 100% had a PSQI >5 (all values in the Results: The series contains 45 children (0.1– were available from 1047 (98%) of 1069 con- 3rd trimester). Anemia (defined as Hb <11 g/dl 17 yrs Av 6.8 yrs; f/m 1.36) and 23 adults(21–66 secutive eligible patients. First SBP in the acute in pregnant women) was found in 18% of yrs Av 39 yrs; f/m 4.75). The patients are also phase was higher than the most recent pre-event affected and unaffected women. Ferritin levels from Germany (14), Holland (8), Austria (5), reading in 65.4% (95% CI: 62.5–68.3) of pa- <35 ug/l were found in 75% of RLS positive and Denmark (3), Italy (3), Norway (1), Sweden (1), tients but only exceeded the highest pre-event in 94% of RLS negative women. Women with Qatar (1): besides Caucasian, middle east origin reading in 26.3% (95% CI: 23.6–29.0). This lat- (pRLS) and without RLS (nRLS) had similar val- (4) and Asian origin (3). One thirds of patients ter proportion varied with TOAST subtype of ues in hemoglobin CRP, and ferritin (mean Hb have basic disease such as neurofibromatosis stroke (p = 0.001), ranging from 16.7% in car- 11.7 mg/dl in pRLS, 12 mg/dl in nRLS; Ferritin type 1, trisomy 21, hemoglobinopathy etc, so dioembolic stroke to 34.2% in lacunar stroke. 22.6 μg/l in pRLS, 19.1 in nRLS). No signifi- that these cases can be categorized as Moyamoya In patients with a first post-event SBP measured cant difference of estrogen levels between pRLS syndrome. Two adult patients presented with within 3 hours of the ictus, mean (SD) acute SBP and nRLS (measured in 15 pRLS and 10 nRLS intracerebral hemorrhage. Almost all other pa- was higher in patients with TIA and minor stroke in 3rd trimester. Leg actigraphy was performed tients presented with ischemia-infarction. (NIHSS ≤3) than in those with major (NIHSS in 7 cases pre and post partum, PLMI dropped One adult patient died of perioperative isch- >3) ischaemic stroke (163.9 vs 156.5 mm Hg, p dramatically. emic edema. Among others, three other patients = 0.017), but was otherwise unrelated to stroke Conclusion: Preliminary results of this ongo- had perioperative ischemic complication but severity. First post-event BP was unrelated to ing study suggest that RLS in pregnancy: 1) is showed good recovery. Other patients profited risk of recurrent stroke or other vascular events. present in 10% of women; 2) frequently appears from augmentation of CBF by multiple bypass Conclusions: Acute post-event SBP is lower early in pregnancy; 3) is often severe/ frequent; surgery. than highest recorded pre-morbid SBP in the 4) may not be related (only) to anemia/low fer- Conclusions: We present the state of the art majority of patients, and is highest in patients ritin levels or estrogen levels; 5) has a significant of management of MMA including diagnosis, with TIA and minor ischaemic stroke, suggesting impact on sleep quality. surgical treatment and the consequential re- that post-stroke hypertension is not a unique sults. In addition, long term follow-up results physiological response to major cerebral infarc- are presented along with distinctions in clini- tion. cal pathophysiology of MMA between patients from Europe and Japan. S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E 2009;160(7):305–18 www.sanp.ch | www.asnp.ch 318
"Abstracts der Jahrestagung SNG Aarau"