Docstoc

Mollaret Meningitis

Document Sample
Mollaret Meningitis Powered By Docstoc
					Mulling over Mollaret’s


          MIDG
     9th October 2007

     Ashwin Swaminathan
            RMH
                           Case 1
• 72 yo male

• Lebanese ethnicity
   – Migrated to Australia 1970’s

• Medical problems
   –   IHD
   –   ↑BP
   –   Depression
   –   Chronic benzodiazepine use
                  Presentation
– R Ear progressive deafness
   • long-standing > 10 years
– 1 yr frontal headache
– 4 weeks “blurry vision”

O/E
  - Diplopia on left lateral gaze
   - Deaf R ear
             MRI scan:
Cerebello-pontine epidermoid tumour




T2 – appears bright         T1 - dark
                        Operation
• Elective craniotomy with excision of right posterior fossa
  epidermoid cyst

• Operative notes:

   “Epidermoid cyst entered and all loose inner material removed”
   “Capsule adherant to cranial nerves and brainstem (was) left intact”
   “Tumour wrapped around CN 7, 8, 9 and 11”
   “Dura closed water-tight”

   No EVD inserted


• Histology: benign epidermoid cyst tissue
                     Post-op progress
• Day + 3:
    – Visual / auditory hallucinations
    - Afebrile
    - CRP 23 WCC 10

• CSF:
   – Neutrophils           - 42 cells / mm3
   – Lymphocytes           - 36 cells / mm3
   – RCC                   - 640 cells / mm3
   – Protein               - 1.66 g / L
   – Glucose               - 2.1 mmol / L
   – Gram stain            - No bacteria seen

•   Management?
    – Neurosurgical unit keen on Abx
    – IV Ceftriaxone + vancomycin for 5/7
• Day + 10
  –   generally improved
  –   CSF culture negative
  –   Anti-biotics ceased
  –   Prescribed NSAID analgesia



• Day + 25
  – Developed L arm and leg weakness
  – MRI scan:
       • R cerebral peduncle infarct
       • Residual epidermoid occluding R sup cerebellar artery
• Day + 33
     – Intermittent confusion
     – Drowsiness
     – Afebrile

•   Ix
     – CRP 49      WCC Normal

     – CSF
        • Neut              13
        • Lymph             17
        • Protein           2.2
        • Glucose           1.8
        • Cytology          No large mononuclear cells or neoplastic cells
        • Crypto Ag         Neg
        • ZN stain          No AFBs seen
        • Herpes MP PCR     Neg

•   Rx
     – Ceftazidime and vancomycin
Day +42 - 46
• Worsening confusion
• Neck stiffness
• Hypoxia ? central

Ix:
      – CRP          40      WCC    17

      – CT Brain: mildly prominent ventricles

      – CSF:
               –   Neut       27
               –   Lymph      17
               –   Protein   3.45
               –   Gluc      1.7
Rx:
      - Vancomycin and meropenem
      - NSAIDS still charted
Differential?
Day +48
• Decreased GCS
• R III CN palsy
• Intubated ICU

Ix:
                                       No response to Rx
      - CRP   15        WCC 17.5
                                       Neurological decline
      - CSF:                           Day +55: deceased
          - No cell count: clotted
          - Prot 3.2
          - Gluc 1.1

Rx:
          - TB Antibiotics commenced
                     CSF profile
Day post-op    +5         +33           + 42          + 48
   Protein     1.7         2.2            3.5           3.2
     g/L


   Glucose     2.1         1.8            1.7           1.1
    mmol/L


 Neutrophils   42          13             27             *
   Cells/mm3


   Lymphs      36          10             17             *
   Cells/mm3


   Other                Cytology -     Cytology -   Herpes PCR -
                       Crypto Ag –    Crypto Ag –
                       Herpes PCR -    TB PCR -
Comments?
              Post-mortem findings
• Mucoepidermoid Squamous Cell Carcinoma arising within
  epidermoid cyst

• SCC infiltrating
   – Pons & cerebral peduncle
   – Cranial nerves
   – Grey matter of R temporal cortex


• No evidence of CNS infection

• “Respiratory problems may be 2o to brainstem involvement”
                      Case 2
• 70 yo man
• Caucasian background
• Lives alone, independent ADLs

• Past Medical History
  – 1996 Right posterior fossa epidermoid cyst excised
• Presenting Complaint
  – R hearing loss
  – Increasing forgetfulness

  MRI Brain:
   - Recurrence of large post fossa epidermoid cyst
                     Operation
• Right fronto-parietal craniotomy
• Debulking / subtotal excision of CP tumour

• “Capsule opened and partially excised”
• “Suction of contents in piecemeal manner”

• No EVD inserted

• Histology
   – Benign epidermoid cyst tissue
• 1st admission
  – Recovered well post-op
  – Discharged home day + 4
  – Weaning course of dexamethasone

• 2nd admission
  –   Day + 14
  –   Subcutaneous CSF leak
  –   Systemically well, afebrile
  –   Debridement and resuture of cyst / wound
  –   3/7 of cephazolin
                                     Admission 3
Day + 22                                            Day + 25 – 33
•     Increasing confusion at home
•     Unsteady gait
•     “Talking gibberish”                           •   “Pleasantly” confused
                                                    •   Occasional paranoia
•     No headache / fever / sweats / meningism
                                                    •   Falls
Ix:                                                 •   Afebrile
      - WCC: normal                  CRP: 3
      - CSF
            - Neut                   284
            - Lymph                  42
            - Protein                2.2
            - Glucose                2.6
            - Cytology               inflammatory

Seen by ID
    - Antibiotics witheld - observation
Day 38

  – Ongoing mild confusion, but not clinically unwell
  – Occasional low grade temp (<38C)

  – CRP 60
  – CSF
     •   Neut              1130
     •   Lymph             130
     •   Prot              1.8
     •   Glucose           2.2

  – Commenced Vancomycin & Ceftriaxone (5/7 course)
     • At surgical units behest
     • Culture negative

  – Discharged Day + 47
                         Admission 4
• Day + 55 - 70
   –   Re-admitted with increasing confusion
   –   Decreased functional state
   –   Drowsy ++
   –   GCS 8 - 13

   – Low grade Temp <38 C

   – CSF Cloudy
        •   PMN           750
        •   Lymph         50
        •   Protein       1.59
        •   Glucose       1.9

   – Commenced Vanc and CTX
• Day + 70 – 80

  –   Insertion of EVD VP shunt
  –   No improvement in GCS
  –   Discussion with family regarding NFR status
  –   Antibiotics ceased after 2/52 with no improvement

  – CSF profile
       •   Neuts         40
       •   Lymphs        9
       •   Protein       0.80
       •   Glucose       2.0

  – CSF Herpes Multiplex PCR +ve
                     CMV
             ? Significance
        Further testing (VIDRL)
• CSF
  – Semi-quantitative viral load ~ 200 copies / ml
  – Earlier specimens tested – not detected

• Blood
  – CMV not detected
  – HIV negative

Conclusion: CMV likely not significant
             Day + 80: MET Call !
• Seizures
   – Generalised tonic-clonic seizures
   – Prolonged post-ictal

   – CT Brain – no new changes

   – Rx: phenytoin

• Patient wakes up next morning
• Lucid, reasonably orientated
• Rapid improvement discharged to rehab following week!

  DIAGNOSIS:           Presumed status epilepticus
Comments ?
                        Review
• Epidermoid cysts
   – Malignant transformation


• “Mollaret’s” meningitis

• Causes of Chronic / Recurrent Meningitis

• Chronic meningitis with ↑ Protein; ↓ Glucose in CSF
                       Epidermoid Cysts
• ~1% of all brain tumours

• Arise from epithelial cells retained
  during closure of the neural tube

• Grow though accumulation of lipid
  and keratin released by
  desquamating cells

• Commonly associated with cranial
  nerves & arteries, often around the
  basal cisterns
                Epidermoid Cysts
• Symptoms 2o to compression (ie. CNs, arteries,
  ventricles)

• Aseptic meningitis 2o to cyst rupture

• Diagnosis:
   – characteristic MRI appearance

• Management:
   – Surgical resection, may not be complete
       Malignant Transformation
• Rare
• Characterised by:
  – Rapid deterioration (median survival post dx 9mo)
  – No improvement or rapid recurrence of cyst tissue
    after resection
  – Leptomeningeal carcinomatosis
• Postulated that surgery might precipitate
  transformation, due to chronic inflammation
                    Mollaret’s Meningitis
•   Recurrent, benign, lymphocytic meningitis

•   First described by Mollaret in 1944

•   Characterised by:
     – 3 or more episodes of fever, meningism

     – Usually lasts 2 – 5 days      spontaneous
       recovery

     – Mollaret’s cells
          • Large, granular plasma cells
          • Present acutely (<24 hrs)

     - Elevated protein, normal glucose
                                                   Mollaret cell
                         Aetiology
– Viral
   •   HSV2: 70 – 85% - often following clinical herpes attack
   •   HSV1
   •   EBV
   •   West Nile
   •   Enteroviruses
– Other
   • Epidermoid cysts
        – Repeat cerebral imaging between attacks
   • Dermoid
  Chronic or Recurrent meningitis
1. Meningeal infections
  - eg. TB, HSV, fungal, parasitic
2. Malignancy
  - eg. Metastatic (breast, lung, melanoma, lymphoma)
3. Non-infectious inflammatory
  - eg. CNS sarcoidosis, SLE, Behçet’s syndrome
4. Chemical & drug-induced meningitis
  - eg. NSAIDs, FQ, sulphur drugs, foreign bodies
5. Para-meningeal infections
  - eg. Otitis media, pleuropulmonary infections
High Protein and Low glucose in CSF
                                                                    Cell
                                   Aetiology
                                                               predominance
                      • Bacterial esp MTB, brucella          • Mononuclear or mixed
Meningeal Infection   • Fungal – coccidioides, histoplasma   • Mixed
                      • Parasitic – cysticercosis            • Mono / Eosinophilic

                                                             • Mono or mixed
    Malignancy        • Metastatic disease
                                                             • Malignant cells

  Non-infectious
                      • Sarcoidosis                          • Mono
  inflammatory
                      • Epidermoid cyst                      • Mixed
     Chemical
                      • Recent CNS injection                 • Mixed
                      • Partially treated / suppressed
  Para-meningeal      suppurative infection                  • Mono or mixed
     infection
                      • Otitis media
                   What We Learnt
• Chronology of events crucial

• Long differential for chronic CNS inflammatory response
   – Many mimic CNS infection
   – Think about epidermoid cysts in DDx chronic/recurrent meningitis

• Think lateral!

• Encourage surgical units to think lateral too!
  (and to do as you say..)
        CMV and CNS infection
• Exceedingly rare cause of encephalitis in an
  immunocompetent host
• Series of 10:
  – All survived
  – 1 had sequelae at 6 mo
  – Various treaments
  (Eddleston CID 1997)

				
DOCUMENT INFO
Shared By:
Categories:
Tags:
Stats:
views:36
posted:11/14/2011
language:English
pages:40