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					                     Neuro-Ophthalmic Disease Cases
                       Kelly A. Malloy, OD, FAAO, Diplomate

Clinical cases will be used to demonstrate the varied presentations related to neuro-ophthalmic
disease. These will include both afferent and efferent manifestations of neuro-ophthalmic
disease, as well as associated ocular health and neurologic manifestations. Conditions that may
be demonstrated through clinical cases are included in the outline below.

Course Learning Objectives:
    1. To emphasize the importance of the optometrist’s role in identifying signs and
       symptoms which suggest a neuro-ophthalmic disease process.
    2. To understand how to conduct an examination oriented to the detection of neuro-ophthalmic
       disease.
    3. To discern the differential diagnoses for a variety of clinical neuro-ophthalmic presentations.
    4. To become familiar with the necessary diagnostic testing for a variety of neuro-ophthalmic
       presentaions.
    5. To emphasize the need to promptly identify and refer patients presenting with
       emergent neuro-ophthalmic disease conditions.
    6. To have a better understanding of the work-up, management, and treatment of
       neuro-ophthalmic disease conditions.

                    Neuro – Ophthalmic Disease Cases - OUTLINE
Conditions that may be demonstrated through actual clinical cases are included in the outline
below.


                                        PAPILLEDEMA
Papilledema - Bilateral/Asymmetric (anatomic difference in lamina)      RARELY Unilateral
ICP (Intracranial pressure) greater than 200 - 250 mm H2O

Features of edema
Axoplasmic stasis in pre-laminar optic nerve
Obscuration of retinal vessels coursing over the disc margin
Paton’s lines temporally

Symptoms of Increased Intra-Cranial Pressure
Headache
Nausea
Vomiting
Diplopia (Abduction deficit – CN VI)
Pulsatile tinnitus
Transient Visual Obscurations (TVOs) Last few seconds (uni or bi-lateral) Transient optic nerve
ischemia

Pattern of Edema
Corresponds with NFL thickness
Superior, Inferior > Nasal > Temp
Superior and Inferior NFL swell first
Last to swell is Temporal NFL

NFL swelling blurs disc margins and obscures underlying vessels
Spontaneous Venous Pulsation
Presence of SVP means ICP normal (at that moment - can fluctuate)
10-20 % of normals may not have SVP


PSEUDOTUMOR CEREBRI IMPOSTERS
Tumor Cerebri
Anomalous Discs, Obesity, Migraine
Venous Sinus Thrombosis
Arteriovenous Malformation
Spinal Cord Tumors

PSEUDOTUMOR CEREBRI IS A SYNDROME BASED UPON:
MODIFIED DANDY’S DIAGNOSTIC CRITERIA
PATIENT MUST BE AWAKE & ALERT
SIGNS & SYMPTOMS OF INCREASED ICP
NO NEUROLOGIC SIGNS EXCEPT CN VI PARESIS
CSF OPENING PRESSURE > 200MM. H20 & NORMAL COMPOSITION
NORMAL MRI, CT

PSEUDOTUMOR CEREBRI - EPIDEMIOLOGY
92% Women
Ages 11-58
No Racial Bias
13/100,000 In Women - 10% Above Ideal Body Weight
19/100,000 - 20% Above Ideal Body Weight

PSEUDOTUMOR CEREBRI INVESTIGATIONS
MRI and MRV (MRA)
LUMBAR PUNCTURE with opening pressure and analysis of CSF

TREATMENT
WEIGHT LOSS (6%)

CARBONIC ANHYDRASE INHIBITORS
acetazolamide (diamox); up to 1000 mg.
reduces CSF by 50% but may be unsustained!
Contraindicated in renal disease

SURGICAL TREATMENT
Lumboperitoneal Shunt
Optic Nerve Sheath Fenestration
Gastric Bypass Surgery

PROGNOSIS
49% Have Some Visual Loss (Corbett 1982; Orcutt 1984)
25% Severe & Permanent Visual Loss (Folley 1955; Boddle 1974)
80% Improve In 8 Months (Corbett 1982)
10% Recurrence Rate
                             CRANIAL NERVE III PALSY
Hyper Deviation Which Increases In Upgaze And Reverses In Downgaze
Exo Which Increases Across From The Vertically Limited Eye

DEATH FROM SUBARACHNOID HEMORRHAGE (SAH) (LOCKSLEY 1969)
20%      Of Patients With SAH Die Within 48 Hours
need to consider aneurysm in all CN III palsy

ANSWER BY PUPIL
INVOLVED=ANEURYSM
SPARED=VASCULOPATHIC
DOES NOT APPLY IF:
              Complicated CNIII
              Incomplete CNIII
              Relative Sparing
              20-50 Years Of Age

ABERRANT REGENERATION OF CN III
Pseudo Graefe Sign
Eyelid Synkinesia
                                       •Light-Gaze Disassociation Pupils
ABERRANT REGENERATION OF CN III - CAUSES
COMMON CAUSES: Aneurysm, Tumor, Trauma
UNUSUAL: Inflammation
NEVER: Diabetes Mellitus

ISOLATED CN III PALSY IN ADULTS
Undetermined                   24%
Aneurysm                       21%
Ischemia                       18%
Trauma                         13%
Neoplasm                       12%


CN III PALSY Work-up ADULTS
20-50 YEARS
CT, MRI, MRA, Arteriogram
50+ YEARS (pupil, palsy, pain)
Neuroimaging
Vasculopathic Evaluation




                                        CNIV PALSY
Vertical Diplopia , Worse at Near, Object Tilting, Relief With Head Position
A Hypertropia That Increases Across From The Vertically Limited Eye And On Ipsilateral Head Tilt

MEASURING EXCYCLOTORSION
Subjective, Maddox Rod, Bagolini Striated Lenses, Fundus, Plot Blindspot

OBJECTIVE vs. SUBJECTIVE EXCYCLOTORSION
Objective = Subjective - Acute
Objective > Subjective - Longstanding
Objective Without Subjective - Congenital
“CHECKLIST” EXAMINATION
what to look for:                        anatomic localization:
CONTRA HORNER’S                                  LOCUS CERULEUS (Copetto 1983)
CONTRA INO                                      MLF (Vanooteghem 1992)
IPSI RAPD                                       BRACHIUM SUP COLL (Elliot 1991)
DMS                                             ANT MEDULLARY VELUM
BILATERAL CNIV                                  ANT MEDULLARY VELUM
TRUNCAL ATAXIA & IPSI DYSMETRIA                 SUP CEREBELLAR PEDUNCLE
IPSI CN III, V1, VI, OSP                        CAVERNOUS SINUS


BILATERAL CNIV
R HYPER> IN L GAZE & RHT, L HYPER> IN R GAZE & LHT
V PATTERN ESO > 25 PD, EXCYCLOTORSION > 10 deg
(LOOK FOR THE DORSAL MIDBRAIN SYNDROME)


ISOLATED CN IV IN KIDS (CAUSE)
Trauma, Congenital

ISOLATED CN IV IN ADULTS
10% Aneurysm, 20% Ischemic, 30% Undetermined, 40% Trauma

TRAUMATIC CNIV PROGNOSIS (SYDNOR 1982)
SPONTANEOUS RECOVERY:
                    UNILATERAL:                  65%
                    BILATERAL:                   25%

WHAT DO I DO?
History Of Trauma?, Old Photos, Vertical Vergences, Neuro-Imaging, Vasculopathic Eval, MG?,
Graves?, Skew?

CNIV: MANAGEMENT?
Segmental Prisms & Patches X 9-12 Months
Monitor For Secondary Contracture - Surgical Intervention
_______________________________________________________________________

                      ABDUCTION DEFICIT/ CN VI PALSY
F ABDUCTION DEFICIT
CN VI PALSY KEY POINTS - Measure At Distance
Imposters – Abduction Deficits
Graves Disease / Thyroid Orbitopathy, Myasthenia Gravis, Loss Of Fusional Reserves, Spasm Of The
Near Reflex, Duane’s Retraction Syndrome

“THE SIGNATURE OF CN VI PARESIS”
At Distance: Eso Which Increases In The Action Of The Palsied Eye

CN VI PALSY - ANSWER BY MOTILITY
Duction > Version, “Glissades” (Slowed Saccades), Asymmetric OKN (Optokinetic Nystagmus),
Negative Forced Duction
ANATOMIC LOCALIZATION
FASICULAR
CN VI +
Contralateral Hemiplegia = (Raymond’s)
VII + Contralateral Hemiplegia = (Millard-Gubler)
Etiology: Infarction, Demyelination, Tumor

SUBARACHNOID
Increased Intracranial Pressure (Papilledema), AICA Aneurysm, Subarachnoid Hemorrhage, Trauma,
Meningitis, Clivus Tumor, Post Infectious, Neurosurgical

PETROUS
Petrous Apex/ Mastoid Infection, Inferior Petrosal Sinus Infection, Petrous Bone Fracture, Trigeminal
Schwannoma, Lumbar Puncture, Myelography, Spinal/ Epidural Anesthesia

CAVERNOUS SINUS/ SOF
Aneurysm, Thrombosis, CCF (Carotid Cavernous Fistula), Dural AVM (Arterio-venous Malformation),
Tumor, Tolossa-Hunt, Herpes Zoster

WORK-UP?
CBC (Complete Blood Count), BS (Blood Sugar) / HEMOGLOBIN A1c, LYME TITER, RPR/ FTA-ABS
(tests for syphilis), ANA (Anti-Nuclear Antibody), ESR (Erythrocyte Sedimentation Rate), C-REACTIVE
PROTEIN, PLATELETS, & HEMOGLOBIN (tests for Giant Cell Arteritis), Exclude Trauma, MRI With
Gadolinium, Lumbar Puncture

CHRONIC CNVI PALSY (= OR > 6 months) – Harbingers of Serious Intracranial Disease

_______________________________________________________
                                  HORNER SYNDROME
MIOSIS, PTOSIS, ANHYDROSIS
Anisocoria > Dim “Lazy Dilator” (Dilation Lag) Anisocoria > 5 Sec Than 12 Sec
Measure in bright AND dim - Greater anisocoria in dim illumination

Pancoast’s Tumor (Apical lung tumor)
TRIAD: Ptosis, Miosis, Arm Pain

CAROTID ARTERY DISSECTION
CLASSIC TRIAD: Pain On Side Of Face, Head Or Neck, Oculosympathetic Paresis Without Anhydrosis,
Delayed Retinal Or Cerebral Ischemia (50-95% Of Patients)
SYMPTOMS
Exploding, Ipsilat Headache
Transient Monocular Blindness
Diplopia
Orbital, Facial, Neck, Jaw Pain
Dysguesia
Facial Numbness
Neck Swelling

SIGNS
Horner’s Syndrome
Neck Bruit Or Swelling
CN VI, IX-XII
CRAO
Cerebral Ischemia
Need to consider CAROTID DISSECTION in EVERY PAINFUL HORNER SYNDROME
Can occur with or without trauma
Medical Emergency
Horner’ s with eye, head, neck pain - Pt to hospital (MRI, MRA, CTA, angiogram)

____________________________________________________________________________
                                        OPTIC NEURITIS
Most common acute monocular vision loss in young and middle-aged
3rd and 4th decades, Females
Association with Multiple Sclerosis

Typical ON
Unilateral
Painful (with eye movements)
Visual loss progressing over one week
Young / middle age adult
Normal fundus or minimal ON edema

Atypical ON
NLP vision
Optic disc or retinal hemes
Severe disc swelling
Macular exudates
No pain
Uveitis
Bilateral vision loss (adults)

DDX of Optic Neuritis
Inflammatory / Autoimmunine Diseasesn ( SLE, Antiphospholipid Syndrome, Primary Sjogren’s
Syndrome, Neurosarcoidosis, Neuro-Bechet’s Disease, Wegener’s Granulomatosis)
Infectious Etiologies ( Lyme , Neurosyphilis, HIV-related disorders of the CNS)
Genetic / Hereditary Disorders
Demyelinating Disorders

50% of MS pts develop ON at some point
ON is 1st clinical symptom of MS in 20%

Work-Up
lab testing to R/O other etiologies
ACE, ANA, Lyme titer, FTA-ABS, RPR
MRI of brain and orbits with contrast
Spinal tap ?

Recommended ON Tx
IV Methylprednisolone
250 mg q 6 hr x 3 days
Oral prednisone taper
1 mg/kg/day x 11 days, 4 day taper       [20 mg day 1; 10 mg days 2 and 4]


MS treatments
High-risk
ON and 2 or more lesions on MRI
Immunomodulators
Copaxone
Interferons
Interferon beta-1a (Avonex or Rebif)
Interferon beta-1b (Betaseron)
Lower risk of further demyelinating events
Reduce MRI activity

OPTIC NEURITIS (CLINICAL PROFILE)
F (77.2%)      M (22.8%)
CAUCASION (85%) > BLACK (15%)
AGES 15-40 YEARS (MEDIAN 31.8)
92.2% PAIN!
VISUAL NADIR: 4.7 DAYS
RECOVERY: 2-3 MONTHS
FELLOW EYE: 20% (68.8%, 33.2%, 31.4%)
IDIOPATHIC OPTIC NEURITIS SYMPTOMS
92.2% PAINFUL, BLURRED VISION
DISCOMFORT ON EYE MOVEMENT

IDIOPATHIC OPTIC NEURITIS FUNDUS EXAM
RETROBULBAR OPTIC NEURITIS 65%
PAPILLITIS 35%

IDIOPATHIC OPTIC NEURITIS VISUAL RECOVERY
RECOVERY BEGINS WITHIN 3 WEEKS OF ONSET OF SYMPTOMS
PLATEAU’S @ 6 WEEKS; IMPROVES UP TO 1 YEAR
@ 5 YEARS: 87%: > 20/25 94%:> 20/40
VISUAL RECOVERY IS WORSE IN PATIENTS WITH FC/LP
RECURRING OF 20% IN 5 YEARS

ONTT(Beck 1992)
Optic Neuritis= MS (Relapsing/ remitting)
DxMS: Clinical Diagnosis + MRI findings
Oral steroids double risk of recurrence (30%)
Treatment affects course of disease (IV sol medrol halves risk of CDMS at 2 years in patients with +
MRI). IV steroids speed visual recovery
MRI can predict CDMS

ONTT TREATMENT RECOMMENTATIONS FOR IDIOPATHIC OPTIC NEURITIS
8 DAYS OF ONSET
MRI SHOULD BE PERFORMED
ASSESS CRITICAL NUMBER OF WHITE MATTER LESIONS
IV METHYLPREDNISOLONE X 3 DAYS (short course of prednisone)

ONTT – 10 year results
Overall risk of MS in 10 yrs = 38% CDMS
One or more brain lesions = 56% CDMS
> 40% with one or more lesions did NOT develop CDMS in 10 yrs
No brain lesions = 22% CDMS
78% with no lesions did NOT develop CDMS in 10 yrs

Risk significantly higher with 1 lesion
No increased risk with greater number of lesions

Mean time to DX = 3 years
Almost 75% of Dx occurred in 5 years
_______________________________________________________________________
                                  GIANT CELL ARTERITIS
ELDERLY - Mean age :72 years
Consider in patients over age 50

Preference for Caucasians (2/3)

Preference for women (2/3)
27% men
73% women

Attacks medium and large sized arteries
Specifically targets arteries with ILM
Elastin likely is inciting antigen (auto-immune)

Thrombosis and Occlusion

SYSTEMIC SYMPTOMS OF GCA
Fever , Headache, Anorexia/Weight Loss, Malaise, Myalgia


GCA SYMPTOMS
(Keltner 1982)
HEADACHE                                   4-100%
TENDER TEMPORAL ARTERY                     28-91%
MYALGIA/ ARTHRALGIA                        28-86%
FEVER                                      30-100%
WEIGHT LOSS                                16-76%
JAW CLAUDICATION                           4-67%
ANOREXIA                                   14-69%
MALAISE                                    12-97%
LEG                                        2-43%


OCCULT GCA
Ocular involvement
No systemic symptoms / signs
21% of GCA cases
Have LOWER values of ESR and CRP
May be a more localized disease process

OCULAR INVOLVEMENT
50% of GCA pts. present with ocular involvement
Characteristics of pts with ocular involvement
OLDER, LOWER ESR (&CRP)
LESS SYSTEMIC SYMPTOMS

VISUAL LOSS
The fellow eye can be affected (28-31%):
Simultaneously
Within days to weeks
BILATERAL ischemic visual loss – CONSIDER GCA!

AMAUROSIS FUGAX
Transient monocular blindness
Most often caused by ON ischemia, can be light-induced
Present in 31-46% of GCA pts.
Precedes permanent visual loss 50-63% of time
Prodrome to AAION


OCULAR SIGNS OF GCA
Arteritic Anterior Ischemic Optic Neuropathy (AAION) (81%)
Central Retinal Artery Occlusion (9 - 14%)
Cilioretinal Artery Occlusion
Posterior Ischemic Optic Neuropathy
Ocular Ischemia (CWS, Choroidal Hypoperfusion, Chorioretinal Degen.)
Ocular Motility Restrictions / Cranial Neuropathies
Nystagmus / Internuclear Ophthalmoplegia


AAION
81% of GCA pts
Most common cause of severe vision loss in GCA
Poor visual prognosis
Fellow eye commonly involved (65% if untreated)
Simultaneously or within weeks (usually)
More severe visual dysfunction than N-AION (usually!)
Extremely poor visual prognosis

“Chalky white” disk swelling
Cupping & parapapillary atrophy (Hayreh et al.2001)


LAB TESTING / WORK-UP
Westergren Sedimentation Rate (ESR)
C-Reactive Protein (CRP)
Platelet count
CBC c differential

Erythrocyte Sedimentation Rate Westergren / STAT
A normal ESR does NOT R/O GCA
Normal in 12-13% of GCA patients

NON-SPECIFIC FOR GCA
Indicator of inflammatory and neoplastic disease
Non-specific marker of an acute phase reaction

        ESR High Normal Cut-Off
        MEN: Age / 2     WOMEN:{Age + 10} / 2

C-Reactive Protein
Immunoassay
Normal Value: <2.54 mg/dL
Elevated in GCA
One of major Acute Phase Reactants
Independent risk factor for coronary artery disease
Returns to normal more rapidly than ESR with resolution of disease process
Elevated CRP and ESR : 97% specific for GCA

CBC with Differential
Anemia associated with GCA (normochromic, normocytic)
Platelet Count
Thrombocytosis: platelet count (>400x10 9/L)
Marker of systemic inflammatory response
Linked with increased cerebral ischemic events

Severe thrombocytosis (> 600x109)
 associated with risk of permanent visual loss

If suspicion remains, get GSTA Biopsy!

BIOPSY OF Greater Superficial Temporal Artery
PERFORMED WITHIN 14 days
SKIP LESIONS in 28 % (17/60), BIOPSY 3-7 mm. SEGMENT


DO NOT WAIT UNTIL AFTER BIOPSY TO TREAT
Try to perform biopsy within 2 weeks of treatment

TEMPORAL ARTERY BIOPSY
2cm sample (preferably more)
Ipsilateral side of any ocular invovement
Skip Lesions (4 - 5% false negatives)

TREATMENT
Visual Involvement: PROTECT OTHER EYE
STAT IV Methylprednisolone
(Solumedrol 250mg IV q 6 hrs x 3 days)
Followed by Oral Prednisone

No Visual Involvement: PROTECT VISION
Oral Prednisone (60-80 mg daily)

PROGNOSIS
UNTREATED: 30-40% BILATERAL BLIND
(highest risk of vision loss within 10 days)
5-10% GO BLIND DESPITE TREATMENT
15-34% HAVE SLIGHT IMPROVEMENT WITH TREATMENT
26% RELAPSE RATE

ANY NEW-ONSET NEUROLOGIC DEFICIT IN A PATIENT OVER 50 YRS. SHOULD BE INVESTIGATED FOR
GCA!
______________________________________________________________________________________

                                 MYASTHENIA GRAVIS
NEUROMUSCULAR DISORDER
WEAKNESS & FATIGABILITY OF VOLUNTARY MUSCLE
DECREASE OF Ach RECEPTORS
AUTOIMMUNE ATTACK

PEAK INCIDENCE
YOUNGER WOMEN (15-20)
OLDER MEN (50-60)
OVERALL F:M 2:1   UNDER 3O: F:M 4.5:1
23% HAVE AN ASSOCIATED IMMUNOLOGIC DISORDER

60% INITIAL PRESENTING SIGN IS AN OCULAR MANIFESTATION
90% WILL DEVELOP EYE SIGNS
15% WILL DEVELOP ONLY EYE SIGNS

MG WORK-UP
AChR ANTIBODY ASSAY (binding, blocking & modulating)
MuSK Antibody
TSH, T4, T3, thyroid antibodies (to r/o associated thyroid dysfunction)

EMG (SINGLE FIBER)
CHEST CT (to r/o thymoma in MG)

PROGNOSIS IN 5 YEARS
40% STAY OCULAR
40% CONVERT TO GENERALIZED
11% SPONTANEOUS REMISSION
85% CONVERT TO GENERALIZED

SYMPTOMATIC THERAPIES
ACh ESTERASE INHIBITORS (MESTINON (pyridostigmine) / PROSTIGMIN (neostigmine))
IMMUNOTHERAPIES
ANTICYTOKINE AGENTS
CORTICOSTEROIDS, CYCLOSPORIN
CYTOTOXIC: IMMURAN (azathioprine)
HUMORAL THERAPY
PLASMAPHERESIS, INTRAVENOUS GAMMA GLOBULIN

SURGICAL THERAPY
THYMECTOMY

DRUGS TO AVOID IN MG
IODINATED CONTRAST AGENTS
CALCIUM CHANNEL BLOCKERS
BETA-BLOCKERS: PROPRANOLOL, TIMOPTIC
NEUROMUSCULAR BLOCKING AGENTS
SUCCINLYCHOLINE, VECURONIUM
QUININE, QUINIDINE, PROCAINAMIDE
SELECTED ANTIBIOTICS
AMINOGLYCOSIDES, CIPROFLOXACIN

__________________________________________________________________________


                                 METASTATIC DISEASE
PRIMARY CANCERS CAN METASTASIZE TO BRAIN OR BONE
LEADING TO NEURO-OPHTHALMIC DISEASE MANIFESTATIONS
CRANIAL NERVE VI PALSY
HORNER SYNDROME
DORSAL MIDBRAIN SYNDROME
OPTIC NEUROPATHY

METS TO BONE (clivus, anterior skullbase)
prostate and breast cancers
METS TO CNS
kidney, lung, stomach and thyroid cancers

DORSAL MIDBRAIN SYNDROME
symptoms
upgaze paresis (sometimes downgaze)
eyelid retraction
convergence retraction nystagmus
skew deviation

_____________________________________________________________________________


                                    NEURO-SARCOID
10-20 x MORE PREVALENT IN African Americans & Scandinavians
OCULAR INVOLVEMENT: 22%
NEUROSARCOIDOSIS: 5%

FEATURES OF ANY OPTIC NEUROPATHY
OPTIC DISC PALLOR
DECREASED COLOR VISION (DYSCHROMATOPSIA)
VISUAL FIELD LOSS
RELATIVE AFFERENT PUPILLARY DEFECT

SARCOID OPTIC NEUROPATHY SYSTEMIC SIGNS
LYMPHADENOPATHY
ERYTHEMA NODOSUM, SKIN NODULES, MACULOPAPULAR RASH, LUPUS PERNIO
NASOPHARNGITIS
HEPATOMEGALY, SPENOMEGALY, PORTAL HYPERTENSION, BONE CYSTS, POLYARTHRALGIAS

SARCOID OPTIC NEUROPATHY LABORATORY
NORMOCHROMIC, NORMOCYTIC ANEMIA
ELEVATED ESR
SERUM ACE (+66%)
HYPERCALCEMIA (+18%); HYPERCALCURIA
ELEVATED SERUM LYSOZYME
LIVER FUNCTION TESTS (+SERUM ALKALINE PHOSPHATASE)
CSF: NORMAL OR PLEOCYTOSIS

SARCOID OPTIC NEUROPATHY INVESTIGATIONS
CHEST X-ray, CT
GALIUM SCAN
LUMBAR PUNCTURE
PULMONARY FUNCTION STUDIES
BRONCOALVEOLAR LAVAGE
FIBEROPTIC BRONCHOSCOPY WITH TRANSBRONCHIAL BIOPSY
BIOPSY (conjuntiva, lung, skin, lymph node, optic nerve sheath)

SARCOID OPTIC NEUROPATHY TREATMENT & PROGNOSIS
CORTICOSTEROIDS
PREDNISONE
IMMUNOSUPPRESSANTS
CYCLOSPORIN
METHOTREXATE
50% SPONTANEOUSLY REMIT
_____________________________________________________________________________


                        OTHER AUTO-IMMUNE DISEASES
LUPUS – SLE
90% WOMEN
MORE COMMON IN BLACKS
SYSTEMIC SYMPTOMS – FATIGUE, MALAISE, FEVER, WEIGHT LOSS
ARHTRALGIAS, MYALGIAS, ARTHRITIS
RASH
HAIR LOSS
EDEMA
KIDNEY PROBLEMS

+ ANA IN 98%
+ ANTI ds-DNA IN 70%
MANY OTHER AUTO ANTIBODIES CAN BE ASSOCIATED AS WELL


SJOGREN’S SYNDROME
MAINLY WOMEN
MAINLY MIDDLE-AGED, BUT CAN ALSO OCCUR IN CHILDHOOD
MAY BE ASSOCIATED WITH OTHER AUTOIMMUNE DISEASES
DIMINSIHED LACRIMATION AND SALIVATION
ARTHRALGIAS / ARTHRITIS
CAN BE ASSOCIATED WITH LYMPHOMA, MENINGITIS, AND MULTIPLE SCLEROSIS
Has been associated with optic neuropathy
Lab Tests: ANA, SSA & SSB Antibodies
_____________________________________________________________________________

                                    LYME DISEASE
LYME TITER
WESTERN BLOT IgG AND IgM STUDIES
NEURO-LYME – Optic neuropathy, Diplopia, Disc edema
_____________________________________________________________________________

                                 CAT-SCRATCH DISEASE
BARTONELLA TITERS (Henselae and Quintana)
Transmitted by a cat (typically a kitten)
Usually occurs in patient’s under age 20

Disc Edema
Neuro-retinitis – macular star

Treatment: Antibiotics – Rifampin, Azithromycin, Bactrim
____________________________________________________________________________


                                   GRAVES’ DISEASE
DISORDER OF IMMUNE REGULATIONS
CYTOTOXICITY DIRECTED AT THYROID GLAND & EOM
HYPERTHYROIDISM
INFILTRATIVE ORBITOPATHY
INFILTRATIVE DERMOPATHY

GRAVES’ DISEASE - WOMEN 5:1
GRAVES’ ORBITOPATHY - WOMEN 3:2

GRAVES’ = INFLAMMED ORBIT
TUMOR (exophthalmos; eyelid edema)
LOSS OF FUNCTION (eyelid retraction; motility defect; optic neuropathy)
REDNESS

PROPTOSIS
RARELY AN ISOLATED FINDING
ABSENCE MAY MEAN POSTERIOR DECOMPRESSION

EYELID EDEMA
“JELLY ROLL”
“FINGER-LIKE”
DIURNAL IMPROVEMENT

LOSS OF FUNCTION (upper eyelid retraction)
MOST RELIABLE SIGN
       50% GRAVES’ DISEASE
       90% GRAVES’ ORBITOPATHY

EYELID LAG = UNRELIABLE

LOSS OF FUNCTION (restricted motility defect)
INFERIOR RECTUS                60-70%
MEDIAL RECTUS                  25%
SUPERIOR RECTUS                10%
DIURNAL VARIATION
GAZE INDUCED IOP RISE

Thyroid Work-Up
TSH
T3
T4
Thyroid Stimulating Immunoglobulin
Thyroperoxidase Antibody
Thyroglobulin Antibody


GRAVE’S MANAGEMENT
PRISMS & PATCHES
DROPS & OINTMENTS
ELEVATED HEAD POSITION
STOP SMOKING !!!
STEROIDS
IMMUNOSUPPRESSIVES
RADIATION THERAPY ??
ORBITAL DECOMPRESSION
EXTRAOCULAR MUSCLE SURGERY
EYELID MARGIN REPOSITIONING
BLEPHAROPLASTY
__________________________________________________
          ARNOLD-CHIARI MALFORMATION
Herniation of cerebellar tonsils through the foramen magnum

SYMPTOMS:
Headache
Neck pain
Oscillopsia
Diplopia
Ataxia
Weakness

SIGNS:
Down-beat nystagmus, especially in lateral gazes
Ocular misalignment
Ataxia

WORK-UP:
Neuro-imaging, preferably mid-sagital MRI

Treatment:
Neuro-surgical decompression if warranted due to symptoms

				
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