NEUROOPHTHALMIC MANIFESTATIONS OF SYSTEMIC DISEASE KELLY A MALLOY

Neuro-Ophthalmic Manifestations of Systemic Disease

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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)
Normal values: Men: 14-18 g/dL Women: 12-16 g/dL

Platelet Count

Thrombocytosis: platelet count (>400x10⁹/L)

May precipitate thrombosis in SPCA, Linked with increased cerebral ischemic events

Severe thrombocytosis (> 600x10⁹) associated with risk of permanent visual loss
GCA related thrombocytosis returns to normal within 4 weeks of steroid tx

TREATMENT of GCA

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)

OCCULT GCA

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

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MYASTHENIA GRAVIS

Neuromuscular disorder, autoimmune attack , decrease of ACh receptors, weakness & fatigability of voluntary muscle

Peak incidence -Younger women (15-20), older men (50-60), can also occur in children

23% have an associated immunologic disorder (DM, thyroid, SLE, RA, increased malignancies)

60% initial presentation is ocular manifestation 90% develop eye signs, 15% develop only eye signs

MG WORK-UP

AChR ANTIBODY ASSAY(binding, blocking & modulating)
ANA TSH, T4 FBS CBC
Chest CT EMG (single fiber) Tensilon ?? (risk of respiratory failure)

PROGNOSIS OF MG IN 5 YEARS

40% stay ocular 40 - 55% convert to generalized 11% spontaneous remission
If convert to generalized, Most do so within 3 years

SYMPTOMATIC THERAPIES FOR MG

ACh esterase inhibitors

Mestinon (pyridostigmine), prostigmin (neostigmine), mytelase (ambenonium)

Immunotherapies

Anticytokine agents - corticosteroids, sandiummune (cyclosporin)
Cytotoxic: immuran (azathioprine)
Humoral therapy - plasmapheresis, intravenous gamma globulin

Surgical therapy - thymectomy

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OTHER AUTO-IMMUNE DISEASES

Can cause optic neuritis, optic neuropathy, cranial nerve palsies, etc.

Positive ANA

Order additional testing

Anti-ds DNA (for Lupus) SSA / SSB (for Sjogren’s, etc.)
p-ANCA / c-ANCA (for vasculitis / Wegner’s) RF

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LUPUS – SLE

90% women, more common in blacks + ANA IN 98% + ANTI ds-DNA IN 70%

Systemic symptoms – fatigue, malaise, fever, weight loss

Arthralgias, myalgias, arthritis, hair loss, rash, edema, kidney problems

Many other auto antibodies can be associated as well

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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

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LYME DISEASE

LYME TITER WESTERN BLOT IgG AND IgM STUDIES NEURO-LYME

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GRAVES’ DISEASE

Disorder of immune regulations, hyperthyroidism, Cytotoxicity directed at thyroid gland & EOM

Infiltrative orbitopathy, infiltrative dermopathy, Graves’ disease women 5:1 Graves’ orbitopathy women 3:2

PROPTOSIS - Rarely an isolated finding - Absence may mean posterior decompression

EYELID EDEMA - “Jelly roll” “finger-like” diurnal improvement

EYELID RETRACTION - Most reliable sign 50% graves’ disease 90% graves’ orbitopathy

RESTRICTED MOTILITY - 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

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HOMOCYSTEINE

Amino acid in the blood
Increased with vitamin B12 or folate deficiency, genetic causes or renal disease
Related to greater risk of cardiovascular disease, stroke, peripheral vascular disease
Elevated levels = atherosclerosis
Folic acid and vitamins B6 and B 12 break down homocysteine in the blood
A 3umol/L drop in homocysteine = 16% less chance of heart attack, 24% less chance of stroke, and 25% less chance of DVT
Levels above 14 umol increase risk of Alzheimer’s by 150%

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METHYLMALONIC ACID

Early indicator of (occult) vitamin B 12 deficiency, or renal insufficiency
Level is elevated when abnormal
Methylmalonyl co A ------ vit B12 -------succinyl coA

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DORSAL MIDBRAIN SYNDROME

Tectal pupils - mid-dilated, poorly reactive to light, better to near
Upgaze paresis (downgaze paresis, or both)
Convergence retraction nystagmus eyelid retraction

DMS (lesser signs)

CN IV palsy skew deviation pseudo CN VI palsy
Vergence problems square wave jerks downbeat nystagmus

DORSAL MIDBRAIN SYNDROME
(cause / location)

Pinealoma, tumors, mets, AVM, III ventricle, infarct, MS, syphilis, aqueductal stenosis, herniation

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SKEW DEVIATION

Vertical misalignment , higher eye is intorted, lower eye is extorted
May have head tilt &subjective visual vertical

SKEW DEVIATION LOCALIZATION

Vestibular nerve, vestibular nuclei, medulla, pons, midbrain

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INTERNUCLEAR OPHTHALMOPLEGIA

UNILATERAL (INO)

Vascular, older males
Associated skew (43%), upgaze nystagmus

BILATERAL (BINO)

Demyelinating, younger, males = females, progressive
Associated skew (13%), upgaze nystagmus

INTERNUCLEAR OPHTHALMOPLEGIA ETIOLOGY

Multiple sclerosis stroke
Hydrocephalus tuberculosis meningitis
Paraneoplastic encephalomyelitis HIV-CMV encephalitis
Head trauma SLE
Supratentorial avm, intracranial tumor migraine

Tags: disease ===================================================, vascular disease, malloy, systemic, neuroophthalmic, kelly, disease, manifestations

NEUROOPHTHALMIC MANIFESTATIONS OF SYSTEMIC DISEASE KELLY A MALLOY