Microsoft word - neuro-ophthalmic disease cases.doc

Neuro-Ophthalmic Disease Cases

● The content of this COPE Accredited CE activity was prepared independently by Kelly A. Malloy without
input from members of the optometric community.
● Kelly A. Malloy has no direct financial or proprietary interest in any companies, products or services
mention in this presentation.
●The content and format of this course is presented without commercial bias and does not claim
superiority of any commercial product or service.

Course Description: 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 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 5. To emphasize the need to promptly identify and refer patients presenting with emergent 6. To have a better understanding of the work-up, management, and treatment of neuro- Neuro – Ophthalmic Disease Cases - OUTLINE
Conditions that may be demonstrated through actual clinical cases are included in the outline below. 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 Meningitis 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 (6-10%) 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 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 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 GR TUMOR (exophthalmos; eyelid edema) LOSS OF FUNCTION (eyelid retraction; motility defect; optic neuropathy) REDNESS PRO RARELY AN ISOLATED FINDING ABSENCE MAY MEAN POSTERIOR DECOMPRESSION EYE “JELLY ROLL” “FINGER-LIKE” DIURNAL IMPROVEMENT LO DIURNAL VARIATION GAZE INDUCED IOP RISE Thy TSH T3 T4 Thyroid Stimulating Immunoglobulin Thyroperoxidase Antibody Thyroglobulin Antibody BLEPHAROPLASTY _______________________________________________________________________ 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 __________________________________________________________________________ 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 OPTIC Neuruitis 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 Interferon beta-1a (Avonex or Rebif) Interferon beta-1b (Betaseron) Lower risk of further demyelinating events Reduce MRI activity New Oral MS Medication – Fingolimod (may cause macular edema) OPTIC NEURITIS (CLINICAL PROFILE) F (77.2%) IDIOPATHIC OPTIC NEURITIS FUNDUS EXAM RETROBULBAR OPTIC NEURITIS 65% 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 ______________________________________________________________ Neuromyelitis optica is, like Multiple Sclerosis an inflammatory, demyelinating syndrome of the Central Nervous System Whereas MS affects the entire CNS (optic nerve , Brain, spinal cord), NMO preferentially affects the optic nerve and spinal cord. Within 5 years of disease onset, more than 50% of patients with relapsing neuromyelitis optica are blind in one or both eyes or require ambulatory help. Typical features are optic neuritis and myelitis (muscle weakness, sensory dysfunction, bladder dysfunction) Serious neurogenic respiratory failure can occur (not common in MS) Work-up: MRI of brain and spine neuromyelitis optica immunoglobulin G (NMO-IgG) an autoantibody, in the serum of patients with neuromyelitis optica, distinguishes neuromyelitis optica from other demyelinating disorders the main channel that regulates water homoeostasis in the CNS NMO –Ig G binds to aquaporin-4 channels on astrocytes, and is toxic, leading to demyelination This process is different than what happens in MS _____________________________________________________________________________ 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% MANY OTHER AUTO ANTIBODIES CAN BE ASSOCIATED AS WELL 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 TITER WESTERN BLOT IgG AND IgM STUDIES NEURO-LYME – Optic neuropathy, Diplopia, Disc edema 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 SA LYMPHADENOPATHY ERYTHEMA NODOSUM, SKIN NODULES, MACULOPAPULAR RASH, LUPUS PERNIO NASOPHARNGITIS HEPATOMEGALY, SPENOMEGALY, PORTAL HYPERTENSION, BONE CYSTS, POLYARTHRALGIAS SA ELEVATED SERUM LYSOZYME LIVER FUNCTION TESTS (+SERUM ALKALINE PHOSPHATASE) CSF: NORMAL OR PLEOCYTOSIS SA BRONCOALVEOLAR LAVAGE FIBEROPTIC BRONCHOSCOPY WITH TRANSBRONCHIAL BIOPSY BIOPSY (conjuntiva, lung, skin, lymph node, optic nerve sheath) SA _____________________________________________________________________________ BARTONELLA TITERS (Henselae and Quintana) Transmitted by a cat (typically a kitten) can be transmitted by scratch or even a lick Usually occurs in patient’s under age 20 Disc Edema – occurs prior to development of the macular star Neuro-retinitis – macular star Treatment: Antibiotics – Rifampin, Azithromycin, Bactrim

Source: http://deoa.wildapricot.org/Resources/Documents/Neuro-Ophthalmic%20Disease%20Cases.pdf