Eye and b develop fr neuro-ect. Their func. to disease. Blood ocular/bra. The eye is The eye is window in

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2 The Patient wit th Visual Loss: Localization of Neuropathologic Disease and Select Diseases of Neuropathologic Interest Steven A. Kan e, MD M.D., PhD Ph.D. The Edward S. Harkness Eye Institute

3 The eye is The eye is window in Shared em mbryology Eye and b develop fr neuro-ect Their func and respo to disease related Blood ocular/bra barriers

4 Localization and characterizatio on of impaired vis ion Pattern of visual lo oss may identify the lesion site Disease course and accompanying symptoms may cla arify its nature

5 ssessment of visual functionn Central vision Acuity Color vision Peripheral vision Visual fields/perimetry i

6 Perimetry

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8 Ocular anatomy Unique exam of structure supporting function Optics Neurotransduction Neurotransmission

9 Bruckner reflex

10 cataract

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12 Retinoblastoma Most common intraocular malignancy in childhood Leukocoria and strabismus 13 q14 mutation ti Direct spread Localized chemotherapy

13 Normal ocular fundus Optic disc Retinal vess Transparent retina Macula Retinal pigm epithelium

14 Retinal nerve fiber layer anat tomy Axons of the optic nerve Papillomacular bundle Branching axons respect the horizontal raphe

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16 Symptoms and signs of optic nerve disease Blurred vision Dimming of vision with decreased color perception Decreased pupillary response to light Centrocecal and arcuate scotomata

17 TB

18 Centrocecal scotomas

19 Bilateral optic atrophy with cent trocoecal scoto oma Hereditary (dominan nt, Leber s) Toxic (medications, metals) methanol, heavy Nutritional (folate, B12) Demyelinating (optic neuritis, multiple sclerosis)

20 acuity and high right eye pressure

21 Glaucoma Common, usually bila ateral, often asymmetric optic neuropathy Initial selective damage to branching axons w sparing of papillomacular bundle = arcuate fie loss

22 Unilateral optic nerve disor rders Ischemic (anterior ischemic optic neuropathy, retinal occlusive disease) Compressive (orbital, anterior fossa) Inflammatory (demyelinating, infectious, rheumatologic)

23 udden painless visual loss with pallid disc swelling

24 Ischemic optic neuropathy Patients usually > 50 Sudden, usually stable visual loss Altitudinal scotoma Optic atrophy in 4-6 wk Causes Idiopathic (anatomic)

25 ant cell arteritis Brain, cardiac, eye, skin, mporal An ophthalmic arteritis s) emergency Senior citizens Subacute, granulomatous, stenosing arterial disease Headache, amaurosis fugax, arthralgia, myalgia, weight loss

26 Compressive optic neuro opathy Insidious visual loss Affects acuity, color, peripheral vision Gradual optic atrophy

27 Subacute Visual Loss Childhood Adult

28 Inflammatory optic Children and younger adults Centrocecal, arcuate, and hemianopic scotomas Subacute, often pain with eye movements neuro opathy Retrobulbar neuritis or papillitis

29 Obese 12 years old with blurred vision and headache

30 Papilledema versus other disc swelling Intracranial mass Pseudotumor cerebri Hydrocephalus Intracranial hemorrhage Venous thrombosis

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32 anatomy

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34 Lesions of Usually compressive the chiasm Pediatric Hypothalamic glioma Craniopharyngioma Adult Pituitary adenoma Meningioma Craniopharyngioma

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36 etrochiasmal lesions Hemianopic scotoma Grossly incongruous field defects Small afferent defect Children: neoplasm > vascular > trauma Adults: vascular > neoplasm > trauma

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38 manifestations of systemic diseases 10 year old unable to walk because of 1 year of upper and lower extremity weakness Slurred speech and drooling for 3 years, Emotionally labile, hypertonic Ataxia, Babinski reflex, intention tremor, palpable liver, slow wed saccades, blepharospasm

39 mcg/dl

40 Cherry red spots Tay Sachs & Sandoff s Niemann Pick type A Metachromatic leukodystrophy Sialidosis Farber disease Central retinal artery obstruction

41 Retinal degeneration retinitis pigmentosa hotoreceptor and/or tinal pigment pithelium disturbance igment released from amaged RPE cells lumps or migrates to the retina any causes

42 Diseases with retinal degene eration Mucopolysaccharid a doses, Gaucher s, Refsum, Neuronal ceroid lipofuscinosis, cystinuria i Abetalipoproteinem t i mia, Kearns-Sayre Hallervorden Spatz z, Spinocerebellar ataxias Usher, Cockayne

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44 diseases with optic atrophy Krabbe, Metachrom Adrenoleukodystro Spinocerebellar ata Friedreich s ataxia matic leukodystrophy y ophy, Alexander axia type I, Canavan s, Pelizaeus-Merzbacher, Alper s

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