52. Downbeat Nystagmus & the Hereditary Ataxias

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Identifier 52_Downbeat_Nystagmus_and_the_Hereditary_Ataxias.mp3
Title 52. Downbeat Nystagmus & the Hereditary Ataxias
Creator J. Lawton Smith, Professor Emeritus of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine
Subject Downbeat Nystagmus; Hereditary Ataxias; vertical nystagmus; tremor; gait disturbance; gait ataxia; progressive or familial spinocerebellar ataxia; olivocerebellar degeneration; cystoid macular edema; spastic gait; degenerative retinitis pigmentosa; Ataxia telangiectasia; Frederick's ataxia; optic atrophy; foramen magnum lesion; bilateral 4th nerve palsy; hypertropia; vertical oscillopsia; optic dysmetria
Clinical 1. Vertical downbeating nystagmus is a hallmark of foramen magnum lesion.<br><br> 2. Cogan drop test - a test for corneal sensation and thus first division of 5th cranial function.<br><br> 3. Ataxia telangiectasia. On eye examination one can see telangiectasia in lower tarsal region, fornix, ears and nose. The patient may have an ocular motility dysfunction including pseudo ophthalmoplegia, pseudo progressive supranuclear palsy, and one-and-a-half syndrome. These patients may have macular edema or degenerative retinitis pigmentosa.<br><br> 4. Frederick???s ataxia: It typically effects children under 20 years of age who may have pes cavus deformity, dysarthria, nystagmus, kyphosis, scoliosis, ataxia, and depressed knee and ankle jerk, may have cardiomyopathy and optic atrophy with or without retinitis pigmentosa.
Neuroimaging NA
Anatomy Pons; medulla; foramen magnum; posterior fossa
Pathology Posterior fossa lesion, foramen magnum lesion, purkinje cell abnormality in cerebellum.
Disease/Diagnosis Familial or progressive spinocerebellar ataxia
Relation is Part of J. Lawton Smith Collection
Contributor Primary J. Lawton Smith, Professor Emeritus of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1975-08-18
Type Sound
Format audio/mpeg
Format Creation Filesize: 101,211,599 bytes; Play length: 54:10; Jeremy Smith, audio technician; Tandberg TB15 Reel-to-reel deck; 16 bit mono; Captured using SAWstudio; Encoded using LAME; Windows 2000
Source 1/4" reel-to-reel audio tape
Rights Management Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
Holding Institution North American Neuro-Ophthalmology Association. NANOS Executive Office 324 North Main Street West Hartford, CT 06117-2507
Collection Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
Language eng
ARK ark:/87278/s64x59gz
Setname ehsl_novel_jls
Date Created 2006-08-08
Date Modified 2017-02-14
ID 180236
Reference URL https://collections.lib.utah.edu/ark:/87278/s64x59gz
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