Walsh & Hoyt: Neuronal Ceroid Lipofuscinoses

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Identifier wh_ch46_p2497
Title Walsh & Hoyt: Neuronal Ceroid Lipofuscinoses
Creator Michael X. Repka, MD
Subject Neurodegenerative Diseases; Metabolic Diseases; Infant; Child; Neuronal Ceroid Lipofuscinoses
Description Neuronal ceroid lipofuscinosis is characterized by brain and retinal atrophy with selective necrosis of neurons, leading to progressive mental and motor deterioration. Visual loss is an important symptom that is prominent among children and less so among adults. These disorders are characterized by the accumulation in the body of two lipopigments: lipofuscin and ceroid. Distinctive membranebound cytoplasmic inclusions have granular, curvilinear, or fingerprint patterns. The infantile Hagberg-Santovouri type, late-infantile Jansky-Bielschowsky type, juvenile Spielmeyer-Vogt form, and adult Kufs type were delineated on clinical grounds.
Date 2005
Language eng
Format application/pdf
Type Text
Source Walsh and Hoyt's Clinical Neuro-Ophthalmology, 6th Edition
Relation is Part of Walsh and Hoyt's Clinical Neuro-Ophthalmology Walsh and Hoyt's Clinical Neuro-Ophthalmology
Collection Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
Publisher Wolters Kluwer Health, Philadelphia
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890
Rights Management Copyright 2005. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6n04g2m
Setname ehsl_novel_whts
ID 186180
Reference URL https://collections.lib.utah.edu/ark:/87278/s6n04g2m
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