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: Walsh and Hoyt Textbook Selections Collection: https://NOVEL.utah.edu |
Publisher |
Wolters Kluwer Health, Philadelphia |
Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
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 |