Document Detail

The adult and a new late adult forms of neuronal ceroid lipofuscinosis.
MedLine Citation:
PMID:  1621503     Owner:  NLM     Status:  MEDLINE    
Three cases of the late adult form of neuronal ceroid lipofuscinosis (NCL) are reported. Two of these are siblings with a late clinical onset at ages 26 and 44 years. The third case, sporadic, has the oldest reported age for the onset of NCL, at 63 years and may be regarded as the first example of the "presenile" form of NCL. The clinical, morphological, histochemical, ultrastructural and genetic features of these three cases are discussed. The literature of the clinicopathological NCL cases with an onset at age of 25 and older is reviewed. The clinical and morphological differences between the late adult form and the presenile form of NCL as well as the difficulties in making the diagnosis are discussed.
J Constantinidis; K E Wisniewski; T M Wisniewski
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Acta neuropathologica     Volume:  83     ISSN:  0001-6322     ISO Abbreviation:  Acta Neuropathol.     Publication Date:  1992  
Date Detail:
Created Date:  1992-08-05     Completed Date:  1992-08-05     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0412041     Medline TA:  Acta Neuropathol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  461-8     Citation Subset:  IM    
Geneva University, Research Department of Morphological Psychopathology, Switzerland.
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MeSH Terms
Brain / pathology
Microscopy, Electron
Middle Aged
Neuronal Ceroid-Lipofuscinoses / genetics,  pathology*
Grant Support

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