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Vol. 13, No. 4-5, 1991
Issue release date: 1991
Dev Neurosci 1991;13:331–338
(DOI:10.1159/000112182)
Ceroid – Lipofuscinoses

Adult Type of Neuronal Ceroid Lipofuscinosis

Martin J.-J.
Born-Bunge Foundation and University of Antwerp, Belgium Dev Neurosci 1991;13:331–338 (DOI:10.1159/000112182)

Abstract

Adult neuronal ceroid lipofuscinosis (NCL), also called Kufs'' disease, is clinically distinct from the other NCLs. It is a rare condition which is difficult to diagnose. More than 50% of the reported cases of Kufs'' disease are not adult NCL and correspond very likely to a heterogeneous spectrum of lipidoses. Various clinical and genetic phenotypes of adult NCL may be recognized, one featuring a progressive myoclonus epilepsy. It is important to stress that in contradistinction with the juvenile and protracted juvenile NCL, there is no pigmentary degeneration of the retina. Adult NCL is an autosomal recessive condition but two families have an autosomal dominant inheritance.

 goto top of outline Author Contacts

Jean-Jacques Martin, MD, Born-Bunge Foundation-Gebouw T-Room 5–18, Universitaire Instelling Antwerpen, Universiteitsplein, 1, B–2610 Wilrijk-Antwerp (Belgium)


 goto top of outline Article Information

Number of Print Pages : 8


 goto top of outline Publication Details

Developmental Neuroscience

Vol. 13, No. 4-5, Year 1991 (Cover Date: 1991)

Journal Editor: Campagnoni, A.T. (Los Angeles, Calif.)
ISSN: 0378–5866 (print), 1421–9859 (Online)

For additional information: http://www.karger.com/DNE


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