Pigment Variant of Neuronal Ceroid-Lipofuscinosis (Kufs’ Disease)

A case of pigment variant of Kufs’ disease is presented. The nature of the extra-neuronal pigment is discussed. Despite some of the histochemical discrepancies that existed between this pigment and the material that had accumulated in the nerve cells, they seemed to he ultrastructurally related. The...

Full description

Saved in:
Bibliographic Details
Published in:Canadian journal of neurological sciences 1977-02, Vol.4 (1), p.67-75
Main Authors: Horoupian, Dikran S., Ross, R. T.
Format: Article
Language:English
Subjects:
Adult
Brain - ultrastructure
Cerebellum - ultrastructure
Female
Frontal Lobe - ultrastructure
Globus Pallidus - ultrastructure
Humans
Inclusion Bodies - ultrastructure
Lipidoses - pathology
Liver - ultrastructure
Neurons - ultrastructure
Putamen - ultrastructure
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:A case of pigment variant of Kufs’ disease is presented. The nature of the extra-neuronal pigment is discussed. Despite some of the histochemical discrepancies that existed between this pigment and the material that had accumulated in the nerve cells, they seemed to he ultrastructurally related. The hepatocytes contained numerous heterogeneous cytosomes, some of which resembled the storage material of Niemann-Pick’s disease. Clinically the syndrome may present with progressive ataxia, spontaneous and reflex, coarse myoclonic jerks and eventual mental deterioration as well as epilepsy and muscle wasting. The pigment variant cannot be distinguished from Kufs’ disease except pathologically.
ISSN:0317-1671
2057-0155
DOI:10.1017/S0317167100120438