ISSN:
1432-0533
Keywords:
Familial Céroïd-Lipofuscinosis
;
Batten Disease
;
Late Infantile Amaurotic Idiocy
;
Consanguinity
;
Curvilmear, Finger-Print Inclusions
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary The authors report histochemical and electron mciroscopic data of brain biopsies in two cases of Battern's disease. The siblings affected, a male and a female, are born from consanguineous North African parents. The diagnosis of Ceroïd-Lipofuscinosis is supported by neuro-glial, endothelial and perithelial autofluorescent cell storage. By electron microscopy the abnormal cytosomes show both curvilinear and finger-print profiles; their lysosomal nature is supported by their obvious acid phosphatase activity. These lipopigment cytoplasmic inclusions are compared with those described in senile brains and in other cases of Batten's disease.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00685289
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