ISSN:
1432-0533
Keywords:
Psychomotor Retardation
;
Smooth Cisterns
;
Zebra Bodies
;
Storage Disorder
;
Mode of Formation of Abnormal Cytosomes
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary The ultrastructural and biochemical study of cerebral and liver biopsies from a two-year-old boy with 20 month history of psychomotor retardation, hypotonia and hyporeflexia is presented. Although the case cannot be considered, at the present time, as a separate entity in view of the failure of the biochemical studies of identifying the stored material, the ultrastructural features, which include clustered agranular cisterns of endoplasmic reticulum and numerous multivesicular bodies and “zebra” bodies in neurons, indicate a storage disorder morphologically different from those reported to date. In the present case, morphological and cytochemical findings suggest that the agranular endoplasmic reticulum may give trise to multivesicular bodies which, in turn, may become dense bodies and “zebra” bodies.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00687601
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