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- Title
La dystrophie neuroaxonale infantile ou maladie de Seitelberger.
- Authors
Toga, M.; Berard-Badier, M.; Gambarelli-Dubois, D.
- Abstract
Two cases of INAD are reported. Clinical features with fatal issue within 2 years, are characterized by psychomotor retardation, pyramidal and extrapyramidal signs, pendular nystagmus with bilateral atrophia of optic discs. Light microscopy shows:-the morphology of spheroids, their glycolipoproteid component and their elective distribution in medulla, brain-stem and cerebellum, phylogenetically the oldest parts of the CNS-a cerebellar cortical atrophia-sudanophilic fatty deposits associated to status dysmyelinatus of pallidum-a degeneration of pyramidal, spinocerebellar tracts and optic pathways. Electron microscopy in the two cortical biopsies allows to specify the internal structure of spheroids made of membrano-tubular and interconnected aggregates, amorphous and lamellar electron-dense bodies, multigranular bodies, cristalline-like inclusions, multivesicular bodies and involved mitochondria. That complex material represents an overproduction of smooth endoplasmic reticulum and abnormal mitochondria located in neuronal perikarya, axons, dendrites and enlarged synaptic bulbs. These pathological findings are compared to those already described in human and experimental cases.
- Publication
Acta Neuropathologica, 1970, Vol 15, Issue 4, p327
- ISSN
0001-6322
- Publication type
Article
- DOI
10.1007/BF00684731