A sporadic juvenile case of the amyotrophic lateral sclerosis with neuronal intracytoplasmic inclusions.

Abstract:

:In an autopsy case of the sporadic juvenile ALS (a 17-year-old girl) intracytoplasmic inclusions are found in the upper and lower motor neurons and in nerve cells of the dentate nucleus, pontine nucleus, brain stem reticular formation, substantia nigra, thalamus, globus pallidus and others. Histochemically they contain RNA-Protein compounds. Electron microscopically, they consist of randomly interwoven tubules with granular endoplasmic reticulums and free ribosomes in the margin. Each tubule measures 90--150 A in diameter and shows no distinct periodic constiction. Amorphous substances as well as ribosome granules are scattered and associated with those tubules. The inclusion-bearing cells are usually swollen and chromatolytic and have a large hydropic nucleus, suggesting a close relation between the development of the inclusion and chromatolysis. Clinically, a rapid progress of the symptoms (total duration: about 12 months) and conspicuous disturbances of the autonomic nerve, such as sinus tachycardia and bladder sphincter dysfunction, should be noticed.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Oda M,Akagawa N,Tabuchi Y,Tanabe H

doi

10.1007/BF00691069

subject

Has Abstract

pub_date

1978-12-15 00:00:00

pages

211-6

issue

3

eissn

0001-6322

issn

1432-0533

journal_volume

44

pub_type

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