Document Detail

An adult-onset case of sporadic motor neuron disease with basophilic inclusions.
MedLine Citation:
PMID:  1703386     Owner:  NLM     Status:  MEDLINE    
A 36-year-old man developed motor neuron signs consisting of weakness and atrophy of the right upper limb, which progressed to involve the other limbs along with development of upper motor neuron signs including pseudobulbar palsy. He died 8.5 years after onset. Bilateral precentral gyri and putamina were grossly atrophic. In addition to severe degeneration of bilateral pyramidal tracts and marked neuronal cell loss of the precentral gyri and putamina, basophilic inclusions were widely distributed in the motor cortex, putamina, general somatic motor neurons such as the hypoglossal nucleus and spinal anterior horns, and other areas like the red nucleus and inferior olive. The inclusions were clearly shown with Nissl stain to be anilinophilic irregular masses with distinct rims. Ultrastructurally the inclusions appeared to consist of thick filamentous structures of 12-25 nm in diameter studded with electron-dense ribosome-like granules. Thick filamentous profiles were relatively short or occasionally fragmentary, haphazardly mingled with various amounts of granules and other organelles. No prominent accumulation of 10-nm neurofilaments or eosinophilic inclusions like Bunina bodies were found. The inclusions were indistinguishable from those reported in so-called "juvenile" amyotrophic lateral sclerosis.
H Kusaka; S Matsumoto; T Imai
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta neuropathologica     Volume:  80     ISSN:  0001-6322     ISO Abbreviation:  Acta Neuropathol.     Publication Date:  1990  
Date Detail:
Created Date:  1991-02-28     Completed Date:  1991-02-28     Revised Date:  2007-11-09    
Medline Journal Info:
Nlm Unique ID:  0412041     Medline TA:  Acta Neuropathol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  660-5     Citation Subset:  IM    
Department of Neurology, Kitano Hospital, Osaka, Japan.
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MeSH Terms
Amyotrophic Lateral Sclerosis / pathology*
Brain / pathology
Microscopy, Electron
Motor Cortex / pathology
Motor Neurons / ultrastructure*
Nervous System Diseases / pathology*
Putamen / pathology
Spinal Cord / pathology
Staining and Labeling

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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