Sporadic amyotrophic lateral sclerosis with multiple system degeneration : a report of an autopsy case without respirator administration

This report concerns an autopsy case of amyotrophic lateral sclerosis (ALS) with unusual clinical and neuropathological findings. The patient was a Japanese man without hereditary burden who was 49 years old at the time of death. His clinical manifestation included dysarthria at age 48, followed by...

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Bibliographic Details
Published in:Acta neuropathologica 1999-11, Vol.98 (5), p.512-515
Main Authors: MACHIDA, Y, TSUCHIYA, K, ANNO, M, HAGA, C, ITO, T, SHIMO, Y, WAKESHIMA, T, IRITANI, S, IKEDA, K
Format: Article
Language:English
Subjects:
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - pathology
Atrophy
Autopsy
Biological and medical sciences
Bunina bodies
Cerebellar Nuclei - pathology
Degeneration
Dementia disorders
Dentate nucleus
Dysphagia
Humans
Japan
Male
Medical sciences
Middle Aged
Motor neurons
Motor Neurons - pathology
Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis
Neurology
Neuropathology
Reflexes
Spinal Cord - pathology
Substantia nigra
Substantia Nigra - pathology
Tremor
Ubiquitin
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Summary:This report concerns an autopsy case of amyotrophic lateral sclerosis (ALS) with unusual clinical and neuropathological findings. The patient was a Japanese man without hereditary burden who was 49 years old at the time of death. His clinical manifestation included dysarthria at age 48, followed by dysphagia, atrophy and fasciculation of the tongue, muscle weakness in the four extremities, tremor, rigidity, increased deep tendon reflexes in the upper and lower extremities, and incoordination of the four extremities. He died of respiratory failure 12 months after the disease onset. No respirator administration was performed throughout the clinical course. The neuropathological examination revealed not only degeneration of upper and lower motor neuron systems, including the presence of Bunina bodies and ubiquitin-immunoreactive neuronal inclusions in the lower motor neurons, but also prominent degeneration of the substantia nigra and dentate nucleus with slight neuronal loss in the locus ceruleus and pontine nucleus. To our knowledge, this is the first reported case of sporadic ALS without dementia and respirator support, showing degeneration of the substantia nigra and dentate nucleus. This report may contribute to the resolution of the question concerning the neuropathological heterogeneity of sporadic ALS with respiratory support.
ISSN:0001-6322
1432-0533
DOI:10.1007/s004010051117