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Titolo:
Sporadic amyotrophic lateral sclerosis with multiple system degeneration: a report of an autopsy case without respirator administration
Autore:
Machida, Y; Tsuchiya, K; Anno, M; Haga, C; Ito, T; Shimo, Y; Wakeshima, T; Iritani, S;
Indirizzi:
Tokyo Metropolitan Matsuzawa Hosp, Dept Lab Med & Pathol, Setagaya Ku, Tokyo 1560057, Japan Tokyo Metropolitan Matsuzawa Hosp Tokyo Japan 1560057 kyo 1560057, Japan Tokyo Metropolitan Matsuzawa Hosp, Dept Neurol, Tokyo, Japan Tokyo Metropolitan Matsuzawa Hosp Tokyo Japan Dept Neurol, Tokyo, Japan Tokyo Inst Psychiat, Dept Neuropathol, Tokyo, Japan Tokyo Inst Psychiat Tokyo Japan sychiat, Dept Neuropathol, Tokyo, Japan Juntendo Univ, Sch Med, Dept Neurol, Tokyo 113, Japan Juntendo Univ Tokyo Japan 113 iv, Sch Med, Dept Neurol, Tokyo 113, Japan Tokyo Metropolitan Matsuzawa Hosp, Dept Psychiat, Tokyo, Japan Tokyo Metropolitan Matsuzawa Hosp Tokyo Japan pt Psychiat, Tokyo, Japan
Titolo Testata:
ACTA NEUROPATHOLOGICA
fascicolo: 5, volume: 98, anno: 1999,
pagine: 512 - 515
SICI:
0001-6322(199911)98:5<512:SALSWM>2.0.ZU;2-O
Fonte:
ISI
Lingua:
ENG
Soggetto:
MOTOR-NEURON DISEASE; NIGRAL ATROPHY; DEMENTIA; OPHTHALMOPLEGIA; INVOLVEMENT;
Keywords:
amyotrophic lateral sclerosis; dentate nucleus; multiple system degeneration; respirator; substantia nigra;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
20
Recensione:
Indirizzi per estratti:
Indirizzo: Tsuchiya, K Tokyo Metropolitan Matsuzawa Hosp, Dept Lab Med & Pathol, Setagaya Ku, 2-1-1 Kamikitazawa, Tokyo 1560057, Japan Tokyo Metropolitan Matsuzawa Hosp 2-1-1 Kamikitazawa Tokyo Japan 1560057
Citazione:
Y. Machida et al., "Sporadic amyotrophic lateral sclerosis with multiple system degeneration: a report of an autopsy case without respirator administration", ACT NEUROP, 98(5), 1999, pp. 512-515

Abstract

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 rime 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 theupper 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 slightneuronal 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.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 15/08/20 alle ore 17:49:34