title: [Amyotrophic lateral sclerosis associated with extrapyramidal symptoms or signs].
abstract: This investigation was conducted to clarify the frequency and characteristics of ALS associated with extrapyramidal symptoms or signs in Wakayama prefecture. The questionnaires to survey ALS cases were mailed to all medical centers in Wakayama prefecture. A total of 252 cases were found to have motor neuron diseases. Among them, 204 cases fulfilled probable or definite according to El Escorial Criteria. In 10 of them, extrapyramidal signs were identified as follows: rigidity 50%, tremor 40% and akinesia 10%. Family history of ALS in these cases (20%) is higher than expected in usual ALS, and all of them are negative for SOD-1 mutation. Dementia and autonomic nervous symptoms were observed in several cases. Incidence of extrapyramidal signs in ALS resulted in 4.8%. The incidence of extrapyramidal signs is more frequent than expected by chance, suggesting that the degeneration of basal ganglia and/or substantia nigra may not be so rare in ALS.
authors.affiliation: Department of Neurology, Wakayama Medical University, 811-1 Kimiidera, Wakayama 641 8510, Japan.
authors.forename: Kiwa
authors.initials: K
authors.lastname: Hama
authors.forename: Tameko
authors.initials: T
authors.lastname: Kihira
authors.forename: Masae
authors.initials: M
authors.lastname: Okawa
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Kajimoto
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Hiwatani
authors.forename: Shuhei
authors.initials: S
authors.lastname: Morita
authors.forename: Ichiro
authors.initials: I
authors.lastname: Nakanishi
authors.forename: Hideto
authors.initials: H
authors.lastname: Miwa
authors.forename: Tomoyoshi
authors.initials: T
authors.lastname: Kondo
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000453:epidemiology; D001480:Basal Ganglia Diseases / Q000453:epidemiology; D005260:Female; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D011795:Surveys and Questionnaires