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Clinical manifestations of 5 patients with idiopathic paroxysmal kinesigenic choreoathetosis
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- Miyagi Tetsuya
- Department of Neurology, National Hospital Organization Okinawa Hospital
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- Okuma Megumi
- Department of Neurology, National Hospital Organization Okinawa Hospital
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- Suwazono Shugo
- Department of Neurology, National Hospital Organization Okinawa Hospital
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- Kido Miwako
- Department of Neurology, National Hospital Organization Okinawa Hospital
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- Tashiro Yuichi
- Department of Neurology, National Hospital Organization Okinawa Hospital
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- Ishihara Satoshi
- Department of Neurology, National Hospital Organization Okinawa Hospital
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- Nakachi Ryo
- Department of Neurology, National Hospital Organization Okinawa Hospital
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- Suehara Masahito
- Department of Neurology, National Hospital Organization Okinawa Hospital
Bibliographic Information
- Other Title
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- 特発性発作性運動誘発性舞踏アテトーシス5症例の検討
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Description
Paroxysmal kinesigenic choreoathetosis (PKC) is a rare disorder characterized by recurrent and brief attacks of choreoathetoid and/or dystonic movements in trunk and limbs triggered by initiation of voluntary movement. Of 5 patients with idiopathic PKC in our hospital, four were men and one was with family history. Age of onset ranged from 8 to 15 years old. They were consistent with previous reports in the characteristics of involuntary movements, normal neurological findings, normal laboratory data, no abnormal findings of standard imaging studies, and good restraining effects on attacks with carbamazepine. Individual body parts where attacks often involved were different among 5 patients. Although previous reports which said the prognosis and outcome of PKC were good, neuropsychological examinations in our study revealed that 2 patients out of 5 had certain cortical dysfunction, one patient was with progressive deterioration, and the other was with underlying mild abnormalities. Detailed and serial neuropsychological examinations might be necessary for some PKC patients.
Journal
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- Rinsho Shinkeigaku
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Rinsho Shinkeigaku 56 (3), 165-173, 2016
Societas Neurologica Japonica
