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paroxysmal kinesigenic choreoathetosis
Paroxysmal kinesigenic choreathetosis (PKC) also called Paroxysmal Kinesigenic Dyskinesia (PKD) is a hyperkinetic movement disorder characterized by attacks of involuntary movements, which are triggered by sudden voluntary movements. The number of attacks can increase during puberty and decrease in a person’s 20s to 30s. Involuntary movements can take many forms such as ballism, chorea or dystonia and usually only affect one side of the body or one limb in particular. This rare disorder only affects about 1 in 150,000 people with PKD accounting for 86.8% of all the types of paroxysmal dyskinesias and occurs more often in males than females. There are two types of PKD, primary and secondary. Primary PKD can be further broken down into familial and sporadic. Familial PKD, which means the individual has a family history of the disorder, is more common, but sporadic cases are also seen. Secondary PKD can be caused by many other medical conditions such as multiple sclerosis (MS), stroke, pseudohypoparathyroidism, hypocalcemia, hypoglycemia, hyperglycemia,〔 central nervous system trauma, or peripheral nervous system trauma.〔 PKD has also been linked with infantile convulsions and choreoathetosis (ICCA) syndrome, in which patients have afebrile seizures during infancy (benign familial infantile epilepsy) and then develop paroxysmal choreoathetosis later in life. This phenomenon is actually quite common, with about 42% of individuals with PKD reporting a history of afebrile seizures as a child.〔
==History of Discovery==
A movement disorder similar to PKD was first mentioned in research literature in 1940 by Mount and Reback. They described a disorder consisting of attacks of involuntary movements but unlike PKD, the attacks lasted minutes to hours and were found to be caused by alcohol or caffeine intake.〔 They named it paroxysmal dystonic choreoathetosis. Kertesz later described another new movement disorder in 1967. He described a disorder that was induced by sudden movements, and responded to anticonvulsants, naming it paroxysmal kinesigenic choreoathetosis. Finally in a review in 1995 Demirkiran and Jankovic stated the disease should be called paroxysmal kinesigenic dyskinesia instead, pointing out that the attacks could manifest as any form of dyskinesia, not just choreoathetosis.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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