[A patient presented with atypical paroxysmal kinesigenic choreoathetosis and Becker muscular dystrophy].
A 22-year-old man had choreatic movements in upper limbs, neck and trunk for over twelve years which were associated with dystonia in lower limbs upon initiating voluntary movements. The choreatic movement lasted for a few seconds and the dystonia lasted for a few minutes. He also had high serum CK levels and hypertrophic calf muscles. His muscle strength and deep tendon reflexes were normal. His choreatic movements fulfill the criteria for paroxysmal kinesigenic choreoathetosis (PKC). However, it was unclear what the symptom of dystonia was due to. From a muscle biopsy and DNA analysis, he was diagnosed as having Becker muscular dystrophy. Administration of anticonvulsant improved the dystonia as well as the choreatic movement, which showed that the dystonia was a symptom of PKC. Coincidence of choreatic movements and dystonias which had different lasting time in a patient of PKC was atypical and had not previously reported.
Fukada
K
K
Department of Neurology, Osaka University Graduate School of Medicine.
Moriya
M
M
Kaido
M
M
Abe
K
K
Umi
M
M
Yanagihara
T
T
D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use; D002220:Carbamazepine / Q000627:therapeutic use; D002819:Chorea / Q000150:complications*; D003402:Creatine Kinase / Q000097:blood; D006801:Humans; D006984:Hypertrophy / Q000150:complications; D007866:Leg; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D016896:Treatment Outcome
D000927:Anticonvulsants; D002220:Carbamazepine; D003402:Creatine Kinase