Paroxysmal kinesigenic choreoathetosis associated with prenatal brain damage.
We describe a 15 year old patient with paroxysmal kinesigenic choreoathetosis. Neurological examinations revealed a paresis of the right arm and hand that was similar to ulnar nerve palsy, a right homonymous hemianopsia and an ocular movement disturbance of smooth pursuit to left. Attacks of dystonic spasms began abruptly, usually following running, and lasted less than 5 min. Magnetic resonance imaging displayed a linear area of increased signal in the T2-weighted images along the lateral margin to the left putamen, atrophies of the frontal and temporal opercula and a large porencephalic cyst in the left parieto-temporo-occipital region. A cerebral blood flow study with single photon emission computed tomography showed hypoperfusion of the lenticular nucleus and the regions corresponding to the atrophies and the porencephalic cyst. Electroencephalograms during the attacks could not demonstrate epileptic abnormality. Only the neuronal plasticity of an immature brain could explain the discrepancy between the observed huge lesions of the brain and the minor neurological symptoms present. Attacks of paroxysmal kinesigenic choreoathetosis might occur when the basal ganglia maturate to some extent, even if the lesions in the brain were caused before birth.
Hamano
S
S
Division of Neurology, Saitama Children's Medical Center, Japan.
Tanaka
Y
Y
Nara
T
T
Nakanishi
Y
Y
Shimizu
M
M
D000293:Adolescent; D001264:Athetosis / Q000150:complications*; D001925:Brain Damage, Chronic / Q000000981:diagnostic imaging / Q000196:embryology* / Q000473:pathology; D002560:Cerebrovascular Circulation; D002819:Chorea / Q000150:complications*; D004421:Dystonia / Q000150:complications; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011877:Radionuclide Imaging