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PubMed1992

Pseudoathetosis as a presenting symptom of spinal multiple sclerosis.

We report on a 38-year-old female patient with acute proprioceptive sensory impairment and pseudoathetosis in the four limbs, particularly in the fingers of both hands. She had great difficulty in buttoning, unbuttoning, using chopsticks and writing, because she was no longer able to feel her fingers in space. There was a profound loss of position and vibration sensation in all limbs, especially in both hands. Pseudoathetoid movement, a rare presentation of multiple sclerosis, was observed in the outstretched hands and extended fingers. T1- and T2-weighted magnetic resonance imaging (MRI) of the spinal cord revealed an inactive intramedullary lesion in the C2-C5 segments. Gadolinium-enhanced MRI revealed an active lesion in the posterior columns of the cervical cord at the C3 vertebral level, which is very likely responsible for pseudoathetoid movement. Based on the disseminated lesions in the spinal cord verified by MRI the four neurologic manifestations, and the abnormal somatosensory-evoked potentials, we made a definitive diagnosis of multiple sclerosis of the spinal cord.

Adult↗
PubMed1992

Factors associated with choreoathetosis after cardiopulmonary bypass in children with congenital heart disease.

BACKGROUND: Choreoathetosis (CHO) after congenital heart surgery has been described since 1960. Risk factors and patient outcome have not been well defined. METHODS AND RESULTS: As our complexity of cases increased and management of pH on cardiopulmonary bypass (CPB) evolved, we noted the appearance of CHO among patients beginning in 1986. We reviewed the hospital course and follow-up of all 19 affected children, including eight younger patients (median age, 4.3 months) who developed a mild transient form of CHO, all of whom survived and had complete resolution of CHO; seven of these eight patients had deep hypothermic circulatory arrest (DHCA). Eleven older patients (median age, 16.8 months) developed severe persistent CHO; 11 had DHCA, 10 were cyanotic, seven had anatomic pulmonary atresia, and three others were physiologically analogous with a systemic to pulmonary artery shunt-dependent circulation. Five of six patients who had pertinent preoperative angiography had systemic to pulmonary collateral vessels arising from the head and neck arteries. Mortality in severe patients was 36% (four of 11); the seven survivors showed improvement, but only one had a normal neurological examination after 60 months of follow-up. When severe CHO patients were compared with 17 age- and diagnosis-matched patients without neurological complications, no differences were found in CPB or DHCA times, arterial blood gases, or hematocrits. Time from onset of CPB to onset of DHCA (time to shutoff) was shorter in the severe persistent CHO group than for comparison patients (22 +/- 7 versus 40 +/- 29 minutes, p = 0.053). CONCLUSIONS: Factors that may be associated with the development of severe persistent CHO include 1) age beyond early infancy, 2) cyanotic heart disease with systemic to pulmonary collaterals, particularly those arising from the head and neck vessels, and 3) the duration of the cooling period used in conjunction with deep hypothermic circulatory arrest. We advocate earlier reparative surgery, precise preoperative diagnosis and preoperative or intraoperative control of systemic to pulmonary artery collaterals, and further study of pH effects during CPB on development of CHO.

Athetosis↗