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Surgical treatment for cervical spondylotic myelopathy associated with athetoid cerebral palsy.

There have been several reports on surgical interventions in patients with adult cervical spondylotic myelopathy associated with athetoid cerebral palsy; however, the long-term effectiveness of these interventions has not been demonstrated. We have performed surgical treatments--posterior fusion with wave-shaped rods and anterior interbody fusion with internal fixators-- in 20 patients. The present study included 17 of these patients, 16 men and 1 woman, and their mean follow-up period was 8.6 years (range, 5-15.5 years). One year after surgery, walking ability was improved in 14 patients. Pain in the upper extremities and muscle weakness of the deltoid were alleviated in all patients. One patient showed recurrence of myelopathy after 8.5 years' follow-up. Our surgical technique is effective in patients with cervical spondylotic myelopathy secondary to athetoid cerebral palsy, even in those with severe involuntary movements. Postoperative rigid external fixations are not required.

Adult↗

Clinical experience with phenytoin prophylaxis in severe preeclampsia.

In North America, magnesium sulfate is the traditional treatment for severe preeclampsia and eclampsia. Its use has been strongly criticized, in that it acts peripherally with no central effect and maternal and neonatal complications are well known. Phenytoin, the mechanism of action of which is clear, seems a logical alternative. Our aim was to develop a safe, practical, effective regimen with the use of phenytoin. In this prospective, descriptive study four dosage regimens were investigated in 104 patients. The initial regimens produced unexpected and unacceptable side effects in comparison with the nonpregnant population. The final regimen (15 mg/kg intravenously, given as 10 mg/kg initially then 5 mg/kg 2 hours later) provided therapeutic levels and had minimal maternal and perinatal side effects. No seizures occurred after its correct usage. This regimen is simple, safe, effective, and provides ongoing anticonvulsant coverage in the postpartum period. We suggest that phenytoin may represent a suitable alternative anticonvulsant in this condition.

Athetosis↗

Choreoathetosis after deep hypothermia without circulatory arrest.

In 8 of 758 patients undergoing an intracardiac operation under cardiopulmonary bypass and hypothermia, choreoathetosis developed 3 to 7 days postoperatively. Before the onset of choreoathetosis, varying degrees of neurological dysfunction were noted. Electroencephalography and neuroimaging failed to detect any responsible functional or structural changes. Six patients are alive 1 to 3 years postoperatively, and their condition is improving. Two patients died of aspiration or sepsis. All patients were grouped based on factors identified as being possibly causative: depth of hypothermia, cooling time, flow rate, and repeated hypothermia. The incidence of choreoathetosis was significantly different in group A (rectal temperature greater than 25 degrees C) compared with group B (rectal temperature less than or equal to 25 degrees C) (0/295 versus 8/463; p = 0.02). Based on cooling time, the incidence of choreoathetosis was significantly different in group B1 (cooling time less than 1 hour) compared with group B2 (cooling time greater than or equal to 1 hour) (1/220 versus 7/243; p = 0.05). Based on flow rate during cooling, group B2 was further divided into the low-flow group (less than 1,500 mL.min-1.m-2) and the high-flow group (greater than or equal to 1,500 mL.min-1.m-2). Although not significant, the incidence of choreoathetosis was higher in the high-flow group (6/153 versus 1/90; p = 0.22). In group B patients having reoperation, the incidence of choreoathetosis was higher than in patients operated on for the first time (5/54 versus 3/409; p less than or equal to 0.0001).(ABSTRACT TRUNCATED AT 250 WORDS)

Athetosis↗

Role of the basal ganglia in a chemically induced dyskinesia in rat.

A complex syndrome consisting of locomotor hyperactivity and a cervical choreoathetoid dyskinesia was produced in rats after 7 to 10 days administration of 3-3 -iminodipropionitrile. Attenuation of motor abnormalities after lesions of the basal ganglia (BG) but not cortical lesions suggests the importance of descending BG influences in this syndrome. Responses of this syndrome to pharmacologic manipulations further argues for BG involvement. This syndrome may be a possible animal model of human hyperkinetic basal ganglia disorders.

Animals↗

Predictive measures of speech proficiency in cerebral palsied speakers.

The present study attempted to investigate the relationship between judged speech proficiency of cerebral palsied speakers and the following quantitative speech parameters: 1) the number of articulatory errors; 2) the intelligibility of the speech of the cerebral palsied; 3) mean vocal fundamental frequency; 4) variation of vocal fundamental frequency; 5) mean speech-sound pressure level; 6) variation of speech-sound pressure level; 7) word-per-minute speech rate. Secondarily, the study investigated the differences in the speech of the spastic and athetoid populations in terms of the parameters listed above.

Adolescent↗

Peroneal muscular atrophy and epilepsy with cerebellar ataxia and choreoathetosis in the same family.

Seven members in three generations of a family are affected with peroneal muscular atrophy. Five have the disorder in combination with epilepsy. The proposita exhibits a cerebellar syndrome, she and one brother show choreiform movements, and she, her mother and a great-uncle have pes cavus. One member has EEG abnormalities but no muscular atrophy. The familial occurrence of this combination of symptoms has not been described before. In this family the syndrome is transmitted as an autosomal dominant with variable expressivity.

Adult↗