No abstract

We treated 15 children with Sydenham's chorea with sodium valproate at a dose of 15 to 20 mg/kg/d. In 13 of them the chorieform movements disappeared within 1 week of therapy. The remaining 2 had incomplete response. Two patients relapsed; 1 of them responded to the reintroduction of valproate. The mean duration of treatment was 6.7 weeks. We observed no major side effects. This study strongly suggests that valproate is an effective drug in the treatment of Sydenham's chorea.

Six girls between 2 years 9 months and 15 years of age with Rett syndrome were thoroughly investigated. Blood ammonia levels varied between 42 and 123 mumol/L, and serum lactate concentration was slightly elevated in two girls. Electroencephalograms showed a dysrhythmic pattern during wakefulness; during drowsiness and light sleep, bilateral bursts of spike or multispike-and-wave activity were seen in all but the oldest girl. In one of the younger girls, slight cortical atrophy was found on computed tomographic scan. Muscle biopsy was performed on all girls, and electron microscopy revealed abnormal mitochondria. Physical signs such as somatic hypotrophy with extremely small muscle mass, and unsatisfactory weight gain in spite of good appetite are found in Rett syndrome. These attributes, as well as reports of ornithine carbamoyltransferase deficiency, may support a mitochondrial dysfunction. The mitochondrial changes indicate either a mitochondrial mutation or more probably an X-borne modulator gene mutation. Another genetic possibility discussed is the "metabolic interference" of an X-borne allele. Further delineation of such mitochondrial changes may clarify the causal metabolic defect in Rett syndrome.

Detailed functional-morphological correlation on co-registered gated SPECT-CT images contributes to accurate assessment of regional functional impairment, and may be useful for surgical planning, prediction of postoperative function and assessment of external beam radiotherapy effects in patients with lung cancer.

Endoscopic CT lymphography is a feasible method for visualizing the direct connection between and the accurate anatomic location of SLNs and lymphatic drainage vessels.