An 8-year-old boy with a variant of the Klippel-Trenaunay-Weber syndrome (KTW syndrome) is described. The hemangiomatous tissue located to the right half of his trunk and extremities was hypotrophic. On the same side, on his face and gingivae the tissue appeared hypertrophic and dental abnormalities were present. Moreover, the patient suffered from psychomotor epilepsy caused by a right-sided temporal astrocytoma. The connection between the KTW syndrome and the neurocutaneous syndromes is discussed.
Eight patients with normal-pressure hydrocephalus (NPH) were studied. The resting mean arterial blood pressure (MABP) was 100 (90-125) mmHg and the mean intracranial pressure (ICP) was 11 (5-17) mmHg. ICP and MABP were continuously measured intraventricularly and intra-arterially, respectively. Changes in global cerebral blood flow (CBF) were estimated by the arteriovenous oxygen difference method. Intravenous nimodipine (15 microgram/kg/hour) was given in the first 2 hours and 30 microgram/kg/hour in the next 2 hours. MABP was reduced 23 (4-47) mmHg (p less than 0.05). ICP was increased 3 (0-10) mmHg (p less than 0.05). CBF was unchanged in the group on the whole, but in 4 of patients a major drop in perfusion pressure was seen, and CBF decreased 6, 11, 23 and 34%, respectively. Thus these findings underline the importance of maintaining the perfusion pressure under treatment with nimodipine.
SUMMARY A case of prolonged, though reversible procaine-induced Parkinsonism treated with L-dopa and trihexyphenidyl (Artane-Sustets) is reported. A pathophysiological explanation is discussed on the basis of recent theories concerning the transmitter systems of the basal ganglia.
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