Benign familial tremor treated with primidone.
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Biomedical subjects
Publications and source records attributed to P Pazzaglia.
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A 16-year-old patient who had a history of complex partial seizures, had frequent episodes of status epilepticus with diffuse slow-wave discharges. The clinical manifestations were apparently insignificant due to the fact that vigilance, orientation and behaviour were unimpaired. Neuropsychological investigations showed that the cognitive processes were selectively impaired during such episodes. The electroclinical pattern was interrupted by break-off of contact concomitant with high-frequency spike discharges. Cognitive impairment is believed to represent the specific feature peculiar to this type of status epilepticus.
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3 cases are reported, where neuralgic amyotrophy occurred simultaneously in 3 members of one family, living in the same household. Despite the familial occurrence and the simultaneous onset, the clinical pictures were those of the common form of the neuralgic amyotrophy.
A case of polyneuropathy in a 14-year-old boy, a chronic gasoline sniffer, is reported. Clinical and electromyographic examination showed a symmetrical motor involvement, mainly distally and in the lower limbs. A sural nerve biopsy showed only slight changes, both of axonal and demyelinating type. The role of gasoline toxic substances in the etiology of this rare polyneuropathy is discussed.
A case of stiff-man syndrome associated with primary generalised epilepsy is reported. In addition, nocturnal polygraphic recording revealed a nocturnal myoclonus. Detailed examination of the central nervous system did not show specific changes. There is no direct proof as to a spinal or supraspinal origin of the stiff-man syndrome. The absence of specific anatomical lesions may indicate a functional rather than a structural disturbance in its physiopathogenesis.
In the area of Cesena, Italy, the prevalence of epilepsy was about 3/1,000 among the 13,000 children of school age, 6-14 years. Only half the epileptic pupils had a normal scholastic record. One-third were in special classes, nearly all because of mental retardation due either to birth injury or to damage in infancy. The others (17%) were behind by one or more classes, mainly because of a depressive reaction owing (in half of the intelligent epileptics) mainly to classmates' fear of seizures and to the hostility of classmates' parents after the pupil had a grand mal seizure in school. Teachers had usually not learned how to manage either seizures in the classroom or the problems of pupils with epilepsy.
The clinical course and prognosis of 282 cases of acute post-traumatic coma have been studied. Death occurred in 140 cases (49 percent), and, of survivors, about two-thirds achieved complete social reintegration, while one-third were partially reintegrated or not at all. The quality of survival did not depend only on the nature of the physical sequelae, but also on other factors, particularly social ones. With regard to early prognosis, the authors believe it is possible to predict the final outcome of the patient by using the indices of age, level of coma, and the nature of the intracranial lesions.
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