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We report on a male patient aged 38, affected by a syndrome whose characteristic features include onset in early childhood, slow progression, diffuse muscle weakness, mental retardation and cardiomyopathy. Muscle biopsy showed myopathic changes compatible with muscular dystrophy. However, immunostaining for dystrophin as well as 50 and 43 kDa dystrophin-associated glycoproteins (DAGs) was normal. Genetic analysis suggested that direct involvement of the dystrophin gene was highly unlikely. No other family members were affected. Although the clinical picture is reminiscent of Duchenne/Becker muscular dystrophy, the immunologically and genetically documented lack of dystrophin involvement suggests that this particular syndrome is as yet undescribed.

Progressive rubella panencephalitis is a very rare slow virus disease of the nervous system. The authors present a case, concerning a young man, aged 20 years, died 11 months after the onset of the disease. The following peculiarities of the case are emphasized: 1) the clinical symptomatology and the evolution (myoclonus, lack of cerebellar impairment) could suggest the diagnosis of SSPE; 2) the EEG recordings showed epileptiform abnormalities, long latency diffuse periodic complexes and--during interferon therapy and simultaneously with a temporary clinical improvement--the appearance of short latency anterior periodic complexes.

Two sisters, affected by late infantile type Sulfatidosis A, died at 22 and 15 years old respectively, presented, after autoptic examination, also a ponto-cerebellar athrophy. The EEG finding was characterized by few bioelectric abnormalities in the earlier studies of the disease and, afterwards, a progressive slower electric activities, with isolated asymmetric spikes. The typical aspects in the EEG recordings of our two patients were the presence of many rapid and diffuse elements, shay wawes without any pharmacological treatment. The significance of these peculiar recordings is there discussed.

The study concerns the EEG records of 153 children, aged within 1 and 4 and suffering from different neuropsychiatric disorders. Particularly during the first stages of sleep, graphoelements of epileptogenic morphology have been recorded in considerable amount. Obviously they were present in the records of clinically epileptic patients but they appeared also in the records of several patients having never suffered from epileptic seizures. The Authors suggest extreme caution in considering "epileptogenic" EEG patterns evoked by sleep in those children who are not clinically epileptic.

Three children, two females and one male, born from unrelated parents show brachycephaly, ogival palate, blindness from 5-6 months and progressive piramidal symptoms. Two subjects had since sixth monthy epileptic seizures, the other one died at 5 th month. In one subject died at 28 month age, central nervous system autoptic examination shows neuronal cortical immaturity, lack of corpus callosum, visual and piramidal patways hypoplasia. The serial EEG recording shows three caracteristic aspects: a) altough a marked changes, the organization of the electric cerebral activity was partially preserved; b) a epileptogenic potentiality was present, that is caracteristic of electro-clinic immage of this subject; c) a suggestive and peculiar tendency to asincrony and asimmetry of epileptic tendency in two hemispheres.

The AA. describe the clinical features of a woman (fourty-one years old) suffering from Stiff-man syndrome. The treatment with diazepam and baclophene improuved the symptoms in a very satisfactory manner. The Author's opinion is that syndrome could be due to abnormal activity of neurotransmitters.