An unusual type of primary cerebral hemihypotrophy with signs of dysfunctional neuronal migration.

We describe the neuropathological features of a complex brain malformation characterized by cerebral hemihypotrophy with ipsilateral lissencephaly, periventricular nodular heterotopia and macrogyria. The contralateral hemisphere showed only slight alterations of the gyral pattern and a limited periventricular gray matter heterotopia. The clinical picture of the patient, who died at the age of 15 years, consisted of severe oligophrenia, intractable seizures and left hemiparesis.

Muscular dystrophy, mental retardation and cardiomyopathy not associated with dystrophin deficiency.

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.

Progressive rubella panencephalitis. Follow-up EEG study of a case.

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.

[Possibilities of error in EEG in sleep and consciousness (electroencephalographic observations in 153 children)].

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.

[Nonpharmacological, ubiquitous, rapid, fused graphic elements in the electroencephalographic pattern of 2 sisters with type A sulfatidosis and ponto-cerebellar systemic atrophy].

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.

[Serial electroencephalographic study of 3 siblings with agenesis of the interhemispheric great commissurae, cortical neuronal immaturity and hypoplasia of the optico-pyramidal tracts].

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.

[Pathogenetic, clinical and therapeutic considerations on a case of the stiff man syndrome].

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.