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"Incontinentia pigmenti" its a rare familial disorder of unknown origin characterized by a distinctive dermatosis, almost exclusive occurrence in females, and developmental abnormalities that affect hair, eyes, teeth and central nervous system. 30.5% of cases described in the world literature had notable C.N.S. disease. Authors report three cases of I.P. in pathognomonic pigmented stage and with severe neurologic involvement. In two cases pneumoencephalography show severe cerebral cortical atrophy. Patients had motor and mental retardation, abnormal E.E.G. and one of them a West syndrome. The third case died at the age of three months, and neuropathological examination of the brain revealed porencephalic lesions.

Seventeen cases of Guillain-Barré's syndrome aged between 14 month ans 7 years are studied from clinical, analytical and evolutives aspects. Neither sex nor season predominance occurs. Sixteen of the patients had a previous viral or bacterial infection. Difficulty in walking was the main symptom at the onset of illness. Arreflexia and motot paralysis were always symmetrical and ascending. Albumino-cytological dissociation in the C.S.F. was found in the whole group. An evolutive study was made of the recovery of movement, C.S.F. and tendon reflexes which occurred in this order except in two cases which showed abnormal evolution. We emphasize the extent and frequency of respiratory difficulties at the onset of illness. Some etiopathogenic aspects, the lack of specifity of the syndrome and the differences of the diagnosis with that of poliomyelitis are commented. We point out the lack of effect of the corticosteroids treatment and the positive effects of phisiotherapy after the acute stage.

The fetal hydantoin syndrome is a variable pattern of altered growth (pre and postnatal), mental deficiency, unusual facies, distal phalangeal hypoplasia, and other defects occurring in some infants exposed in utero to hydantoins. This altered pattern of morphogenesis is distinct from other recognized disorders. It has been reported only in the offspring of women using hydantoins. One case is presented and several aspects of the diagnosis are discussed.

Two siblings, a 3 1/2 years-old male and a 2 years-old female, with Sjögren-Larsson syndrome (SLS) are described. The SLS is a genetically determined syndrome with autosomal recessive inheritance, characterized by the three main symptoms: congenital laminar ichthyosis, spastic displegia and mental deficiency. Parental consanguinity not was found. Pregnancy and perinatal period of the two children was normal. Ichthyosis was found at birth. Spastic displegia was noted before the age of one year and mental retardation was suspected at the same time. The EEG is abnormal. Glistening dots in the optic fundus were not observed. Diagnostic features of this rare syndrome are reported.

The authors report a case of rectal perforation eight months after the placement of a ventriculo-peritoneal shunt. There was no peritonitis, but an "E. coli" meningitis was found. The patient died two hours after surgical removal of the shunt. At autopsy, a perforation by the tip of the catheter was found in the anterior face of the rectosigmoid.

Four cases of Aicardi's syndrome are presented, reporting in two of them angiographic and bone abnormalities such as arterial microaneurysm and scapula displacement, that have not been previously reported in the literature.

Twenty full-term newborns showing acute neurologic symptoms of different etiologies, with neonatal paroxistic EEG tracings are reported. Clinical and electroencephalographic follow-up at three and twelve months confirmed in every case the presence of severe neurologic sequelae. A review of the literature is made, comparing the results of other investigators with those obtained in the present study specially from an ethiologic, clinical, electroencephalographic and evolutionary points of view.