[Muscle and brain biopsy in a case of mitochondrial encephalomyopathy. Demonstration of a mitochondrial vasculopathy]. / Biopsies musculaire et cérébrale d'un cas d'encéphalomyopathie mitochondriale. Mise en évidence d'une vasculopathie mitochondriale.

The authors report the case of a 14-year-old boy who presented with shunted growth and low weight gain, asthenia with fatiguability on effort, clinical signs of myopathy, ataxia, epilepsy, deafness, loss of visual acuity and reduced intellectual capacities. Muscle biopsy revealed numerous ragged-red fibres and mitochondrial anomalies at ultrastructural examination. Cerebral biopsy showed spongiform changes. Both muscular and cerebral biopsies showed vascular lesions: swollen endothelial cells. Mitochondria increased in number with concentric whorls of cristae were observed in pericytes and smooth muscle cells. This mitochondrial vasculopathy could explain the neurological symptoms.

[Cerebrovascular ischemic accident and congenital protein C deficiency in children: apropos of a case]. / Accident vasculaire cérébral ischémique de l'enfant et déficit congénital en protéine C: à propos d'une observation.

The authors report the case of a 15-month-old boy with left hemiparesia related to a right sylvian ischemic incident, confirmed by CT scan and carotid angiography. Long-term evolution was favourable with anti-aggregant platelet treatment. A large constitutional protein C deficiency (40%) proved to be the etiology. Protein C is a powerful, physiological, vitamin K-dependent anti-coagulant which neutralizes activated factors V and VIII. Congenital protein C deficiency is responsible for recurrent spontaneous thrombosis affecting the young, mainly in the venous areas, since arterial incidents are less frequent. Protein C assays should be included in the systematic etiologic check-up for thrombotic incidents in children.

[Cerebral abscess caused by Proteus mirabilis in the neonatal period]. / Abcès cérébraux à Proteus mirabilis de la période néonatale.

Eight cases of cerebral abscesses due to Proteus mirabilis in the newborn period are reported. The authors emphasize the insidiousness of the clinical features, and the use of ultrasound screening for an early recognition, essential for the therapeutic results.

[Medulloblastoma of the posterior cranial fossa. A second tumor after Hodgkin's disease]. / Médulloblastome de la fosse postérieure. Deuxième tumeur après une maladie de Hodgkin.

The case of a child presenting with Hodgkin's disease (stage I A, mixed cellular type) at the age of 23 months is reported. Chemotherapy (six treatments with MOPP) enabled a clinical remission, but an in situ relapse with the same histological pattern was observed at the age of 3 years and 9 months. After local irradiation, a second complete remission was observed. At the age of 6, a clinical picture of intraskull hypertension with ataxia, lead to the diagnosis of posterior cavity tumor. Complete excision was performed and the pathological examination showed a typical medulloblastoma. Adjuvant radiotherapy was carried out. Eight months later, a fatal plurifocal medullar relapse was observed. Second tumors in Hodgkin's disease are well known. However, this new case appeared to be the first report of a medulloblastoma after Hodgkin's disease.