Child's brain最新文献

A congenital anaplastic infratentorial ependymoma, occurring in a female infant whose sister also died of brain tumor, is described. The tumor, located in the cerebellum and the fourth ventricle, is characterized by predominantly undifferentiated neuroepithelial cells associated with numerous mitoses, and partially of the cells demonstrating differentiation into ependymal cells and astrocytes. Of special interest is the fact that the elder sister of the patient had also died of a congenital glioma located in the cerebellum and the fourth ventricle, which leads to the discussion about the influences of genetic factors in the development of the familial gliomas as well as about the histogenesis of the tumor examined.

The authors report a 1-year-old Japanese boy, who was noted to have unilateral alopecia and a cloudy cornea since birth. Additional features included mild spasticity and mental retardation, DQ being 70. A non-communicating cystic lesion was found in the right middle cerebral fossa by metrizamide CT scan, and resection of the lesion by craniotomy revealed an arachnoid cyst. The case was diagnosed as encephalocraniocutaneous lipomatosis, a recently described neurocutaneous syndrome.

Of 5 children with glomerulonephritis with infected ventriculoatrial shunt, 3 had improved renal function after antibiotic therapy and removal of the infected shunt. 1 patient with endoextracapillary proliferative glomerulonephritis with 70% glomerular crescents developed a rapidly progressive renal insufficiency. Renal failure was successfully managed by hemodialysis and kidney transplantation. 1 patient died from extrarenal causes in the course of a septic episode.

Ventriculo-pleural shunting for the management of hydrocephalus was initially introduced by Ransohoff in 1954. However, because of the reported cases of pleural effusion with this procedure, the operation has not achieved popularity in the management of hydrocephalus. In the course of a review of 1,500 patients with hydrocephalus treated at the Hospital for Sick Children over the years 1960-1981, we encountered 59 patients who had been treated with a ventriculo-pleural shunt during the years 1971-1981. The commonest reason for insertion of the ventriculo-pleural shunt was a preexisting infected ventriculo-peritoneal shunt. 12 of the 59 patients developed pleural effusions, and 6 of these 12 patients were under 11 months of age. The incorporation of an antisiphon device in the shunting system seemed to decrease the possibility of a pleural effusion. 23 of the 59 patients continue to function on their inserted ventriculo-pleural shunt, and in 9 of these the shunts have been functioning for over 5 years. Ventriculo-pleural shunting seems to be a safe and simple form of diversionary CSF bypass. The risk of pleural effusion seems to be highest in the infant, but can occur at any age. However, even in the infant the ventriculo-pleural shunt provides a good temporary site for diversion of CSF when the peritoneal cavity is contaminated or hazardous to shunt function. Ventriculo-pleural shunting provides a valuable alternative for the management of hydrocephalus in those patients where the heart or the peritoneal cavity are unsuitable sites.

A 5-year-old child with the unusual association of late-onset diencephalic cachexia and pituitary insufficiency is described. At operation a suprasellar epidermoid cyst was found and excised. This curable tumor should be added in the differential diagnosis of diencephalic syndrome.

2 cases of Sturge-Weber syndrome without facial nevus are reported. The patients presented different forms of epilepsy. The diagnosis was made by computed tomography (CT) which showed typical intracranial calcifications in both occipital regions. The problems concerning the atypical and incomplete forms of the syndrome are briefly discussed. The importance of CT as a diagnostic procedure for this disease is emphasized.

Learning deficits have been noted in children with acute leukemia given methotrexate (MTX) with and without cranial irradiation (RT) for prophylaxis. A rat model has been developed to assess treatment effects on learning. The test used was altered performance of a simultaneous discrimination task in a standard operant conditioning box, employing the mean number of days needed to score 80% correct responses as the criterion. An illustrative experiment distributed suckling rats among four groups: (1) 36 controls; (2) 14 cranial RT (1,000 R); (3) 14 MTX (5 mg/kg i.p.); (4) 36 RT + MTX 24 h later, and (5) 12 undernourished controls (to match poor weight gain patterns of treated animals). Survivors were tested 10-12 weeks later: values for groups 1-5 in order were 3.9, 4.1, 4.7, 5.0 and 4.0 days. Only group 4 results were significantly different from group 1 (p = less than 0.05).

14 young patients, operated upon for opto-chiasmatic arachnoiditis by craniotomy are presented. 2 main etiopathogenetic forms (and their respective clinical equivalents) of the disease could be recognized. Only 1 postoperative death occurred, in a patient with a dominant clinical picture of intracranial hypertension. Results of surgery (craniotomy and lysis of adhesions) could be distinguished as positive (functional improvement) in 5 cases, indifferent or negative in the others, with a follow-up duration of up to 23 years. The role of the diagnostic value of the pneumoencephalogram as a basis for surgical indication is discussed: it is felt that this examination, when reported as negative, is not of sufficient value to rule out the diagnosis, which must essentially rely upon clinical data.