[Hydranencephaly. Report of a new case]. / Hydranencéphalie. A propos d'un nouveau cas.

Hydranencephaly is the total or virtually total absence of the cerebral hemispheres, which are reduced to a membranous sac of glial tissue, with no ependymal coating, in an intact skull. This is rare disorder (0.5 per thousand births). It is classified as a circulatory encephalopathy. Two opposing theories are generally advanced: the encephaloclastic or destructive theory and the theory of dysontogensis or malformation, due to early disruption of organogenesis. It has many causes (vascular, viral, parasitic, genetic, toxic, estrogenic...). It appears to be readily diagnosed by ultrasound, the neurological findings may be normal at birth, but transillumination of the skull remains a simple and reliable diagnostic test. Transfontanellar ultrasound, CT scanning and anatomical confirmation alone can establish the diagnosis. The prognosis is hopeless and there is no treatment.

[The interest of CSF examinations in the etiological diagnosis of intracranial hypertension (author's transl)]. / Intérêt de l'examen cytologique du LCR dans le diagnostic étiologique de l'hypertension intra-crânienne.

The authors discuss the case of a 15-year-old girl suffering from intra-cranial hypertension. Neuroradiological examinations do not reveal any obstruction in the cerebral spinal fluid circulation. The CSF cylologic examination was the first to give etiological indications, showing the existence of malignant cells which the cerebral biopsy later enabled us to classify as glioblastoma. This case affords an opportunity to recall the three stages of the diagnosis: first, intra-cranial hypertension; secondly, chronic meningitis; thirdly, cytological difficulties in the examination of CSF in patients having undergone neuro-radiological examinations.