Cephaloceles are congenital malformations with herniation of intracranial structures through a defect in the cranium. On the basis of a review of the literature and 31 personal observations the authors discuss the clinical and neuroradiological presentation of their various anatomical locations: sphenoidal, ethmoidal, frontal, occipital and parietal.

SUMMARY Regional cerebral blood flow (rCBF) was studied using SPECT (single photon emission computed tomography) with 133-Xenon in 13 patients with confirmed Sturge-Weber disease, aged 9 months to 18 years. CT scan, performed at the same time, showed evident cerebral angioma in 10 but not in three. A marked hypoperfused area was found in all patients, ranging from -32% to -72% and of the same location as the CT signs. The hypoperfusion seems to result from post ictal phenomenon as well as from chronic ischaemia. SPECT imaging is therefore a sensitive method for visualising intracranial angioma in Sturge-Weber disease and it provides an aid for diagnosis when a CT scan is not reliable.Sturge-Weber (SW) angiomatosis is a non-inherited neurocutaneous syndrome characterised by a congenital trigeminal port-wine stain and a piamater angioma.' This intracranial angioma, venous and purely meningeal,23 is embryologically derived from the neural crest. Along with the angioma, cerebral lesions, which are ischaemic in nature, are seen in the cortex underlying the angioma.4

The hamartoma of the tuber cinereum, a midline dysraphic syndrome, corresponds to a mass of normal neuronal tissue implanted in the area of the mamillary bodies. It has no evolutionary potential, but association to other cerebral malformations has been observed. On the basis of 18 personal cases and a review of the literature, the authors evaluate the actual diagnostic criteria and the incidence of different clinical signs such as precocious isosexual puberty, seizures, and mental impairment.

A review of cranial CT studies of 233 patients for the Second Medulloblastoma Trial of the International Society of Paediatric Oncology showed "typical" CT appearances in only 30% of patients. The varied appearances encountered are described with particular emphasis on atypical CT features.

This is a report of seven cases of aneurysm of the vein of Galen (AVG) with a review of the clinical and radiological aspects of 48 cases in the literature. The natural evolution and pathophysiology of this vascular malformation are discussed. The clinical signs of this condition are often misleading. The appearance of AVG on CT is pathognomic. Surveillance of the vascular malformation and associated cerebral lesions can be maintained with subsequent CT. Spontaneous thrombosis of the aneurysm was observed in three cases. The diagnosis of thrombosis of the AVG was always made at operation or by histological examination. The radiological appearance of a thrombosed aneurysm is typical.