[Multiple cerebral telangiectasias. Apropos of an anatoma-radioclinical case]

Telangiectasias are vascular malformations corresponding to dilated capillary separated by nervous parenchyma. They are generally asymptomatic and discovered during autopsy.

We describe the case of a 2-year-old girl who was admitted for drowsiness and vomitings with craniomegaly and right hemiparesis. The cerebral imaging demonstrated large amounts of calcium in the region of pons and left thalamus with a contrast enhancement throughout these calcifications. The thalamic lesion was cystic with obstruction of the third ventricle and hydrocephalus. There were multiple calcifications scattered throughout the basal ganglia and the white matter. The latter had a low density on CT and high signal intensity on MRI (T2). The cerebral angiographic study showed a blush in the pons and the thalamus. Clinical course was fluctuating or intermittently progressive. Finally, in spite of radiotherapy and oral corticosteroid treatment, the child became quadriparesic with oculomotor palsy. She suddenly died at age 4 years and 6 months from a hemorrhage of the pons. Neuropathological study showed two clusters of telangiectasias with calcifications in the pons and the thalamus. Other calcified vascular malformations were seen in the white matter and the basal ganglia. Near the lesions, astrocytar gliosis and edema were observed. The white matter was malacic with isolated calcifications.

Cerebral telangiectasias may be multiple and may produce severe neurologic disorders, occurring in infancy with fluctuating clinical course. Arteriography may show these usually cryptic vascular malformations. This diagnostic should be kept in mind in view to the neuroradiological aspect we report here.