[Canavan disease or n-acetyl aspartic aciduria [one case]]

Canavan's disease [CD] or N-Acetylaspartic aciduria, is an autosomal recessive leukodystrophy characterized by spongy degeneration of brain. The disease is an inborn error of metabolism caused by aspartoacylase deficiency resulting from accumulation of N- acetylaspartic acid in the brain. The authors report a case in a ten-month old boy who presented with developmental delay and megalencephaly noticeable afterfour months of age. Magnetic resonance imaging of the brain show diffuse white matter degeneration. The diagnosis of CD was confirmed by nuclear magnetic resonance spectroscopy and gas chromatography-mass spectrometry

[Cerebral peduncle hydatid cyst.]

The cerebral trunk localization of the hydatid cyst is exceptional. We report the case of a 3-year old girl who has a primary localization of the hydatid cyst on the level of cerebral [peduncle]. Our patient has been admitted with signs of intracranial hypertension. The initial CT scan reveals a triventricular hydrocephaly without an obvious [obstacle] and the magnetic resonance imaging showed a cystic lesion measuring 10 mm located in the mesencephale. This lesion obstructs the Sylvius's aqueduct hence, leading to the hydrocephaly. The patient has benefited from a ventriculo-peritoneal derivation. After 4 months she presented the same symptoms. the magnetic resonance imaging and the scan revealed a lesion measuring 36mm.the patient underwent a new operation and the diagnosis of intracranial hydatid cyst was confirmed with the histopathological examination. Our patient didn't have any other localization and her hydatid serology was negative, too