[Choroidal type aneurysmal malformation of the vein of Galen associated with Dandy-Walker malformation in an adult].

Lasjaunias differentiated true vein of Galen aneurysmal malformations (MAVG), which drain to the median prosencephalic vein of Markowski, the embryonic precursor of the vein of Galen, from the aneurysmal dilatations of vein of Galen (VGDA), which reveal venous drainage into a dilated vein of Galen, but already formed. In angioarchitectural terms, MAVG can still be divided in two subtypes: the mural form and the choroidal form, the most common. The authors describe the clinical case of a 18 years-old female, without symptoms until July 2001, who presented an episode of impaired equilibrium associated with vomits and non-specific vision disturbances.

[Merosin-positive congenital muscular dystrophy, white matter abnormalities, and bilateral posterior occipital cortical dysplasia].

Congenital muscular dystrophy (CMD) is one of the most frequent dystrophies of childhood, which is commonly characterized by neonatal muscle impairment with or without clinical evidence of central nervous system involvement. CMDs were classified into five clinically distinct forms: the two classical CMDs with and without deficit of the a2 laminin chain (merosin) caused by mutations on chromosome 6q2, the Fukuyama CMD (severe form, initially described in Japanese patients and recently linked to the chromosome 9q31-33), Walker-Warburg syndrome and the muscle-eye-brain disease described in Finnish patients. The majority of these forms have severe clinical and imagiological involvement of SNC.