Abstract:
:A new cerebral disorder, described in three unrelated children, has recognizable clinical, radiologic, and neuropathologic findings. The onset occurs from early infancy to adolescence with slowing of cognitive performance, rare convulsive seizures, and a mixture of extrapyramidal, cerebellar, and pyramidal signs. CT shows progressive calcifications in the basal and cerebellar gray nuclei and the central white matter. MRI reveals diffuse abnormal signals of the white matter on T2-weighted sequences. A special feature is the development of parenchymal cysts in the cerebellum and the supratentorial compartment, leading to compressive complications and surgical considerations. Neuropathologic examination of surgically removed pericystic samples reveals angiomatous-like rearrangements of the microvessels, together with degenerative secondary changes of other cellular elements. Both the anatomic findings and the course of the disease suggest a constitutional, diffuse cerebral microangiopathy resulting in microcystic, then macrocystic, parenchymal degeneration.
journal_name
Neurologyjournal_title
Neurologyauthors
Labrune P,Lacroix C,Goutières F,de Laveaucoupet J,Chevalier P,Zerah M,Husson B,Landrieu Pdoi
10.1212/wnl.46.5.1297subject
Has Abstractpub_date
1996-05-01 00:00:00pages
1297-301issue
5eissn
0028-3878issn
1526-632Xjournal_volume
46pub_type
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