Extensive brain calcifications, leukodystrophy, and formation of parenchymal cysts: a new progressive disorder due to diffuse cerebral microangiopathy.

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

Neurology

journal_title

Neurology

authors

Labrune P,Lacroix C,Goutières F,de Laveaucoupet J,Chevalier P,Zerah M,Husson B,Landrieu P

doi

10.1212/wnl.46.5.1297

subject

Has Abstract

pub_date

1996-05-01 00:00:00

pages

1297-301

issue

5

eissn

0028-3878

issn

1526-632X

journal_volume

46

pub_type

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