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Extensive brain calcifications, leukodystrophy, and formation of parenchymal cysts

A new progressive disorder due to diffuse cerebral microangiopathy

Department of Pediatrics, Hôpital Beclére, Clamart, France (Dr. Labrune) (Dr. Chevalier)
Department of Radiology, Hôpital Beclére, Clamart, France (Dr. de Laveaucoupet)
Unit of Pediatric Neurology, Hôpital Necker-Enfants Malades, Paris, France (Dr. Goutiéres)
Department of Neurosurgery, Hôpital Necker-Enfants Malades, Paris, France (Dr. Zerah)
Laboratory of Neuropathology, Hôpital de Bicêtre, France (Dr. Lacroix) (Dr. Landrieu)
Department of Pediatric Radiology, Hôpital de Bicêtre, France (Dr. Husson)
Department of Pediatric Neurology, Hôpital de Bicêtre, France. (Dr. Landrieu)

Address correspondence and reprint requests to Dr. P. Labrune, S de Pédiatrie, Hôpital A, Beclére 92140, Clamart, France.

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 T,-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.

Received March 21, 1995. Accepted in final form September 18, 1995.

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