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A Neuroradiologic Syndrome With Variable Clinical Expression

Luis Menezes, MD; Jean Aicardi, MD; Françoise Goutières, MD

Arch Neurol. 1988;45(5):542-545.

Abstract
• We studied 12 patients with absence of the septum pellucidum associated with porencephalies. Seven patients had bilateral and five apparently unilateral defects of the cerebral mantle. The clinical manifestations were relatively mild in half of the patients and limited to isolated partial seizures in one. In one case, extensive calcification was present adjacent to the defects. In two cases, the presence of a cleft was demonstrated only by magnetic resonance imaging, although it had been suspected by computed tomography in one.

Author Affiliations
From the Department of Pediatrics and National Institute of Medical Investigation (IN-SERM), Hôpital des Enfants Malades, Paris.

Footnotes
Accepted for publication Dec 11, 1987.

Reprint requests to Hôpital des Enfants Malades, 149 rue de Sèvres, 75743 Paris Codex 15, France (Dr Aicardi).

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