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April 1970

Familial Spongy Degeneration of the Brain

Author Affiliations

Shiraz, Iran
From the departments of neurology (Dr. Mahloudji), pathology (Dr. Daneshbod [Mechanic] ), and pediatrics (Dr. Karjoo), Pahlavi University and Nemazee Hospital, Shiraz, Iran.

Arch Neurol. 1970;22(4):294-298. doi:10.1001/archneur.1970.00480220008002

VAN BOGAERT and Bertrand,1 who were mainly responsible for the delineation of familial spongy degeneration of the brain as a nosological entity, have recently published a monograph covering the historical background and reviewing the literature up to 1966. They discuss the various features of the syndrome in full. The purpose of the present communication is to report a family from Iran, to bring the literature up to date, and to review critically the genetic aspects of the disorder.

Report of a Case  A 14-month-old girl was admitted into hospital with the chief complaint of fever, cough, and convulsions for eight days. She was the product of an uneventful pregnancy and fullterm delivery. She appeared normal at birth. From 1 month of age her mother noticed irritability and continual crying, suspecting "the family disease." She was unable to hold her head and was generally limp. At about 3 months

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