Abstract
Cases of the juvenile form of familial amaurotic idiocy, in this paper called juvenile cerebroretinal degeneration, are reported in the literature from time to time. Recently, publications have appeared concerning the electroencephalographic findings in healthy relatives of these patients.1-3Hoffman1reports a family of 4 children in which 2 sisters showed the usual clinical and electroencephalographic findings of the disease, and 2 healthy, younger siblings showed some abnormal tracings, although there were no neurological symptoms or signs. The parents showed no clinical or electroencephalographic abnormality. Jervis2has reported a patient with the disease who had a younger sibling, 4 years old, with a definitely abnormal electroencephalogram, although the child was intellectually normal. It is possible that the deviations in the electroencephalogram of the healthy siblings are preclinical manifestations of the disease. These observations remind one of the characteristic blood findings in such patients first described by Bagh
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