Electroencephalographic Findings in Two Families with Friedreich's Ataxia

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Abstract
Seven members, belonging to 2 families, suffering from Friedreich''s ataxia were studied electro-encephalographically; 4 displayed the usual clinical symptoms (hyperreflex form), while 3 were unimpaired from a clinical point of view. The patients, as well as their relatives displayed no clinical signs showed abnormal EEG tracings with a marked irritability. None of the cases was subject to epileptic seizures or psychic disturbances. In one family, the feature of irritability was predominant in EEG alterations, while in the second one the immaturity prevailed. A similarity exists between the EEG tracings of the subjects not affected with the disease and those displaying Friedreich''s ataxia and belonging to the respective family. Comments are made on the importance of EEG findings in Friedreich''s ataxia, from the nosological and genetic viewpoint (detection of latent forms). The EEG abnormalities observed in the clinically unimpaired parents may represent the equivalent manifestations of the same abiotrophic gene (familial alternance of the phenotype).

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