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Reply from the Authors: We thank Drs. Berciano and Polo for clarifying the clinical phenotype of the seven affected individuals from the two families that they reported in 1991 as having an early onset cerebellar ataxia.2 In that report they based the designation "cerebellar ataxia, retinitis pigmentosa, and neuropathy" on a 1983 report by Tuck and McLeod that described four cases with a similar phenotype.4 Three of the cases described by Tuck and McLeod4 had a sensorineural hearing loss and radiologic evidence of cerebellar atrophy. We reported a clinicoradiologic syndrome that affected six individuals from a family of 3,338 persons that belong to a homogeneous, sectarian population.1 This community has been genetically …
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