CEREBELLAR-ATAXIA, AREFLEXIA, PES CAVUS, OPTIC ATROPHY, AND SENSORINEURAL HEARING-LOSS (CAPOS) - A NEW SYNDROME

There are a large number of well recognised syndromes comprising cereb ellar ataxia in association with other neurological features. We repor t three family members who presented with a relapsing, early onset cer ebellar ataxia, associated with progressive optic atrophy and sensorin eural deafness. AU three patients have areflexia (in the absence of a peripheral neuropathy), a pes cavus deformity, and show varying degree s of severity. Extensive neurological investigations have been normal, and the aetiology and pathophysiology of this disorder remain unclear . This may represent a separate syndrome of early onset cerebellar ata xia with associated features (''cerebellar ataxia plus''), which is Li kely to either have an autosomal dominant or maternal mitochondrial pa ttern of inheritance. The recognition of this association under the ac ronym of CAPOS (cerebellar ataxia, areflexia, pes cavus, optic atrophy , and sensorineural deafness) may help in the delineation of a new syn drome.