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A very rare congenital neurologic syndrome that presents in infancy. CAPOS is an acronym for cerebellar ataxia with areflexia, pes cavus, optic atrophy, and sensorineural hearing loss.

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Three cases (mother and two children) have been described. Autosomal dominant or maternal mitochondrial inheritance has been suggested.

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In the cases described to date, a nonspecific recurrent febrile illness triggers acute onset of neurologic symptoms with generalized hypotonia and cerebellar ataxia in previously healthy infants between the age of 12 and 24 months. Initially, full recovery between episodes is possible. Other features include progressive, nonreversible optic nerve atrophy, nystagmus, sensorineural hearing loss, and pes cavus.

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Anesthesia has not been reported in this syndrome. Assess neuromuscular function. Avoid aminoglycosides and other ototoxic drugs in the presence of preexisting hearing loss.

Nicolaides P, Appleton RE, Fryer A: Cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS): A new syndrome. J Med Genet 33:419, 1996.  [PubMed: 8733056]

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