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