Cerebellar ataxia, areflexia, pes cavus, optic atrophy and sensorinural hearing loss

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Disease Overview

Cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) syndrome is a neurological disorder. The syndrome gets its name from the different parts of the body it usually affects:[14082][14177][14178]

CAPOS syndrome typically begins after a fever-related illness with a sudden episode of ataxia, such as having a hard time walking or coordinating leg or arm movements. The ataxia is usually associated with generalized weakness and/or inflammation of the brain (encephalopathy). The first episode most often happens between the ages of 6 months and 5 years.[14082][14177] Pregnancy and delivery may also trigger episodes.[14178] Most people with CAPOS syndrome have one to three episodes during their lifetime.[14177] Other signs and symptoms during an episode may include low muscle tone, unusual eye movements (nystagmus or strabismus), problems with speech (dysarthria), difficulty swallowing (dysphagia), reduced or absent reflexes, and hearing loss. Some people may lose consciousness or go into a coma during an episode. Though many of the signs and symptoms of CAPOS syndrome get better as the fever and illness improve, some symptoms, including movement problems, may continue. Vision changes (optic atrophy) and sensorineural hearing loss tend to worsen over time, although the severity and rate of progression varies.[14082][14177][14178] Long-term management may include physical therapy, hearing and vision aids, and regular exams to check for changes in vision, hearing, and muscle coordination.[14082] 


Synonyms

  • CAPOS
  • CAPOS syndrome
  • Cerebellar ataxia-areflexia-pes cavus-optic atrophy-sensorineural hearing loss syndrome
  • Cerebellar ataxia - areflexia - pes cavus - optic atrophy - sensorineural hearing loss

For more information, visit GARD.

National Organization for Rare Disorders