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Detailed information of HARDING ATAXIA
HARDING ATAXIA
DEFINITION:
A movement disorder characterized by progressive ataxia but no
cardiac, musculoskeletal, or endocrine abnormalities.
EPIDEMIOLOGY:
- incidence: rare
- age of onset:
- always prior to 20 years of age
- risk factors:
PATHOGENESIS:
- this disorder is often confused with Friedreich Ataxia but
there are several clinical distinctions between the two
- in Harding Ataxia there is:
- no loss of deep tendon reflexes
- absence of cardiac, musculoskeletal, and endocrine
anomalies
- better prognosis
CLINICAL FEATURES:
- early-onset ataxia
- progressive
2. Others
- soon follow after onset of ataxia
- dysarthria
- limb weakness (legs > arms)
- increased tone in affected limbs
- preservation of deep tendon reflexes
INVESTIGATIONS:
- may show cerebellar atrophy
2. EMG
- prolonged nerve conduction velocities
MANAGEMENT:
- no treatment is available
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Pediatric Database - HARDING ATAXIA
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