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Start free with EleplanFriedreich ataxia
ORPHA:95Disease
Also called FA · FRDA
What it is
Friedreich ataxia (FRDA) is an inherited neurodegenerative disorder classically characterized by progressive gait and limb ataxia, dysarthria, dysphagia, oculomotor dysfunction, loss of deep tendon reflexes, pyramidal tract signs, scoliosis, and in some, cardiomyopathy, diabetes mellitus, visual loss and defective hearing.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Adolescent, Childhood
- Inheritance
- Autosomal recessive
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
6Common30–79%
28- Abnormal EKG
- Abnormal foot morphology
- Abnormality of central motor conduction
- Abnormality of the autonomic nervous system
- Abnormal saccadic eye movements
- Acrocyanosis
- Areflexia of lower limbs
- Cardiomyopathy
- Cervical spinal cord atrophy
- Clumsiness
- Dysmetria
- Falls
- Impaired vibratory sensation
- Impaired visually enhanced vestibulo-ocular reflex
- Inflammation of the large intestine
- Intention tremor
- Muscle spasm
- Muscle weakness
- Nystagmus
- Optic atrophy
- Pes cavus
- Poor fine motor coordination
- Restless legs
- Scoliosis
- Sensory axonal neuropathy
- Talipes equinovarus
- Urinary bladder sphincter dysfunction
- Urinary urgency
Sometimes5–29%
17- Anxiety
- Cerebellar atrophy
- Decreased motor nerve conduction velocity
- Depression
- Diabetes mellitus
- Dysdiadochokinesis
- Dysphagia
- Dysphonia
and 9 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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