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Start free with EleplanRoussy-Lévy syndrome
ORPHA:3115Disease
Also called Hereditary areflexic dystasia, Roussy-Lévy type
What it is
A rare demyelinating hereditary motor and sensory neuropathy characterized by prominent gait ataxia, pes cavus, tendon areflexia, distal limb weakness, tremor in the upper limbs, distal sensory loss, kyphoscoliosis, and progressive muscle atrophy. The disease becomes symptomatic in infancy or childhood, mode of inheritance is autosomal dominant.
Key facts
- Age of onset
- Childhood, Infancy
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Signs and symptoms
Common30–79%
21- Acute demyelinating polyneuropathy
- Areflexia
- Clumsiness
- Decreased motor nerve conduction velocity
- Distal amyotrophy
- Gait ataxia
- Gait disturbance
- Impaired pain sensation
- Impaired temperature sensition
- Impaired vibratory sensation
- Kyphoscoliosis
- Limb ataxia
- Lower limb muscle weakness
- Nystagmus
- Pes cavus
- Postural tremor
- Scoliosis
- Sensorimotor neuropathy
- Somatic sensory dysfunction
- Talipes equinovarus
- Unsteady gait
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.
Genes
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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