Autosomal recessive spastic paraplegia type 39

Orpha code: 139480OMIM code: 612020

Definition

A rare autosomal recessive complex spastic paraplegia characterized by upper motor neuron involvement and peripheral neuropathy with an onset between childhood and early adulthood. Patients present with progressive spasticity, hyperreflexia, and distal upper and lower muscle wasting. Reduced cognitive functioning and cerebellar ataxia have also been reported. MR imaging may reveal cerebellar and/or spinal cord atrophy.

Disease data
Classification

Disease

Synonyms
SPG39
Paraplegia spastyczna z powodu mutacji NTE
Spastyczna paraplegia z powodu mutacji neuropatycznej esterazy
SPG39
Spastic paraplegia due to NTE mutation
Spastic paraplegia due to neuropathy target esterase mutation
ORPHA code
139480
OMIM code
612020
ICD10 code
G11.4
ICD11 code
8B44.01

No additional description.

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