X-linked distal spinal muscular atrophy type 3

Orpha code: 139557OMIM code: 300489

Definition

X-linked distal spinal muscular atrophy type 3 is a rare distal hereditary motor neuropathy characterized by slowly progressive atrophy and weakness of distal muscles of hands and feet with normal deep tendon reflexes or absent ankle reflexes and minimal or no sensory loss, sometimes mild proximal weakness in the legs and feet and hand deformities in males.

Disease data
Classification

Disease

Synonyms
ATP7A-related distal motor neuropathy
DSMAX
SMAX3
dHMN typu 3 sprzężony z chromosomem X
dSMA typu 3 sprzężony z chromosomem X
Dystalna neuropatia ruchowa zależna od ATP7A
Dystalna dziedziczna neuropatia ruchowa typu 3 sprzężona z chromosomem X
DSMAX
SMAX3
X-linked dHMN3
X-linked dSMA3
X-linked distal hereditary motor neuropathy type 3
ORPHA code
139557
OMIM code
300489
ICD10 code
G12.2
ICD11 code
-

No additional description.

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