A rare genetic motor neuron disease with characteristics of late childhood or adolescent onset of slowly progressive severe distal limb muscle weakness and wasting, in association with pyramidal signs, normal sensation and absence of bulbar involvement. Leads to degeneration of motor neurons in the brain and spinal cord.
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Extended Properties
No extended properties.
Concept Alternate Designations
SDO Designation ID
Concept Designation Description
Status
Designation Type
PHIN Preferred Term Flag
Code Flag
3763685016
Amyotrophic lateral sclerosis type 4
Active
Synonym
false
false
3763686015
ALS4 - amyotrophic lateral sclerosis type 4
Active
Synonym
false
false
3763687012
dHMN (distal hereditary motor neuropathy) with upper motor neuron signs