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Amyotrophic lateral sclerosis type 4

Disease definition

A rare, genetic motor neuron disease characterized by 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, leading to degeneration of motor neurons in the brain and spinal cord.


Classification level: Disorder
  • Synonym(s):
    • ALS4
    • Distal hereditary motor neuropathy with upper motor neuron signs
    • dHMN with upper motor neuron signs
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood, Adolescent
  • ICD-10: G12.2
  • OMIM: 602433
  • UMLS: C1865409
  • MeSH: -
  • GARD: 10502
  • MedDRA: -

Detailed information


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