Distal hereditary motor neuronopathy

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  • Heterogeneous group of autosomal dominant inherited conditions (in past recessive conditions also classified by this name):
    • HMN type I
    • HMN type II
      • HMN2A - juvenile onset
      • HMN2B - adult onset
    • HMN types III and IV now classified as DSMA3
    • HMN type V (HMN5) - upper limb involvement
    • HMN type VII - with vocal cord paralysis
    • HMN type VI (dHMN6, DSMA1 or HMN6) - mutations in the IGHMBP2 gene at 11q13.2-q13.4 that codes for cardiac transcription factor-1.
      • HMN7A
      • HMN7B
  • Anterior horn cell degeneration
  • Patients have:
    • Progressive distal motor weakness
    • Muscular atrophy
    • No sensory impairment