dSMA

• dHMN I: +/- UMN
  • HSPB1 - AD
  • HSPB8 - Junveile onset
  • GARS - Distal LE
  • DYNC1H1
  • 7q34
• dHMN IIB: +/- UMN
  • HSPB1 - AD
• dHMN IIA: +/- UMN
  • HSPB8: Distal LE
• dHMN II: BSCL1
• dHMN IIC: HSBP3: AR
• dHMN III: 11q13: AR, childhood onset, distal predominant
• dHMN IV: PLEKHG5: AR, childhood onset, distal predominant, diaphgragmatic paralysis, scapular winging.
• dHMN VA: GARS: AD, onset in hands, +/- UMN
• dHMN VB: BSCL2: AD onset in hands, ventilatory distress
• dHMN VI: IGHMBP2: infantile, vocal cord paralysis
• dHMN VIIB:
  • DCTN1: AD distal LE
  • TRPV4
• dHMN VII A: 2q14: UMN
• fALS4: SETX: AD distal LE
• ATP7A: X-linked distal LE
• 9p21.1: AR distal LE: Abnormal cervical spine imaging.

Benign focal amyotrophy

Scapuloperoneal

• PMP22
• TRPV4