Motor predominant peripheral neuropathy

Evidence-based neurology checklist on motor predominant peripheral neuropathy: Hereditary causes Motor neurone disease (MND) Spinal muscular atrophies (SMA) Spinal and bulbar muscular atrophy (SBMA, Kennedy’s disease) Distal hereditary motor neuropathies (dHMNs) Porphyria PIGG recessive variants…

Hereditary causes

  • Motor neurone disease (MND)
  • Spinal muscular atrophies (SMA)
  • Spinal and bulbar muscular atrophy (SBMA, Kennedy’s disease)
  • Distal hereditary motor neuropathies (dHMNs)
  • Porphyria
  • PIGG recessive variants

Sporadic causes

Infectious causes

Drug induced

Immune-mediated

Other causes

References

  1. Garg N, Park SB, Vucic S, et al. Differentiating lower motor neuron syndromes. JNNP 2017; 88:474-483.
  2. Verschueren A. Motor neuropathies and lower motor neuron syndromes. Rev Neurol (Paris) 2017; 173:320-325.
  3. Alport AR, Sander HW. Clinical approach to peripheral neuropathy: anatomic localization and diagnostic testing. Continuum (Minneap Minn) 2012; 18:13-38.
  4. Willison HJ, Winer JB. Clinical evaluation and investigation of neuropathy. JNNP 2003; 74(Suppl II):ii3-ii8.
  5. Record CJ, O'Connor A, Verbeek NE, et al. Recessive variants in PIGG cause a motor neuropathy with variable conduction block, childhood tremor, and febrile seizures: expanding the phenotype. Ann Neurol 2025; 97:388-396.

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