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Hereditary Motor Sensory Neuropathies (HMSN): Overview

See Table H-3.

Table H-3 Hereditary Motor Sensory Neuropathies (HMSN): An Overview
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Table H-3 Hereditary Motor Sensory Neuropathies (HMSN): An Overview
TypeInheritanceEponym/SynonymCharacteristics
HMSN I (classic CMT I)Autosomal dominantCharcot-Marie-Tooth Disease
  • Onset during late childhood or adolescence
  • Variable degree of nerve hypertrophy
  • Myelin sheath degeneration
  • At least three subtypes (IA, IB, IC)
HMSN I variantAutosomal dominantRoussy-Levy SyndromeCombination of HMSN type I and Friedrich's ataxia
HMSN IIAutosomal dominantCowchock Syndrome
  • Onset usually later than type I
  • Known as neuronal type
  • No nerve hypertrophy
HMSN IIIAutosomal recessive Dejerine Sottas Syndrome
  • Onset in infancy or childhood
  • Delayed motor development
  • Often severe nerve hypertrophy
HMSN IVAutosomal recessiveRefsum Syndrome
  • Slow motor nerve conduction velocity
  • Retinitis pigmentosa
  • Cerebellar signs
HMSN VMainly autosomal dominant (recessive forms exist)Strümpell-Lorrain DiseaseNeuropathy with spastic paraplegia with peroneal muscular atrophy and sensory loss
HMSN VIMitochondrial DNA transmission (from mother only)Leber Hereditary Optic Neuropathy (LHON)Neuropathy with optic atrophy
HMSN VIIOften Included in Refsum SyndromeNeuropathy with retinitis pigmentosa
X-Linked HMSNX-Linked
  • Similar to HMSN I but limited to males
  • Females can be mildly affected
HMSNLAutosomal recessiveHereditary Motor and Sensory Neuropathy-Lom Type
  • Peripheral neuropathy
  • Tendon areflexia
  • Sensory loss

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