Hereditary Motor Sensory Neuropathies (HMSN): Overview

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Table H-3 Hereditary Motor Sensory Neuropathies (HMSN): An Overview

Table H-3 Hereditary Motor Sensory Neuropathies (HMSN): An Overview

Type	Inheritance	Eponym/Synonym	Characteristics
HMSN I (classic CMT I)	Autosomal dominant	*Charcot-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 variant	Autosomal dominant	*Roussy-Levy Syndrome*	Combination of HMSN type I and Friedrich's ataxia
HMSN II	Autosomal dominant	*Cowchock Syndrome*	Onset usually later than type I Known as neuronal type No nerve hypertrophy
HMSN III	Autosomal recessive	*Dejerine Sottas Syndrome*	Onset in infancy or childhood Delayed motor development Often severe nerve hypertrophy
HMSN IV	Autosomal recessive	*Refsum Syndrome*	Slow motor nerve conduction velocity Retinitis pigmentosa Cerebellar signs
HMSN V	Mainly autosomal dominant (recessive forms exist)	*Strümpell-Lorrain Disease*	Neuropathy with spastic paraplegia with peroneal muscular atrophy and sensory loss
HMSN VI	Mitochondrial DNA transmission (from mother only)	*Leber Hereditary Optic Neuropathy (LHON)*	Neuropathy with optic atrophy
HMSN VII		*Often Included in Refsum Syndrome*	Neuropathy with retinitis pigmentosa
X-Linked HMSN	X-Linked		Similar to HMSN I but limited to males Females can be mildly affected
HMSNL	Autosomal recessive	*Hereditary Motor and Sensory Neuropathy-Lom Type*	Peripheral neuropathy Tendon areflexia Sensory loss

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