Charcot-Marie-Tooth disease type 4

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Charcot-Marie-Tooth disease type 4

ORPHA:64749Clinical group

Also called AR-CMT1 · Autosomal recessive demyelinating Charcot-Marie-Tooth · CMT4

What it is

A group of autosomal recessive demyelinating Charcot-Marie-Tooth disease (CMT) characterized by progressive, distally accentuated weakness and atrophy of muscles innervated by the peroneal nerve in the lower limbs, followed by weakness and atrophy of hands, sensory loss, and characteristic foot abnormalities. CMT4 is usually more severe than other forms of CMT, and the onset occurs earlier.

Key facts

Prevalence
1-5 / 10 000 (Europe)
Age of onset
Childhood, Infancy
Inheritance
Autosomal recessive
Classified as
Clinical group

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes reported in subtypes

EGR2FGD4FIG4GDAP1HK1MTMR2NDRG1PRXSBF1SBF2SH3TC2SURF1

Orphanet records these genes on 12 more specific entries under this disorder, not on this entry itself:

A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.

Cross-references

GARD 12440MONDO 0018995MONDO 18995UMLS C4082197

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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