Charcot-Marie-Tooth Disease

WHAT IS IT?

Charcot-Marie-Tooth disease (hereditary motor and sensory neuropathy/HMSN) is a group of hereditary nerve disorders that involve the compromised structure/function of the myelin sheath or peripheral nerves.

WHAT CAUSES IT?

This is a genetic condition (typically autosomal dominant inheritance): there are actually multiple mutations that all can result in this condition.

WHY IS IT A PROBLEM?

It is associated with foot deformities, lower extremity weakness, and also sensory deficits.

WHAT MAKES US SUSPECT IT?

Risk factors

Family history of this condition.

Initial Presentation

Common Chief Complaints:

  • Weakness
  • Sensory issues
  • Foot deformity

Physical Exam Findings

Motor exam can reveal weakness in the extremities

Sensory exam can reveal sensory loss in the extremities

Musculoskeletal exam can show high arched feet are associated with this condition.

Characteristic high arches often seen in Charcot-Marie-Tooth disease (source)
Characteristic high arches often seen in Charcot-Marie-Tooth disease (source)
CLINICAL WORKUP

EMG can demonstrate characteristic changes that can support a diagnosis of Charcot-Marie-Tooth disease

HOW DO WE TREAT IT? 

This condition is not curable.

ARCHIVE OF STANDARDIZED EXAM QUESTIONS 

This archive compiles standardized exam questions that relate to this topic.

 
FURTHER READING

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Page Updated: 08.30.2016