Charcot-Marie Tooth Disease (CMT)

Synonyms

  • Hereditary motor sensory neuropathy (HMSN)
  • Peroneal muscular atrophy (PMS)

Codes

ICD-10: G60.0
ORPHA: 65753

Estimated occurrence

30:100 000 inhabitants

Etiology

CMTmay arise through new mutation or from autosomal dominant, autosomal recessive or x-linked recessive inheritance. There are many different forms of the disease, the most common of which is CMT1a which is caused by a duplication of the PMP22 gene in chromosome 17.

General symptoms

CMT is a peripheral nerve disease (polyneuropathy) that affects the muscles entailing a slowly progressing muscle weakness in legs, feet, hands and arms. Foot drop, extremely high arch, hammer toes, scoliosis and poor balance are common.  Respiratory problems may occur. The loss of nerve function often causes a pricking and a burning sensation in hands and feet. CMT belongs to the group of neuromuscular diseases.

Orofacial/odontological symptoms

CMT has no specific affect on teeth and bite. Nor are the muscles in the mouth and face generally affected since they are controlled by cranial nerves. These nerves are not usually affected by the disease. If children are extremely tired or if breathing is affected, speech may be somewhat unclear. Some medicines can cause dryness in the mouth.

Orofacial/odontological treatment

  • In the event of dryness in the mouth intensified preventive dental care is recommended.

Sources

  • The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
  • The Documentation from the Ågrenska national competence centre for rare diseases.
Updated: 2017-09-12 13:51