Dejerine-Sottas syndrome

Orpha code: 64748OMIM code: 145900

Definicja

A clinical entity that represents a severe phenotype of Charcot-Marie-Tooth disease characterized by onset occurring in infancy, severe motor weakness, delayed motor development, extremely slow nerve conduction (< 10-12 m/s), areflexia and foot deformity. Mutations in the genes <i>PMP22</i> (17p12), <i>MPZ</i> (1q22), <i>EGR2</i> (10q21.1) and <i>PRX</i> (19q13.2) have been implicated.

Disease data
Klasyfikacja

Disease

Synonimy
Charcot-Marie-Tooth disease type 3
Choroba Charcot, Marie i Tooth typu 3
Hereditary motor and sensory neuropathy type 3
HMSN 3
Hypertrophic neuropathy of infancy
HMSN 3
HMSN III
Hereditary motor and sensory neuropathy type 3
Hereditary motor and sensory neuropathy type III
Kod ORPHA
64748
Kod OMIM
145900
Kod ICD10
G60.0
Kod ICD11
-

No additional description.

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