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Mitochondrial neurogastrointestinal encephalomyopathy

ORPHA:298· ICD-10 G71.3

Definition

Mitochondrial NeuroGastroIntestinal Encephalomyopathy (MNGIE) syndrome is characterized by the association of gastrointestinal dysmotility, peripheral neuropathy, chronic progressive external ophthalmoplegia and leukoencephalopathy.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Adolescent, Adult, Childhood