Myoneurogenic gastrointestinal encephalopathy or MNGIE is a rare mitochondrial disease typically appearing between the second and fifth decades of life.

Presentation

MNGIE is a multisystem disorder causing ptosis, progressive external ophthalmoplegia, gastrointestinal dysmotility (often pseudoobstruction), diffuse leukoencephalopathy, thin body habitus, peripheral neuropathy, and myopathy.

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