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.