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Polyneuropathy, Ophthalmoplegia, Leukoencephalopathy, and Intestinal Pseudo-Obstruction:POLIP Syndrome
Ann Neurol 28:349-360, Simon,L.T.,et al, 1990
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Article Abstract
We describe 5 individuals(from three separate families)with a progressive neurological disorder characterized by sensorimotor peripheral polyneuropathy,cranial neuropathies(external ophthalmoplegia,deafness),and the syndrome of chronic intestinal pseudo-obstruction.Magnetic resonance imaging showed widespread abnormality of the cerebral and cerebellar white matter in the 2 patients studied.Autopsy examination in 3 revealed widespread endoneurial fibrosis and demyelination in the peripheral nervous system,possibly secondary to axonal atrophy,and poorly defined changes in cerebral white matter(leukoencephalopathy).The cranial nerves and spinal roots were less severely involved and the neurons in the brainstem and spinal cord were intact.The fatal gastrointestinal dysmotility was due to a severe visceral neuropathy.We suggest that these patients manifested a hereditary disorder with distinctive clinical, radiological,and neuropathological features,and propose the acronym POLIP to emphasize the distinctive tetrad of polyneuropathy,ophthalmoplegia, leukoencephalopathy,and intestinal pseudo-obstruction.
 
Related Tags
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cranial neuropathy,multiple
deafness
familial
intestinal pseudoobstruction
leukoencephalopathy
MNGIE syndrome
MRI
MRI,abnormal
neuropathology
neuropathy
neuropathy,peripheral
ophthalmoplegia
polyneuropathy
visceral neuropathy
white matter disease

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