Progressive polyneuropathy with bilateral striatal necrosis
Progressive polyneuropathy with bilateral striatal necrosis is a rare genetic disorder of thiamine metabolism and transport characterized by the childhood-onset of recurrent episodes of flaccid paralysis and encephalopathy associated with bilateral striatal necrosis and chronic progressive axonal polyneuropathy with proximal and distal muscle weakness areflexia contractures and foot deformities.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version December 2023
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Progressive polyneuropathy with bilateral striatal necrosis?
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