Monarch Disease Ontology term MONDO_0013382 (progressive demyelinating neuropathy with bilateral striatal necrosis) can be described as follows. 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. Also known as: thiamine metabolism dysfunction syndrome 4 (progressive polyneuropathy type).