Autosomal recessive hereditary disorder	Chronic brain syndrome	Chronic metabolic disorder	Disorder of basal ganglia	Hereditary disorder of nervous system	Hereditary metabolic disease	Injury of peripheral nerve	Lesion of brain	Metabolic encephalopathy	Necrosis of anatomical site	Polyneuropathy	Traumatic or nontraumatic brain injury
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Progressive polyneuropathy with bilateral striatal necrosis (disorder)
	Progressive polyneuropathy with bilateral striatal necrosis
	A rare genetic disorder of thiamine metabolism and transport with characteristics of 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. Caused by homozygous mutation in the SLC25A19 gene on chromosome 17q25.
	progressieve polyneuropathie met bilaterale striatale necrose

Id	771305006
Status	Primitive

Finding site

Peripheral nerve structure

Associated morphology	Necrosis
Finding site	Corpus striatum structure

Clinical course

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	G60.8
Rule	TRUE
Advice	ALWAYS G60.8
Correlation	SNOMED CT source code to target map code correlation not specified

Target	G23.8
Rule	TRUE
Advice	ALWAYS G23.8
Correlation	SNOMED CT source code to target map code correlation not specified