|||||||
leukencefalopathie met calcificaties en cysten (aandoening)
leukencefalopathie met calcificaties en cysten
leuko-encefalopathie met calcinose en cysten
Leukoencephalopathy with calcifications and cysts
Labrune syndrome
A rare genetic cerebral small vessel disease characterized by leukoencephalopathy and cerebral calcification and cysts due to diffuse cerebral microangiopathy resulting in microcystic and macrocystic parenchymal degeneration. The condition can present at any age from early childhood to late adulthood and manifests as a progressive cerebral degeneration. Symptoms are variable, but restricted to the central nervous systems, and include, among others, slowing of cognitive performance, seizures, and movement disorder with a combination of pyramidal, extrapyramidal, and cerebellar features.
Id1186710001
StatusPrimitive
Associated morphologypathologische calcificatie
Finding sitestructuur van cerebrum
Associated morphologycyste
Finding sitestructuur van cerebrum
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetI67.8
TermOverige gespecificeerde cerebrovasculaire ziekten
SNOMED CT to Orphanet simple map542310
SNOMED CT to ICD-10 extended map
TargetI67.8
RuleTRUE
AdviceALWAYS I67.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified