Autosomal recessive hereditary disorder
Cardiovascular system hereditary disorder
Cerebral calcification
Cerebral cyst
Hereditary degenerative disease of central nervous system
Leukoencephalopathy
Small vessel cerebrovascular disease
|||||||
Leukoencephalopathy with calcifications and cysts (disorder)
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 system and include among others slowing of cognitive performance, seizures and movement disorder with a combination of pyramidal, extrapyramidal and cerebellar features.
leukencefalopathie met calcificaties en cysten
leuko-encefalopathie met calcinose en cysten
Id1186710001
StatusPrimitive
Finding siteCerebrovascular system structure
Finding siteCerebral white matter structure
Associated morphologyPathologic calcification
Finding siteCerebrum
Associated morphologyCyst
Finding siteCerebrum
ICD-10 complex map reference set
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