Amyotrophic lateral sclerosis
Autosomal dominant hereditary disorder
Chronic nervous system disorder
Hereditary degenerative disease of central nervous system
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Amyotrophic lateral sclerosis type 4 (disorder)
Amyotrophic lateral sclerosis type 4
dHMN (distal hereditary motor neuropathy) with upper motor neuron signs
ALS4 - amyotrophic lateral sclerosis type 4
A rare, genetic motor neuron disease characterized by late childhood- or adolescent-onset of slowly progressive, severe, distal limb muscle weakness and wasting, in association with pyramidal signs, normal sensation, and absence of bulbar involvement, leading to degeneration of motor neurons in the brain and spinal cord.
Id784341001
StatusPrimitive
Associated morphologyDegenerative abnormality
Finding siteStructure of central nervous system
Clinical courseProgressive
SNOMED CT to Orphanet simple map
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetG12.2
TermZiekte van motorische neuronen
SNOMED CT to ICD-10 extended map
TargetG12.2
RuleTRUE
AdviceALWAYS G12.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified