ICD-11 code : 8B60.0;
Preferred Label : Amyotrophic lateral sclerosis;
ICD-11 definition : Amyotrophic lateral sclerosis (ALS) is a progressive, fatal disorder in which progressive
signs of LMN and UMN degeneration are seen within one or more of the four regions:
bulbar, cervical, thoracic and lumbosacral. Electrophysiological studies may be required
to confirm lower motor neuron degeneration and to exclude alternative causes. Neuroimaging
may be performed to exclude other causes, which might explain the clinical and electrophysiological
features. Familial ALS (FALS) of autosomal dominant inheritance, constitutes 5 to
10% of ALS. The clinical profile of FALS and sporadic ALS is similar. Mutations in
the C9ORF72 and Cu/Zn superoxide dismutase (SOD1) genes constitute 50-60% of FALS.;
ICD-11 synonym : ALS - [amyotrophic lateral sclerosis]; amyotrophic sclerosis;
ICD-11 acronym : ALS;
ICD-11 inclusion : amyotrophy lateral sclerosis; amyotrophic paralysis;
Origin ID : 1982355687;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- False automatic mappings
- ICD-10 Mapping
Amyotrophic lateral sclerosis (ALS) is a progressive, fatal disorder in which progressive
signs of LMN and UMN degeneration are seen within one or more of the four regions:
bulbar, cervical, thoracic and lumbosacral. Electrophysiological studies may be required
to confirm lower motor neuron degeneration and to exclude alternative causes. Neuroimaging
may be performed to exclude other causes, which might explain the clinical and electrophysiological
features. Familial ALS (FALS) of autosomal dominant inheritance, constitutes 5 to
10% of ALS. The clinical profile of FALS and sporadic ALS is similar. Mutations in
the C9ORF72 and Cu/Zn superoxide dismutase (SOD1) genes constitute 50-60% of FALS.
