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Amyotrophic Lateral Sclerosis (CUI C0002736) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0002736

NCI Thesaurus Code: C34373  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: A neurodegenerative disorder characterized by progressive degeneration of the motor neurons of the central nervous system. It results in weakness and atrophy of the muscles which leads to an inability to initiate and control voluntary movements.

RADLEX Definition: A degenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, fasciculation, hyperreflexia, dysarthria, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous astrocytes and atrophy of anterior spinal nerve roots and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94) [MeSH]

GARD Definition: Amyotrophic lateral sclerosis (ALS), also referred to as "Lou Gehrig's disease," is a progressive motor neuron disease which leads to problems with muscle control and movement. There are various types of ALS, which are distinguished by their signs and symptoms and their cause. Early symptoms may include muscle twitching, cramping, stiffness, or weakness, eventually followed by slurred speech and difficulty chewing or swallowing (dysphagia). As the disease progresses, individuals become weaker are are eventually wheelchair-dependent. Death often results from respiratory failure within 2 to 10 years after the onset of symptoms. Most affected individuals have a sporadic (not inherited) form of ALS; about 5-10% have a familial (inherited) form of the condition. Familial ALS may caused by mutations in any one of several genes and the pattern of inheritance varies depending on the gene involved. Treatment is generally supportive. - this information is from GARD/ORDR/NCATS.

MEDLINEPLUS Definition: 

Amyotrophic lateral sclerosis (ALS) is a nervous system disease that attacks nerve cells called neurons in your brain and spinal cord. These neurons transmit messages from your brain and spinal cord to your voluntary muscles - the ones you can control, like in your arms and legs. At first, this causes mild muscle problems. Some people notice

  • Trouble walking or running
  • Trouble writing
  • Speech problems

Eventually, you lose your strength and cannot move. When muscles in your chest fail, you cannot breathe. A breathing machine can help, but most people with ALS die from respiratory failure.

The disease usually strikes between age 40 and 60. More men than women get it. No one knows what causes ALS. It can run in families, but usually it strikes at random. There is no cure. Medicines can relieve symptoms and, sometimes, prolong survival.

NIH: National Institute of Neurological Disorders and Stroke

CSP Definition: progressive degeneration of the neurons that give rise to the corticospinal tract and of the motor cells of the brain stem and spinal cord, resulting in a deficit of upper and lower motor neurons.

MSH Definition: A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)

Synonyms & Abbreviations: (see Synonym Details)
ALS (Amyotrophic Lateral Sclerosis)
ALS - Amyotrophic lateral sclerosis
ALS
Amyotrophe Lateralsklerose
Amyotrophic lateral sclerosis (disorder)
Amyotrophic Lateral Sclerosis [Disease/Finding]
Amyotrophic Lateral Sclerosis/Progressive Muscular Atrophy
amyotrophic lateral sclerosis
Amyotrophic sclerosis
Amytrophic lateral sclerosis
Bulbar motor neuron disease
Charcot disease
Disease, Lou-Gehrigs
Gehrig Disease
Gehrig's Disease
Gehrigs Disease
GEHRIGS DIS
LOU GEHRIG DISEASE
LOU GEHRIG DIS
Lou Gehrig's disease
LOU GEHRIGS DIS
Lou-Gehrigs Disease
MOTOR NEURON DIS AMYOTROPHIC LATERAL SCLEROSIS
Motor Neuron Disease, Amyotrophic Lateral Sclerosis
Motor neuron disease, bulbar
Sclerosis, Amyotrophic Lateral

External Source Codes: 
NCI Thesaurus Code C34373 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000020002 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
CTV3ID XUMTv SNOMEDCT_US
DATE_CREATED 04/23/1999 MEDLINEPLUS
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2015-01-22T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 4000-0117 CSP
DISEASE_IDENTIFIER_ID 17173 GARD
DISEASE_IDENTIFIER_ID 17174 GARD
DISEASE_IDENTIFIER_ID 34130 GARD
DX 19660101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
HAS_GARD_PAGE true GARD
ICE Motor neuron disease (bulbar) (mixed type) ICD9CM
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
MDA 19990101 MSH
MESH_DEFINITION A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94) NDFRT
MESH_DUI D000690 NDFRT
MESH_NAME Amyotrophic Lateral Sclerosis NDFRT
MESH_UI M0001056 NDFRT
MMR 20130708 MSH
MN C10.228.854.139 MSH
MN C10.574.562.250 MSH
MN C10.574.950.050 MSH
MN C10.668.467.250 MSH
MN C18.452.845.800.050 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
MP_HEALTH_TOPIC_URL https://www.nlm.nih.gov/medlineplus/amyotrophiclateralsclerosis.html MEDLINEPLUS
MP_OTHER_LANGUAGE_URL Spanish https://www.nlm.nih.gov/medlineplus/spanish/amyotrophiclateralsclerosis.html MEDLINEPLUS
MP_PRIMARY_INSTITUTE_URL National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/ MEDLINEPLUS
NDFRT_KIND DISEASE_KIND NDFRT
NUI N0000000376 NDFRT
ORDER_NO 05810 ICD10CM
PRIMARY_PATH 10002026$10028004$10029317$10029205$Amyotrophic lateral sclerosis$Motor neurone diseases$Neuromuscular disorders$Nervous system disorders MDR
PRIMARY_SOC 10029205 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/5786/amyotrophic-lateral-sclerosis/resources/1 GARD
REF http://orcid.org/0000-0001-5208-3432 HPO
RXAUI 3098807 RXNORM
RXAUI 3108741 RXNORM
RXAUI 3108743 RXNORM
RXAUI 3164999 RXNORM
RXAUI 3190128 RXNORM
RXAUI 3190129 RXNORM
RXAUI 3199733 RXNORM
RXAUI 3287877 RXNORM
RXAUI 5930541 RXNORM
RXCUI 1024867 RXNORM
SNOMED_CID 86044005 NDFRT
SNOMEDID DA-23010 SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS G12.2 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~G12.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS G12.21 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~G12.21 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XUMTv SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~DA-23010 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T002090 MSH
TERMUI T002091 MSH
TERMUI T002092 MSH
TERMUI T366063 MSH
TERMUI T366064 MSH
TERMUI T366065 MSH
TERMUI T753625 MSH
TERMUI T840894 MSH
TH BIOETHICS (1998) MSH
TH GHR (2014) MSH
TH NLM (1966) MSH
TH NLM (1990) MSH
TH NLM (2000) MSH
TH NLM (2010) MSH
TH NLM (2014) MSH
TH ORD (2010) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0002736

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