Preferred Name

Motor Neuron Disease

Definitions

Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy ( BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation (MeSH).

ID

http://uri.neuinfo.org/nif/nifstd/birnlex_12565

createdDate

2007-10-05

definingCitation

Adams et al., Principles of Neurology, 6th ed, p1089

definingCitationURI

http://www.ninds.nih.gov/disorders/motor_neuron_diseases/motor_neuron_diseases.htm

definition

Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy ( BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation (MeSH).

hasBirnlexCurator

http://uri.neuinfo.org/nif/nifstd/readable/Bill_Bug

hasCurationStatus

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

hasDefinitionSource

http://uri.neuinfo.org/nif/nifstd/readable/MeSH_defSource

hasExternalSource

http://uri.neuinfo.org/nif/nifstd/readable/MeSH

label

Motor Neuron Disease

MeshUid

D016472

modifiedDate

2007-11-18

nifID

_8.3_2.5

preferred label

Motor Neuron Disease

prefixIRI

BIRNLEX:birnlex_12565

synonym

Motor System Disease

UmlsCui

C0085084

usageNote

Note that MeSH includes the following sub-types which cannot easily be specified as subtypes without leading to multiple inheritance (e.g., Anterior Horn Cell Disease; Familial Motor Neuron Disease; Lateral Sclerosis; Lower Motor Neuron Disease; Upper Motor Neuron Disease; Primary Lateral Sclerosis; Secondary Motor Neuron Disease). These subtypes describe either the location within the CNS axis where motor neuron degeneration is observed, the complex temporal development of that degeneration, and the hereditory nature of the disorder. In the course of many specific patient cases, the location of degenerating MNs starts in one location and then procedes to others, as is the case for some of the concrete child classess created for this parent class. In the end, a more detailed OWL representation would need to be employed where the location, timing, and putative casue of degenerating MNs is specified using ObjectProperties, and such subtypes as LOWER MOTOR NEURON DISEASE would then be inferred types. The practical problem with this approach is that terms such as ALS and LOWER MN DISEASE have a particular meaning in a clinical context. Though ALS might be inferred to be a member of the set of LOWER MN DISEASEs, since there is loss of MNs in the lower spinal cord, clinicians typically consider LOWER MN DISEASE to have a distinct set of presenting signs when compared to ALS. Clinicians do not typically consider ALS to be a type of LOWER MN DISEASE (BB: 2007-10-05)

subClassOf

http://uri.neuinfo.org/nif/nifstd/birnlex_2086

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