Motor neuron disease

Motor neuron disease
Synonyms motor neurone disease[1]
spinal diagram
Specialty Neurology

A motor neuron disease (MND) is a neurodegenerative disorder that selectively affects motor neurons, the cells that control voluntary muscles of the body.[2]

Motor neuron diseases include:

The different classes of spinal muscular atrophy (SMA) are sometimes included in the group by some neurologists; however spinal muscular atrophies are distinct diseases with clear genetic causes.[3][4]

Terminology

In the United States, the term is often used to denote ALS, the most common disorder in the group.[3] In the United Kingdom, the term is spelled motor neurone disease and is sometimes used for the entire group,[5] but can also refer specifically to ALS.[6]

While MND refers to a specific subset of similar diseases, there are numerous other diseases of motor neurons that are referred to collectively as "motor neuron disorders", for instance disease belonging to spinal muscular atrophies.[2] However, they are not classified as "motor neuron diseases" by the tenth International Statistical Classification of Diseases and Related Health Problems (ICD-10), which is the definition followed in this article.

Classification

Motor neuron diseases affect either upper motor neurons (UMN) or lower motor neurons (LMN), or both:

TypeUMN degenerationLMN degeneration
Amyotrophic lateral sclerosis (ALS)YesYes
Hereditary spastic paraplegia (HSP)Yes[7]No[7]
Primary lateral sclerosis (PLS)YesNo
Progressive muscular atrophy (PMA)NoYes
Progressive bulbar palsy (PBP)NoYes, bulbar region
Pseudobulbar palsyYes, bulbar regionNo

See also

References

  1. ↑ Neilson, Stuart; Rose, Frank Clifford. Motor Neurone Disease: The 'at Your Fingertips' Guide. Class Publishing Ltd. p. 2. ISBN 9781859590478. Retrieved 4 August 2016.
  2. 1 2 Ellison, edited by Seth Love, David N. Louis, David W. (2008). Greenfield's neuropathology (8th ed.). London: Hodder Arnold. p. 947. ISBN 9780340906811.
  3. 1 2 "Motor Neuron Diseases Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS)". www.ninds.nih.gov. Archived from the original on 13 April 2014. Retrieved 7 November 2010.
  4. ↑ Kimura, Jun (2013). Electrodiagnosis in Diseases of Nerve and Muscle: Principles and Practice. Oxford University Press. p. 599. ISBN 9780199969296.
  5. ↑ "An introduction to motor neurone disease (MND)" (PDF). motor neurone disease association. 2015.
  6. ↑ "Motor neurone disease". nhs.uk. Retrieved 2018-02-07.
  7. 1 2 "A Look at Upper Motor Neuron Diseases Hereditary Spastic Paraparesis and Primary Lateral Sclerosis" (PDF). sp-foundation.org. Archived from the original (PDF) on 25 March 2017. Retrieved 25 March 2017.
Classification
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