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Motor neuron diseases

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Motor neuron diseases

Motor neuron diseases or motor neurone diseases (MNDs) are a group of rare neurodegenerative disorders that selectively affect motor neurons, the cells which control voluntary muscles of the body. They include amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), pseudobulbar palsy, progressive muscular atrophy (PMA), primary lateral sclerosis (PLS), spinal muscular atrophy (SMA) and monomelic amyotrophy (MMA), as well as some rarer variants resembling ALS. Motor neuron diseases affect both children and adults. While each motor neuron disease affects patients differently, they all cause movement-related symptoms, mainly muscle weakness. Most of these diseases seem to occur randomly without known causes, but some forms are inherited. Studies into these inherited forms have led to discoveries of various genes (e.g. SOD1) that are thought to be important in understanding how the disease occurs. Symptoms of motor neuron diseases can be first seen at birth or can come on slowly later in life. Most of these diseases worsen over time; while some, such as ALS, shorten one's life expectancy, others do not. Currently, there are no approved treatments for the majority of motor neuron disorders, and care is mostly symptomatic.

Infobox

Specialty
Neurology

Tables

· Diagnosis › Classification
Sporadic MNDs
Sporadic MNDs
Type
Sporadic MNDs
Sporadic amyotrophic lateral sclerosis (ALS)*
Sporadic amyotrophic lateral sclerosis (ALS)*
Type
Sporadic amyotrophic lateral sclerosis (ALS)*
UMN degeneration
Yes
LMN degeneration
Yes
Primary lateral sclerosis (PLS)*
Primary lateral sclerosis (PLS)*
Type
Primary lateral sclerosis (PLS)*
UMN degeneration
Yes
LMN degeneration
No
Progressive muscular atrophy (PMA)*
Progressive muscular atrophy (PMA)*
Type
Progressive muscular atrophy (PMA)*
UMN degeneration
No
LMN degeneration
Yes
Progressive bulbar palsy (PBP)*
Progressive bulbar palsy (PBP)*
Type
Progressive bulbar palsy (PBP)*
UMN degeneration
Yes
LMN degeneration
Yes, bulbar region
Pseudobulbar palsy
Pseudobulbar palsy
Type
Pseudobulbar palsy
UMN degeneration
Yes, bulbar region
LMN degeneration
No
Monomelic amyotrophy (MMA)
Monomelic amyotrophy (MMA)
Type
Monomelic amyotrophy (MMA)
UMN degeneration
No
LMN degeneration
Yes
Inherited MNDs
Inherited MNDs
Type
Inherited MNDs
Familial amyotrophic lateral sclerosis (ALS)*
Familial amyotrophic lateral sclerosis (ALS)*
Type
Familial amyotrophic lateral sclerosis (ALS)*
UMN degeneration
Yes
LMN degeneration
Yes
Type
UMN degeneration
LMN degeneration
Sporadic MNDs
Sporadic amyotrophic lateral sclerosis (ALS)*
Yes
Yes
Primary lateral sclerosis (PLS)*
Yes
No
Progressive muscular atrophy (PMA)*
No
Yes
Progressive bulbar palsy (PBP)*
Yes
Yes, bulbar region
Pseudobulbar palsy
Yes, bulbar region
No
Monomelic amyotrophy (MMA)
No
Yes
Inherited MNDs
Familial amyotrophic lateral sclerosis (ALS)*
Yes
Yes
· Prognosis
Amyotrophic lateral sclerosis (ALS)
Amyotrophic lateral sclerosis (ALS)
Type
Amyotrophic lateral sclerosis (ALS)
Median survival time from start of symptoms
2–5 years
Primary lateral sclerosis (PLS)
Primary lateral sclerosis (PLS)
Type
Primary lateral sclerosis (PLS)
Median survival time from start of symptoms
8–10 years
Progressive muscular atrophy (PMA)
Progressive muscular atrophy (PMA)
Type
Progressive muscular atrophy (PMA)
Median survival time from start of symptoms
2–4 years
Progressive bulbar palsy (PBP)
Progressive bulbar palsy (PBP)
Type
Progressive bulbar palsy (PBP)
Median survival time from start of symptoms
6 months – 3 years
Pseudobulbar palsy
Pseudobulbar palsy
Type
Pseudobulbar palsy
Median survival time from start of symptoms
No change in survival
Type
Median survival time from start of symptoms
Amyotrophic lateral sclerosis (ALS)
2–5 years
Primary lateral sclerosis (PLS)
8–10 years
Progressive muscular atrophy (PMA)
2–4 years
Progressive bulbar palsy (PBP)
6 months – 3 years
Pseudobulbar palsy
No change in survival

References

  1. Greenfield's neuropathology
    https://books.google.com/books?id=nrEWkAc7W7IC&pg=PA947
  2. "Motor Neuron Diseases Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS)"
    https://web.archive.org/web/20140413093035/http://www.ninds.nih.gov/disorders/motor_neuron_diseases/detail_motor_neuron_diseases.htm
  3. nhs.uk
    https://www.nhs.uk/conditions/motor-neurone-disease/
  4. healthdirect.gov.au
    https://www.healthdirect.gov.au/motor-neurone-disease-mnd
  5. Clinical and molecular aspects of motor neuron disease
    https://search.worldcat.org/oclc/860981760
  6. Neurologic Clinics
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4629510
  7. Clinical and molecular aspects of motor neuron disease
    https://search.worldcat.org/oclc/860981760
  8. openi.nlm.nih.gov
    https://web.archive.org/web/20181215225214/https://openi.nlm.nih.gov/detailedresult.php?img=3996815_bmjopen2013004353f02&query=amyotrophic+lateral+sclerosis&it=xg&lic=by&req=4&npos=5
  9. Neurologic Clinics
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3922643
  10. StatPearls
    https://www.ncbi.nlm.nih.gov/books/NBK537305/
  11. Motor System Disorders, Part I: Normal Physiology and Function and Neuromuscular Disorders
    https://doi.org/10.1016%2FB978-0-323-98818-6.00031-5
  12. ninds.nih.gov
    https://web.archive.org/web/20220508165538/https://www.ninds.nih.gov/health-information/patient-caregiver-education/fact-sheets/motor-neuron-diseases-fact-sheet
  13. Proceedings of the National Academy of Sciences of the United States of America
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6410842
  14. Neurologic Clinics
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4629510
  15. The American Journal of Medicine
    https://doi.org/10.1016%2Fj.amjmed.2018.07.012
  16. mndnsw.asn.au
    https://www.mndnsw.asn.au/about-mnd/what-is-mnd/44-mndforms.html
  17. Neuroanatomy through clinical cases
    https://search.worldcat.org/oclc/44628054
  18. StatPearls
    https://www.ncbi.nlm.nih.gov/books/NBK539814/
  19. Physical Medicine and Rehabilitation Clinics of North America
    https://doi.org/10.1016%2Fj.pmr.2012.08.022
  20. Irish Motor Neurone Disease Association
    https://imnda.ie/about-mnd/different-types-of-mnd/
  21. Journal of Neurology, Neurosurgery, and Psychiatry
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1739758
  22. motor neurone disease association
    https://www.mndassociation.org/wp-content/uploads/2015/02/an-introduction-to-mnd-booklet.pdf
  23. Neurodegeneration
    https://search.worldcat.org/oclc/958876527
  24. ICD-11 for Mortality and Morbidity Statistics
    https://icd.who.int/browse11/l-m/en#/http%3a%2f%2fid.who.int%2ficd%2fentity%2f661720689
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