Motor neuron disease

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The motor neuron diseases (MND) are a group of neurological disorders that selectively affect motor neurons, the cells that control voluntary muscle activity including speaking, walking, swallowing, and general movement of the body. They are generally progressive in nature, and cause increasingly debilitating disability and, eventually, death.

Terminology

Terms used to describe the motor neuron diseases can be confusing. In the United Kingdom, motor neurone disease (sometimes spelt as motor neuron disease) refers both to amyotrophic lateral sclerosis and to the broader spectrum of related motor neuron disorders that includes progressive muscular atrophy, primary lateral sclerosis, and progressive bulbar palsy. In the United States, amyotrophic lateral sclerosis (ALS) is commonly used both for the specific disorder amyotrophic lateral sclerosis (frequently referred to there as Lou Gehrig's disease) and as a blanket term for the entire spectrum of MND.[1] To avoid confusion, the annual scientific research conference dedicated to the study of MND is called the "International ALS/MND Symposium".

Although MND refers to a specific subset of pathologically similar diseases, there are numerous other afflictions of motor neurons that are pathologically distinct from MND, have a different clinical course and should not be confused with MND, such as spinal muscular atrophy, spinobulbar muscular atrophy, Charcot–Marie–Tooth disease, and many others.

Classification

In the most common classification, the term "motor neuron disease" applies to the following five disorders which affect either upper motor neurons (UMN) or lower motor neurons (LMN), or both:

Type UMN degeneration LMN degeneration
Amyotrophic lateral sclerosis (ALS) yes yes
Primary lateral sclerosis (PLS) yes no
Progressive muscular atrophy (PMA) no yes
Progressive bulbar palsy (PBP) no yes – bulbar region
Pseudobulbar palsy yes – bulbar region no

Even though diseases known as spinal muscular atrophies, including the most common spinal muscular atrophy (SMA), affect motor neurons and are classified as MND by the disease terminology classification system Medical Subject Headings (MeSH), they are not classified as such by the tenth International Statistical Classification of Diseases and Related Health Problems (ICD-10) published in 1992 and thus are not discussed in this article.

References

External links

Template:CNS diseases of the nervous system

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