Introduction to Novel Motor Neuron Disease
Motor neuron disease (MND) is a progressive and fatal neuromuscular disease; the most common and severe form of MND presentation is amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease. The majority of ALS patients die within 2–5 years of receiving a diagnosis. Familial ALS is...
Guardado en:
| Autores principales: | , |
|---|---|
| Formato: | Capítulo de libro |
| Lenguaje: | inglés |
| Publicado: |
IntechOpen
2020
|
| Materias: | |
| Acceso en línea: | https://www.intechopen.com/chapters/71937 |
| Etiquetas: |
Sin Etiquetas, Sea el primero en etiquetar este registro!
|
| Sumario: | Motor neuron disease (MND) is a progressive and fatal neuromuscular disease; the most common and severe form of MND presentation is amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease. The majority of ALS patients die within 2–5 years of receiving a diagnosis. Familial ALS is a hereditary form of the disease and accounts for 5–10% of cases, whereas the remaining cases have no clearly defined etiology. ALS affects persons of all ethnicities and races; currently, no curative treatment for ALS is available worldwide. ALS is also the major adult-onset MND and is clinically, pathologically, and genetically associated with fronto-temporal dementia in some cases, which is the second cause of dementia in elderly people. However, MND does not affect sphincter, sexual function, or eye movements. MND is the most common degenerative disorder affecting the upper and lower motor neurons at the same time. Most of the patients presenting MND in our series complained of muscle weakness, muscle wasting, fasciculation, and spasticity plus lower cranial nerve disturbances. According to our bibliographic studies, apart from nusinersen, it seems to be that riluzole and edaravone also improve motor neuron function by acting on SK channels. |
|---|---|
| ISBN: | 978-1-83880-773-3 |
| DOI: | 10.5772/intechopen.91921 |