Introduction to Novel Motor Neuron Disease

Abstract

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.

Navigating ALS

“…The CDC does not require ALS cases to be reported; however, every 90 minutes, someone is diagnosed with, and someone dies from ALS. 1,2 Nurses are often on the front line of caring for patients with ALS. Nurses need to understand and be prepared to work with the patient and family to manage the significant challenges created by the disease.…”

“…Because no records on ALS have been kept it is hard to estimate the actual number of ALS cases in the US. The CDC does not require ALS cases to be reported; however, every 90 minutes, someone is diagnosed with, and someone dies from ALS 1,2…”