Amyotrophic Lateral Sclerosis: Etiology, Pathology, Clinical maifestations, Diagnosis and Treatment

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Amyotrophic Lateral Sclerosis: Etiology, Pathology, Clinical maifestations, Diagnosis and Treatment

Amyotrophic lateral sclerosis is a progressive neurological disease that usually initiates after age 55 years.
ALS causes upper and lower motor neuron loss.
ALS is usually a sporadic disease, but 10% of cases are familial. The most well-known cause of familial ALS is the genetic mutation in superoxide dismutase 1 (SOD1), although other more recently discovered genetic mutations, especially C9ORF72, represent a much more common cause.
Loss of neurons occurs in the motor cortex or the anterior horn cells. Reactive gliosis is a side effect of neuron loss.
Bunina bodies are eosinophilic intracytoplasmic inclusions that occur in lower motor neurons that are not lost in ALS. Bunina bodies are pathognomonic for ALS.
Nucleus ambiguus and hypoglossal nuclei are two brainstem nuclei that can be affected, leading to significant dysarthria and dysphagia.
At initial presentation, only upper or lower motor neuron signs may be present, but the disease will progress to involve both upper and lower motor neurons.
Patients present with weakness, atrophy, fasciculations, hypotonia, hypertonia, hyporeflexia, and hyperreflexia, with the possibility of each muscle having different symptoms.
It is important to differentiate ALS from progressive muscle atrophy and primary lateral sclerosis.
Prognosis for a patient with ALS is a life expectancy of 3-5 years.
Riluzole and edaravone are two approved treatments for ALS. However, these treatments do not cure ALS.

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