What is Amyotrophic Lateral Sclerosis?

Amyotrophic Lateral Sclerosis
Immunohistochemical staining of anterior horn motor neurons and motor cortex of ALS patients with antibodies against γ-synuclein. On two adjacent sections through the spinal cord of a sALS patient (case 60) E20 goat-polyclonal antibodies detect diffuse cytoplasmic staining in the cell body of a motor neuron in the left bottom corner and large inclusions (arrows) in the cell body of a motor neuron in the right top corner (A). Similar cytoplasmic inclusions were revealed in motor neurons of case 63 (B, left panel) and case 73 (B, middle and right panels) fALS patients with C9ORF72 repeat expansion using SK109 rabbit-polyclonal antibodies. In the motor cortex of a sALS patient (case 18), γ-synuclein-positive ballooned neurites (left panel) and amorphous deposits (right panel) were detected using SK109 antibody. Scale bars = 50 μm for A, B and 10 μm for C. fALS, familial amyotrophic lateral sclerosis; sALS, sporadic amyotrophic lateral sclerosis.Gamma-synuclein pathology in amyotrophic lateral sclerosis. Peters OM, Shelkovnikova T, Highley JR, Cooper-Knock J, Hortobágyi T, Troakes C, Ninkina N, Buchman VL - Annals of clinical and translational neurology (2014). Not Altered. CC

Amyotrophic lateral sclerosis is a progressive nervous system disease that affects nerve cells in the brain and spinal cord, causing loss of muscle control. ALS is also known as Lou Gehrig’s disease, after the baseball player who was diagnosed with it.

What is the Pathology of Amyotrophic Lateral Sclerosis?

Etiology: The cause of amyotrophic lateral sclerosis is genetic in 5% to 10% of the people. For the rest, the cause isn’t known.

Genes involved: Unknwn.

Pathogenesis: The sequence of events that lead to Amyotrophic lateral sclerosis is upper motor neuron and lower motor neuron signs. 

Histology: The histology associated with amyotrophic lateral sclerosis shows ubiquinated inclusions in lower motor neurons and axonal swellings that are thought to contain disarrayed neurofilaments.

How does Amyotrophic Lateral Sclerosis Present?

Patients with amyotrophic lateral sclerosis are usually males before the age of 65 years old. After 70 years, there is no sex difference. The symptoms, features, and clinical findings associated with Amyotrophic lateral sclerosis include difficulty walking, weakness, slurred speech, cramps, and clumsiness. 

How is Amyotrophic Lateral Sclerosis Diagnosed?

Amyotrophic lateral sclerosis is diagnosed by history, physical examination, and electromyography (EMG). 

How is Amyotrophic Lateral Sclerosis Treated?

Amyotrophic lateral sclerosis is symptomatically managed. The medication Riluzole may be helpful. 

What is the Prognosis of Amyotrophic Lateral Sclerosis?

The prognosis of amyotrophic lateral sclerosis is poor and the disease is fatal.