Disease State Diagnosis

ALS, known as 'Lou Gehrig's disease,' was originally described in 1869 and is a rapidly progressing neurodegenerative disease affecting both upper and lower motor neurons of the ventral horn of the spinal cord, motor cortex, and brainstem.86 Large axonal caliber neurons are predominantly affected, and a pathological feature is the presence of axonal inclusions of protein aggregates. As with all other neurodegenerative diseases, an inflammatory response is also a pathological feature of the disease.87

Disease onset occurs typically in midlife and, upon diagnosis, life expectancy is in the range of 3-5 years. Males appear to have a higher incidence of ALS and, overall, 1-7 people in 100000 develop the disease.86-88 In comparison to AD and PD, the incidence of ALS is low.

ALS diagnosis is based upon clinical presentation and electrophysiological and neuropathological evidence of motor neuron degeneration. ALS is classified into three variants/types: (1) sporadic (no genetic component); (2) familial

Table 2 NCEs evaluated in genetic models of Huntington's disease

Compound % Survival increase Mechanism Clinically evaluated

Table 2 NCEs evaluated in genetic models of Huntington's disease

Compound % Survival increase Mechanism Clinically evaluated

Mithramycin

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