[Extramotor disorders in amyotrophic lateral sclerosis: multisystem disease?]

The purpose of this article is to review several studies on extramotor feature in Amyotrophic Lateral Sclerosis (ALS), its association with cognitive impairment, dementia, sensory and autonomic abnormalities suggesting that ALS is a multisystem disorder rather than a pure motor neuron disease. Evidence from neuroimaging, neuropsychological and histologic studies suggests that other brain regions can be involved in the pathologic process. However, the extent of the extramotor involvement remains unclear. A recent finding on the deposition of pathological TDP-43 (43 kDA transactivating responsive sequence DNA binding protein) in the Central Nervous System of Amyotrophic Lateral Sclerosis patients implies that ALS may be situated of clinicopathological spectrum of multisystem degeneration including Fronto-Temporal Lobar degeneration, that is related to the same protein.