Syndrome amyotrophic lateral sclerosis — Alz heimer's dementia

Abstract

Recently, there is more and more evidence of the presence of a cognitive defect of varying severity in the clinical picture of ALS. A rare form of the disease is the amyotrophic lateral sclerosis (ALS) — dementia complex, characterized by a combination of dementia (usually frontotemporal) with ALS symptoms. The profile of cognitive deficit in ALS includes impairment of executive functions, memory, speech and visual-spatial disorders. A literature review on this problem is presented with a description of the clinical observation of ALS–dementia syndrome (frontal variant of possible Alzheimer’s disease). A patient with a reliable diagnosis of ALS showed rapidly progressive cognitive impairments in the form of hippocampal memory impairments, speech, visual-spatial impairments, and defective executive functions, accompanied by behavioral changes (apathy, decreased criticism). Magnetic resonance imaging of the brain revealed significant atrophy of the hippocampus, frontal lobe cortex, and left temporal lobe. In the literature, there are practically no descriptions of patients with a clinical picture of a combination of AD and ALS. Difficulties in diagnosing this condition are discussed. The relationship between these neurodegenerative diseases is discussed. The presented literature data and the presented clinical observation confi rm the expediency of studying cognitive functions in patients with suspected or signifi cant ALS, on the one hand, and analysis of the state of the central and peripheral neurons in patients with neuropsychiatric disorders of the frontotemporal type, on the other hand, which can be useful for diagnostics and treatment and rehabilitation measures.