Cognitive Assessment of A Sample of Egyptian Patients with Amyotrophic Lateral Sclerosis

Abstract

Amyotrophic lateral sclerosis (ALS) is an idiopathic and fatal neurodegenerative disease of the motor system. ALS was first identified by Jean Martin Charcot in 1874 as a heterogeneous neurodegenerative disease, characterized by degeneration of both upper motor neurons. ALS patients are categorized according to the age of onset into juvenile, early and late onset. Juvenile onset subtype is known to occur before the age of 25 years. It is associated with a strong positive family history and a slow progressive course. Early onset subtype affects patients before the age of 45 years, resulting in an overlap between these two subtypes. It is observed that earlier age of onset is associated with a stronger genetic risk. Patients homozygous dominant for mutation in SOD1 gene suffer from more aggressive type of ALS with earlier onset. Late onset subtype, affecting patients older than 45 years, is the most common. Lately, cognitive impairment in amyotrophic lateral sclerosis (ALS) has become a focus of detailed clinical and neurophysiological research, as a noticeable proportion of ALS patients present with a full-blown picture of frontotemporal dementia (typically behavioral variant), which is considered as the most common form of cognitive impairment in ALS with an average percentage of 25-33 % of the patients being affected. Meanwhile, the main affected cognitive functions in ALS patients are executive functions, emotional processing, social cognition, verbal fluency, verbal memory, language, and behavioral changes in form of apathy. Aim: 1. Comparing cognitive functions in ALS patients and healthy controls. 2. Comparing cognitive functions in patients with early and late onset amyotrophic lateral sclerosis. Method: Type of Study: This was a case control study at the Neuromuscular Unit at the Neuropsychiatry Department, Faculty of Medicine, Ain shams University, which involved 30 patients fulfilling the criteria of diagnosis of ALS (15 early onset cases, 15 late onset cases) and 30 healthy controls of the same age, sex and education during the study period. The study population was assessed using Stanford-Binet Intelligence Scales for Adults (Arabic Version), GHQ scale (Arabic version), ALS FRS R, King’s staging scale and ECAS.