Cognitive and behavioural profile of patients with Amyotrophic Lateral Sclerosis spectrum in the Indian Context.

Introduction Amyotrophic lateral sclerosis (ALS) is characterized by motor, cognitive and behavioral impairment. There is a paucity of evidence about the cognitive/behavioral features of ALS patients from India. We aimed to investigate the cognitive/behavioral profile of ALS spectrum disorders in the Indian context. Methods Sixty patients with ALS spectrum and 40 age, gender and education matched healthy controls were recruited. The scales used were: Addenbrooke's Cognitive Examination (ACE-III) Clinical Dementia Rating (CDR) scale, and Frontal Systems Behavior Scale (FrSBe). Results The median age of the overall cohort was 54 years (IQR, 14), and male-to-female ratio was 2.5:1. Median duration of illness of the cohort was 12 months (IQR, 12). Patients were classified as ALS with: normal cognition (ALS-cn, n=21), mild cognitive or behavioral deficits (ALS-ci/bi, n=28), and Frontotemporal Dementia (ALS-FTD, n=11). ALS-cn had poorer scores compared to healthy controls in global cognition, memory, and language (p<0.05). ALS-ci/bi performed poorer than healthy controls on all cognitive domains (p < 0.05). ALS-FTD had poorer scores than healthy controls and ALS-cn on all cognitive domains (p< 0.001). Behavioral assessment showed an increase in apathy among all subtypes. ALS-FTD showed significant worsening in disinhibition and executive function compared to ALS-cn and ALSci/bi. Conclusion Our findings suggest that there are key cognitive and behavior characteristics in Indian patients with ALS spectrum. This further strengthens the evidence of a cognitive continuum in ALS and FTD in a diverse context and highlights the importance of meticulous evaluation and correct diagnosis that would assist in better management.

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