CLINICAL CHARACTERISTICS OF ALS IN GEORGIAN PATIENTS.

Area of Science:

• Neurology
• Neurodegenerative Diseases

Background:

• Amyotrophic Lateral Sclerosis (ALS) is a fatal, progressive neurodegenerative disease impacting upper and lower motor neurons.
• Clinical diversity and phenotypes of ALS in the Caucasus region remain understudied.
• Establishing a unified clinical picture for ALS in Georgia is crucial.

Purpose of the Study:

• To investigate the clinical diversity and symptoms of ALS patients in Georgia.
• To assess the prevalence of different ALS subtypes and associated conditions.
• To evaluate cognitive function and quality of life in Georgian ALS patients.

Main Methods:

• A cohort of 47 ALS patients in Georgia (2018-2021) diagnosed via clinical assessment, electromyography (AWAJI), and EL ESCORIAL-Revised criteria.
• Utilized Mayo Clinic Laboratory Neurological Questionnaire, Addenbrooke's Cognitive Examination (ACE III), Frontal Behavioral Questionnaire, and ALSFRS-R for symptom, cognitive, and quality of life assessment.
• Analyzed patient demographics, age of onset, ALS subtypes (bulbar, upper limb, lower limb), and prevalence of Frontotemporal Dementia (FTD).

Main Results:

• The study identified bulbar (21.3%), upper limb (38.3%), and lower limb (40.4%) ALS types.
• Frontotemporal Dementia (FTD) was diagnosed in 12.7% of patients, with no significant correlation found between ALS forms and FTD.
• ALS presents as a multisystem disease, extending beyond motor neuron damage, indicating polysystemic degeneration.

Conclusions:

• ALS is a multisystem disorder with predominant motor neuron damage.
• Cognitive impairment is a significant feature in ALS patients.
• Routine screening for additional symptoms, particularly cognitive function, is recommended for comprehensive ALS patient management.