The clinical profile of amyotrophic lateral sclerosis patients presenting to the National Hospital of Sri Lanka

OBJECTIVES Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease. Published data on ALS in Sri Lanka is scarce. The aim of this study was to describe the clinical profile of ALS patients presenting to the National Hospital of Sri Lanka(NHSL). METHODS A descriptive cross-sectional study was carried out in 33 consecutive patients with “clinically definite ALS” according to revised El-Escorial and Awaji criteria, who presented from May 2018-May 2020 to two neurology units at NHSL. RESULTS The mean age was 59.7 (±12.3) years while 57.6%(n=19) were males. The average duration for diagnosis was 15.48 (±12.4) months. The commonest first symptom was upper limb weakness 39.4%(n=13) followed by lower limb weakness 33.3%(n=11) and bulbar symptoms 27.3%(n=9). According to King’s Staging System, 18.2%(n=6) were in stage 1, 39.4%(n=13) were in stage 2, 36.4%(n=12) were in stage 3 and 3%(n=1) each were in stage 4A and 4B at the time of diagnosis. None had a family history of ALS. Four were treated with Riluzole. Out of all, 45%(n=15), 24.2%(n=8) and 6.1%(n=2) were referred for physiotherapy, occupational therapy and psychiatry assessment respectively. Seventy five percent (18/24) patients with bulbar symptoms, were referred to speech therapists and one had gastrostomy. Advance directives were not discussed with any of the patients. CONCLUSION The ALS patients presenting to the NHSL had a mean age of 59.7yrs with a slight male preponderance. The average duration taken for diagnosis was 15.5months while the commonest first presentation was upper limb weakness. Most present in King’s stage 2 or 3 and only a few were treated with disease modifying agents. Multidisciplinary care provided and management of disease related complications were suboptimal. Discussing about advance directives with ALS patients, is yet not part of Sri Lankan practice. This emphasizes the need to enhance standards of care for ALS patients to improve their quality of life.