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Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that results in progressive weakness of skeletal muscles including respiratory muscles. Epidemiological and clinical aspects of ALS are derived from a few world regions with very little representation of low- and middle-income countries. We therefore set out to determine the epidemiological and clinical phenotype of individuals with ALS in Ethiopia. Methods: Multicenter retrospective analysis was conducted using clinical records from ALS patients seen in Ethiopia at Tikur Anbessa Specialized Hospital and Yehuleshet specialty clinic between January 2016 and August 2021. The data collected included clinical characteristics, disease-related symptoms, a revised ALS functional rating scale, and medications. Results: Patients in Ethiopia had a younger age of onset with a mean age of disease onset of 51.9 years. 2.9% of patients had juvenile ALS, and the male-to-female ratio was almost 2:1. 4.9% had a positive family history of the disease. 68% of patients had spinal region involvement at onset, while 32% had bulbar region involvement at onset. Riluzole was used by 31% of ALS patients. 20.6% of patients had some respiratory symptoms, but none received a standard respiratory function assessment. 33.3% of patients were wheelchair-bound. Conclusion: In this retrospective study spanning 5 years, we examined the clinical phenotype of ALS in Ethiopian patients. Our findings suggest that most patients had clinically definite ALS with spinal region involvement. Further research, including genetic and epigenetic information, is necessary to understand the early onset of the disease in Ethiopia.
RESUMO
Background: Sydenham chorea is thought to be an autoimmune condition that usually develops following a group A beta-hemolytic streptococcal infection.The onset of Sydenham chorea in adults is rare and most of the adult cases usually are secondary to recurrence following childhood illness. Risk factors for chorea recurrence include irregular antibiotic prophylactic use, failure to reach remission within 6 months, and symptom persistence for longer than a year. Case Presentation: A 27-year-old young adult Ethiopian female patient with chronic rheumatic valvular heart disease for the last 8 years experienced repetitive uncontrollable movements of her extremities and torso for three years prior to her current visit. Physical examination was significant for holosystolic murmur at the apical area radiating to the left axilla and choreiform movements apparent on all limbs and trunk. Investigations were significant for mildly raised ESR, echocardiography findings of thickened mitral valve leaflets and severe mitral regurgitation. She was successfully treated with valproic acid and the frequency of penicillin injection was made every 3 weeks with no recurrence for the first 3 months follow-up period. Conclusion: We believe that this is the first case report of adult onset recurrent Sydenham chorea (SC) from a resource-limited setting. Though Sydenham chorea and its recurrence is rare in adults, it should be considered in adults after ruling out other competing differential diagnoses. Because of the lack of evidence on treatment of such rare cases, individualized mode of therapy is advised. Valproic acid is preferred for symptomatic treatment and more frequent benzathine penicillin G injections, for example every three weeks, may help in the prevention of recurrence of Sydenham chorea.
