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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: Tuberculosis (TB) is the leading cause of morbidity and mortality in low and middle income countries (LMIC). Approximately 50% of cases of skeletal TB involve the spine. Failure to identify and treat these areas of involvement at an early stage may lead to serious complications such as vertebral collapse, spinal compression, and spinal deformity. The clinical and radiologic features of Pott's disease may mimic other spine diseases such as, metastatic lesions and other infectious etiologies, this is especially imperative in older patients. CASE REPORT: We report a 60-year-old right handed male patient presented with back pain, paraparesis, and sensory symptoms 2 weeks duration. He has history of dry cough, fatigue, and reduced appetite, but no history of weight loss, fever, night sweat, and bowel/bladder incontinence. No contact history with TB patients. He has a borderline hypertension and diabetes mellitus. Serology for HIV was negative. Thoraco-lumbar magnetic resonance image (MRI) showed destruction of L2 and L3 vertebral body and the inter-vertebral disc; with T2 hyper and T1 hypointensity of the affected vertebral bodies. Probable tuberculous spondylitis with paraparesis was considered and the patient was initiated on antituberculous regimen and short course steroid therapy. After five months treatment, the patient showed significant clinical and radiological improvement. CONCLUSION: In summary, the present case describes, a patient with Pott's paraplegia due to probable spine tuberculosis and showed significant clinical and radiological improvement following initiation of antituberculous drugs and short course of steroid; indicating the crucial role of imaging in the diagnosis of TB, especially in resource limited settings.
