Abstract

Background: Motor neuron disease (MND) presents a considerable health challenge globally and is characterized by progressive neurodegeneration leading to debilitating weakness and eventual respiratory failure. Despite its impact, studies on MND from the Middle East and North Africa region are scarce. Considering the seriousness of the disease, it is important to recognize the demographics, clinical spectrum, and heterogeneity of MND cases to enable early diagnosis and the prompt initiation of supportive interventions to improve survival, quality of life, and physical functioning as there are presently no therapies to arrest or reverse this fatal disease. Objective: This study aimed to elucidate the demographics, clinical characteristics, management strategies, and outcomes of MND in Omani patients admitted and/or referred to a single tertiary care hospital in Muscat, Oman. Methods: A total of 29 patients diagnosed with MND who were treated and followed up at the neuromuscular disorders clinic, Khoula Hospital, Muscat, Oman, between January 2016 and April 2020 were retrospectively reviewed. Data collection and analysis included patient demographics, site of onset, duration of symptoms, clinical features, and the results of diagnostic workup. The initial primary diagnosis, clinical course follow-up, and treatments and supportive interventions were also reviewed. Furthermore, outcome measures and survival were recorded. Results: The patients had a male-to-female ratio of 1.23:1, a mean age of symptom onset of 54.7±12.7 years, and a mean duration of the disease from its onset to diagnosis of 20.3±20.9 months. Most patients (69%) presented with significant limb muscle weakness at diagnosis, with an overall revised amyotrophic lateral sclerosis functional rating scale of 35.14±10.49. MND was considered initially in 21% of the cases, while the most common primary diagnoses were cervical and lumbosacral radiculopathy (in 34% of the cases). The classic amyotrophic lateral sclerosis of spinal onset (cervical and lumbar) was the most common clinical phenotype (in 41.4% of the studied patients). The mean duration of follow-up was 48 months, and by the end of the study, 27.6% of the patients with MND were on mechanical ventilation (invasive and noninvasive), 34.5% had parenteral tube feeding, and 86% were on treatment with riluzole. Survival analysis revealed gender-related differences, with females exhibiting shorter survival times than males (26 vs. 42 months). Conclusion: As a novel, comprehensive report on the Omani MND population, this study revealed that the epidemiological and clinical characteristics of the disease in Oman were similar to those observed in international populations. However, our study indicated a higher survival rate because of the early initiation of supportive interventions. In addition, this study showed that Omani women had a longer duration of diagnostic delay and a shorter median survival time than men. We suggest a longer-term (5–10 year follow-up) multicenter study involving local and regional centers to include a larger number of patients with MND for obtaining a better understanding of the disease in this region. 

Keywords

motor neuron disease; amyotrophic lateral sclerosis, clinical phenotypes, oman