Overview of the Current Situation and Challenges about Neuromyelitis Optica Spectrum Disorders in the Republic of Macedonia

BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) are rare, progressive inflammatory disorders of the central nervous system characterized by severe, immune-mediated demyelination targeting optic nerves and spinal cord. Prior establishment of diagnostic criteria, patients were often misdiagnosed which led to delayed/inappropriate treatment and disability. Current practice involving immunotherapies is insufficient. Recent data are encouraging since the novel treatments allow effective prevention. AIM: The primary objective was to evaluate the current situation to identify challenges and develop intervention that might improve the current state as secondary objectives. METHODS: Standard questionnaire containing 22 questions was developed. Collected data were analyzed and descriptive report was created. RESULTS: Current estimated prevalence is approximately 20 NMOSD patients; trend is unknown due unavailability of patient registry. Six neurologists from one health-care institution are responsible for the whole management. Despite physician’s insufficient experience, ~80% of them are willing to switch patients into innovative treatments once available. Aquaporin-4-IgG testing is not routinely available resulting in ~30% testing rate. Approximately 80–90% of patients are on maintenance treatment with immunosuppressant, corticosteroids are used for acute relapse. Lack of novel innovative medications is evident. CONCLUSION: Current NMOSD management is challenging with significant unmet needs. Highest priorities that might provide improvement are: APQ4-IgG testing availability, establishment of patient registry, and availability of novel treatments