Intractable Vomiting and Hiccups: An Atypical Presentation of Neuromyelitis Optica Spectrum Disorder

Area of Science:

• Neurology
• Immunology
• Gastroenterology

Background:

• Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease primarily affecting the central nervous system.
• Symptoms like nausea and vomiting can be atypical presentations of NMOSD, potentially misdiagnosed as gastrointestinal issues.
• The area postrema (AP), crucial for regulating emesis, can be affected by demyelinating lesions in NMOSD.

Purpose of the Study:

• To report a case of NMOSD presenting with persistent vomiting in a young male patient.
• To highlight the importance of considering central nervous system disorders in cases of unexplained gastrointestinal symptoms.
• To illustrate the diagnostic pathway and successful treatment of NMOSD affecting the area postrema.

Main Methods:

• Clinical presentation of a 17-year-old male with intractable nausea and vomiting.
• Diagnostic workup including upper gastrointestinal endoscopy and neuroimaging (MRI).
• Cerebrospinal fluid (CSF) analysis for anti-aquaporin 4 (AQP4) autoantibodies.
• Treatment with intravenous methylprednisolone, intravenous immunoglobulin (IVIg), and rituximab.

Main Results:

• Neuroimaging revealed a demyelinating lesion in the area postrema (AP).
• Positive anti-AQP4 autoantibodies in CSF confirmed the diagnosis of NMOSD.
• The patient experienced significant symptom relief following immunotherapy.

Conclusions:

• Atypical presentations of NMOSD, such as persistent vomiting due to AP lesions, should be considered in differential diagnoses.
• Prompt diagnosis and aggressive immunotherapy are critical for managing NMOSD and improving patient outcomes.
• Multidisciplinary management and regular follow-up are essential for patients with NMOSD.