Neuromyelitis optica spectrum disorders with multiple brainstem manifestations: a case report

Area of Science:

• Neurology
• Neuroimmunology

Background:

• Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare autoimmune condition affecting the central nervous system.
• Multiple simultaneous brainstem lesions are an uncommon presentation in NMOSD.

Observation:

• A 39-year-old Asian woman experienced numerous brainstem symptoms during one NMOSD attack.
• Symptoms included nausea, vomiting, vertigo, diplopia, facial palsy, hypogeusia, ophthalmoplegia, hemiplegia, dysphagia, and tonic spasms.
• Brain MRI revealed progressive, multifocal lesions within the brainstem.

Findings:

• The patient tested positive for NMO-IgG (aquaporin-4 antibody, AQP4-Ab) in serum and cerebrospinal fluid.
• Hypogeusia resolved spontaneously without immunotherapy, distinguishing it as a characteristic, reversible symptom.
• Immunosuppressive therapy effectively controlled the acute symptoms, with no relapse during a 15-month follow-up.

Implications:

• This case highlights the diverse and severe neurological deficits possible in NMOSD brainstem attacks.
• Reversible hypogeusia may serve as a key diagnostic indicator for NMOSD.
• Prompt diagnosis and immunosuppressive treatment are crucial for managing NMOSD and preventing relapses.