Diagnostic dilemma in the management of a cervical spinal cord lesion: neuromyelitis optica spectrum disorder: a case report

Abstract

Abstract Background Neuromyelitis optica spectrum disorder, known eponymously as Devic’s disease, is a condition in which antibodies are elaborated against aquaporin water channels in the end foot processes of astrocytes due to autoimmune dysregulation thereby initiating a cascade of neuroinflammatory events that culminates in astrocytic injury, dysfunctional axonal blebs and demyelination of the central nervous system neurons. Its cervical spine symptoms may sometimes mimic a similar presentation of a cervical spine lesion. This is further validated by its core clinical magnetic resonance image feature of longitudinally extensive transverse myelitis bearing radiologic similarities with an intramedullary spinal cord lesion thereby resonating the need to maintain a high index of suspicion when evaluating patients with either condition. It is pertinent to highlight that while surgery may confer a potential benefit in patients with acute exacerbations of symptoms attributable to cervical spine compressive myelopathy due to the disease, the mainstay of treatment involves the use of steroids, immunosuppressive therapy and plasmapharesis. Case presentation We report a case of a 50-year-old Nigerian female of Yoruba ethnicity who presented with progressive weakness involving all limbs of 2 weeks’ duration. She had associated history of photophobia and a 1-week history of fever with sore throat a month prior to presentation. Cranial and cervical spine T2-weighted magnetic resonance imaging showed increase signal intensity within the right optic nerve and bulbous expansion with signal intensity noted in the C3–C7 segments of the spinal cord, respectively. She had C3–C6 cervical decompression surgery to relieve her symptom of progressive weakness of all her limbs with associated worsening difficulty with breathing. Laboratory investigations parameters were in keeping with neuromyelitis optica spectrum disorder and histological analysis of resected tissue specimen revealed features of neuroinflammation. She was placed among other medications, prednisolone, tenofovir, and azathioprine. She died on postoperative day 27 following development of sudden-onset respiratory distress. Conclusion This article highlights the need to suspect a diagnosis of Devic’s disease when evaluating patients with cervical spine lesions. It underscores the need for early diagnosis, the need for intensive ventilatory support, and the potential role of immunosuppressive therapy in improving outcomes.