Neuromyelitis optica spectrum disorder (NMOSD) is a demyelinating disease of the central nervous system characterised by longitudinal extensive transverse myelitis and involvement of the optic nerve and is associated with many autoimmune disorders. The index case, a known case of Hashimoto’s thyroiditis, presented with quadriparesis and tea-coloured urine. Investigations revealed ongoing rhabdomyolysis related to autoimmune myositis and autoimmune haemolytic anaemia leading to pigment-induced acute kidney injury. Suspicion of other autoimmune disease prompted an immunological workup, which showed a positive Schirmer’s test and a positive anti-nuclear antibody (ANA) antibody test, disclosing Sjögren’s syndrome. Lack of improvement in muscle power with corticosteroids despite reduction in muscle enzymes led to an MRI of the spine, which showed longitudinal extensive transverse myelitis and involvement of the intracranial segment of the right optic nerve. These findings, along with a positive test for aquaporin-4 antibodies, confirmed NMOSD. Treatment with cyclophosphamide led to improvement in muscle power to grade 4 at discharge.
- Sjogren's syndrome
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Contributors NvS was involved in the diagnosis and management of the patient, along with drafting and preparation of the final manuscript. NlS made significant contributions in the management of the patient, along with preparation of the final manuscript. AS was involved in the diagnosis and management of the patient. NvS played a significant role in the treatment of the patient, along with preparing the initial draft.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.