Authors: Wei Kok Poh , Zaidatul Wahida Abd Wahab , Yong Chin Kok

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Article Info

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Abstract

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Background: Cortical involvement and leptomeningeal enhancement are rare in neuromyelitis optica spectrum disorder (NMOSD). Cerebrospinal fluid (CSF) total protein is usually raised typically ranged between 0.5 and 1.5 g/l. Case Presentation: A 22-year old lady who was previously well presented with a subacute onset of bilateral lower limb weakness for 1 week, which progressed in an ascending pattern involving the upper limbs. Magnetic resonance imaging spine showed longitudinally extensive transverse myelitis as well as leptomeningeal enhancement of the entire spine. CSF protein was high with 3.33 g/l. Serum Aquaporin-4 IgG was positive. After failed steroid therapy, she responded dramatically with plasma exchange. Conclusion: The presence of very high CSF protein and the presence of extensive leptomeningeal enhancement should not dissuade clinicians from considering the diagnosis of NMOSD, but merely reflects its severity and may need aggressive immunotherapy such as combination of steroids and plasma exchange.

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Keywords: Neuromyelitis optica spectrum disorders, spinal leptomeningeal enhancement, aquaporin-4 IgG, longitudinally extensive transverse myelitis, green

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