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Neuromyelitis Optica Spectrum Disorder with marked CSF Protein Elevation and Bilateral Thalamic Involvement
Conference poster   Peer reviewed

Neuromyelitis Optica Spectrum Disorder with marked CSF Protein Elevation and Bilateral Thalamic Involvement

D Kaval, Christopher John Morici, D Kotekal, A Prahalad and T F Scott
Multiple sclerosis, v 32(2_suppl), pp 168-168
May 2026
DOI: https://doi.org/10.1177/13524585261430792

Abstract

Background & Objectives: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy associated with aquaporin-4 (AQP4) antibodies. Methods: 74-year-old woman with a history of normal pressure hydrocephalus (NPH) status post ventriculoperitoneal (VP) shunt placement presented after a ground-level fall, preceded by one week of right leg weakness and dysesthesia. Neurological examination revealed progressive asymmetric quadriparesis, distal sensory loss, dysesthesias, and right Horner syndrome. Brain MRI demonstrated bilateral thalamic signal abnormalities with patchy enhancement and pachymeningeal enhancement along the bilateral convexities, interhemispheric falx, and tentorial leaflets. Spinal MRI revealed a longitudinally extensive T2 hyperintensity from the cervicomedullary junction to T9. Cerebrospinal fluid (CSF) showed markedly elevated protein (>600 mg/dL), pleocytosis (WBC 111/µL; 66% neutrophils, 5% lymphocytes), normal glucose, and negative infectious studies. Multiple cytology specimens were negative for malignant cells. Shunt series were normal without malfunction. Aquaporin-4 antibody testing via cell-based assay was positive in serum, confirming NMOSD. The patient received high-dose corticosteroids, plasma exchange, and eventually started on rituximab. Patient discharged to inpatient rehab facility. Results: This case highlights several atypical features in a case of NMOSD. First, the degree of CSF protein elevation was unusual. Protein elevation exceeding 100 mg/dL is frequently observed during NMOSD relapses; however, values exceeding 600 mg/dL are exceedingly rare. The presence of a VP shunt further complicated the interpretation, as shunts can chronically raise CSF protein levels, though typically only mildly. Therefore, while the VP shunt may have contributed to a degree, the marked elevation of protein in this case remains an outlier. Second, pachymeningeal enhancement is not a typical radiographic feature of NMOSD and was likely a reactive finding due to the presence of a VP shunt. Lastly, bilateral thalamic signal abnormalities, while infrequent, fall within the recognized diencephalic manifestations of NMOSD. The initial diagnostic uncertainty in this case required careful workup to exclude alternative etiologies before NMOSD could be confirmed. Conclusions: Awareness of atypical imaging and CSF patterns are important to prevent misdiagnosis.

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