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            : NMDA encephalomyelitis with MRI abnormalities isolated to ventral spinal cord gray matter

Zubkov, Sarah; Joshi, Puja; Shepherd, Timothy M.; Kothare, Sanjeev V.

doi:10.1212/wnl.0000000000001839

Cited by 2 publications

(1 citation statement)

References 2 publications

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“…The discovery of serum aquaporin-4 antibody (AQP4)-Abs helped explain her inflammatory myelopathy from coexisting NMOSD. The evidence in our case is highly suggestive of an ‟overlap” syndrome 5-7 (table 3).…”

Section: Discussionsupporting

confidence: 56%

Encephalitis and Myelitis in a Young Woman

Esfanhani

Wundes

Varkey

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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A 31-year-old woman presented with fever, ataxia, double vision, confusion, and auditory hallucinations, preceded by 2 weeks of cough, increased oral secretions, and dermatomal rash. Her medical history was unremarkable. Her family history was notable for first-degree relatives with autoimmune disorders, including Crohn disease and immune thrombocytopenic purpura.On initial examination, she was intubated, febrile (≥38.5°C), and comatose. She had right leg hyperreflexia and exhibited autonomic instability, characterized by labile blood pressures (systolic/diastolic [86-161/21-96] mm Hg) and heart rate (50-117 bpm), requiring intermittent administration of vasopressors.Brain MRI, head and neck CT angiogram, chest CT, and EEG were unremarkable. Serologic studies demonstrated low TSH (TSH <0.015 IU/mL), high T4 (free T4, 16.7 ng/dL), elevated antithyroid peroxidase (93 IU/mL; 0.0-8.9), thyrotropin receptor (9.67 IU/L; 0.0-1.75), and thyroglobulin antibodies (1,917 IU/mL; 0.0-1.9). Thyroid ultrasound was consistent with thyroiditis. Initial CSF studies showed a pleocytosis (90 cells/mm 3 ; 97% lymphocytes), normal total protein (39 mg/dL), and normal glucose (47 mg/dL). Despite treatment for possible CNS infection with ceftriaxone, vancomycin, and acyclovir, and with dexamethasone and methimazole for possible thyroid storm, further neurologic decline prompted transfer to our hospital.Repeat brain MRI (figure 1) showed a punctate focus of diffusion restriction with apparent diffusion coefficient correlation along the wall of the left lateral ventricle with no enhancement, whereas spinal MRI demonstrated multiple short-segment T2 hyperintensities within the cervical and thoracic spinal cord with associated enhancement (figure 1). EEG revealed background slowing without epileptiform discharges. Repeat CSF analyses revealed WBC of 139 cells/mm 3 (90% lymphocytes), mildly elevated protein (69 mg/dL), normal glucose (58 mg/dL), and 5 CSFrestricted oligoclonal bands. Extensive infectious disease workup in the CSF, blood, and sputum was negative. CSF and serum Mayo Clinic autoimmune encephalopathy panel revealed positive antiaquaporin-4 antibody and negative antimyelin oligodendrocyte glycoprotein antibody (MOG-Ab) in serum and positive anti-N-methyl-D-aspartate receptor (NMDAR) and anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) antibodies in the CSF.

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Section: Discussionsupporting

confidence: 56%