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A Case of Progressive Myelopathy in a Middle-aged Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 48-year-old woman presented to the emergency department with a 2-month history of progressive lower extremity weakness, sensory loss, and sphincter dysfunction. In the weeks prior to onset, she traveled to Yosemite National Park but denied any particular infectious exposures or symptoms including rash. She reported no personal or family medical history. Although she did consume a variety of nutraceuticals, she denied medication and recreational drug use.

On examination, mental status, cranial nerves, and upper extremities were normal. Lower extremities were significant for her right side having greater pyramidal weakness than the left, with brisk reflexes and right ankle clonus, as well as a sensory level to pinprick at T6. Magnetic resonance imaging (MRI) of the total spine with and without gadolinium is seen in Figure 1. Initial investigations were notable for normal complete blood cell count, electrolytes, kidney and liver function, B12, and thyrotropin as well as an unremarkable MRI of the brain. Three lumbar punctures were performed over the course of the patient’s hospitalization, each with a white blood cell count less than 5 /µL (to convert to ×109/L, multiply by 0.001), normal IgG index, absent oligoclonal bands, normal protein and glucose levels, and benign cytology. A comprehensive infectious and inflammatory workup including HIV, herpes simplex virus, varicella-zoster virus, Lyme disease, antinuclear antibodies, astrocyte aquaporin-4 autoantibody, myelin oligodendrocyte glycoprotein autoantibody, and serum and cerebrospinal fluid autoimmune panels were negative. Whole-body positron emission tomography demonstrated hypermetabolism of the midthoracic cord.

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C. Glioma

The clinical case and imaging were most suggestive of spinal cord tumor; subsequent spinal cord biopsy was consistent with H3-K27M mutant diffuse midline glioma. This case highlights an important early diagnostic consideration in the approach to myelopathy. Etiologies can be broadly categorized as inflammatory or noninflammatory, and cerebrospinal fluid is used to distinguish between them. Although the patient’s imaging demonstrated extensive enhancement, cerebrospinal fluid was repeatedly noninflammatory (absent pleocytosis and oligoclonal bands and normal IgG index). Infectious and postinfectious causes of myelopathy would typically be associated with these inflammatory markers as would immune-mediated causes of spinal cord dysfunction including neurosarcoidosis.

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Article Information

Corresponding Author: Alexandra Muccilli, MD, University of California, San Francisco, 505 Parnassus Ave, Box 0114, San Francisco, CA 94143-0114 (Alexandra.muccilli@gmail.com).

Conflict of Interest Disclosures: None reported.

Published Online: September 3, 2019. doi:10.1001/jamaneurol.2019.2806

Additional Contributions: We thank the patient for granting permission to publish this information. We also gratefully acknowledge the thoughtful care bestowed on the patient by Sasha Gupta, MD (University of California, San Francisco); Courtney Lane-Donovan, MD, PhD (University of California, San Francisco); and Reena Thomas, MD, PhD (Stanford University). None of these individuals were compensated outside their salary.

References
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Buerki  R, Lapointe  S, Solomon  D,  et al Path-09: clinical characteristics of adults with H3 K27M-mutant gliomas at UCSF.  Neuro Oncol.2018;20(suppl 6):vi159-vi160. doi:10.1093/neuonc/noy148.665Google ScholarCrossref
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Arrillaga-Romany  I, Chi  AS, Allen  JE, Oster  W, Wen  PY, Batchelor  TT.  A phase 2 study of the first imipridone ONC201, a selective DRD2 antagonist for oncology, administered every three weeks in recurrent glioblastoma.  Oncotarget. 2017;8(45):79298-79304. doi:10.18632/oncotarget.17837PubMedGoogle ScholarCrossref
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