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JAMA Neurology Clinical Challenge
September 3, 2019

A Case of Progressive Myelopathy in a Middle-aged Woman

Author Affiliations
  • 1University of California, San Francisco, San Francisco
  • 2Stanford University Medical Center, Stanford, California
JAMA Neurol. Published online September 3, 2019. doi:10.1001/jamaneurol.2019.2806

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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