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Images in Neurology
October 22, 2018

Myelin Oligodendrocyte Glycoprotein Antibody–Associated Demyelination Comorbid With Leber Hereditary Optic Neuropathy

Author Affiliations
  • 1Department of Neurology, University of Iowa Hospitals and Clinics, Iowa City
  • 2Department of Ophthalmology, Oregon Health & Science University, Portland
  • 3Department of Neurology, Oregon Health & Science University, Portland
  • 4Neurology Department, VA Portland Health Care System, Portland, Oregon
JAMA Neurol. 2019;76(2):227-228. doi:10.1001/jamaneurol.2018.3207

A patient with genetically established Leber hereditary optic neuropathy (LHON) who was diagnosed at age 24 years (after presentation with rapidly progressive, severe, painless, bilateral vision loss and the identification of an m.11778G→A LHON mutation) developed ascending sensory loss and dysesthesias 1 year after diagnosis. Neuroimaging revealed nonenhancing longitudinally extensive lesions in the cervical spinal cord, subtle areas of increases in T2 signals in the midthoracic and upper thoracic spinal cord, and normal brain imaging (Figure, A). Serum testing for aquaporin-4 IgG produced negative results by both enzyme-linked immunoassay and cell-based assays; cerebrospinal fluid test results for oligoclonal bands and IgG index abnormalities were also negative. The patient then developed acute vertigo and urinary retention. Follow-up neuroimaging revealed new enhancement at the level of the C2 vertebra, new and enhancing lesions in the brainstem and thalami, and more pronounced T2 hyperintensity at the levels of T1 through T7 vertebrae. The brain lesions included symmetric areas of hyperintense signal change in the periaqueductal gray, medial thalami, pons, cerebral peduncles, and dorsal medulla (Figure, B and C). Myelopathic symptoms responded to intravenous steroids. At this time, the patient tested positive for anti–myelin oligodendrocyte glycoprotein (MOG) IgG. Based on this testing, along with the clinical and radiographic features, immunosuppressive therapy was offered.

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