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Bilateral Panuveitis and Myeloradiculopathy in a 12-Year-Old Girl

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

A 12-year-old girl presented for assessment of abnormal gait. A year prior, she was found to have bilateral panuveitis with occlusive retinal periphlebitis involving all quadrants of the central and peripheral retina with capillary nonperfusion (Figure 1). Her subsequent workup revealed elevated inflammatory markers and subcentimeter chest and abdominal lymph nodes. Given a positive interferon-γ release assay, therapy was initiated with rifampin, isoniazid, pyrazinamide, ethambutol (RIPE) and corticosteroids for presumed tuberculosis.

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A 12-year-old girl presented for assessment of abnormal gait. A year prior, she was found to have bilateral panuveitis with occlusive retinal periphlebitis involving all quadrants of the central and peripheral retina with capillary nonperfusion (Figure 1). Her subsequent workup revealed elevated inflammatory markers and subcentimeter chest and abdominal lymph nodes. Given a positive interferon-γ release assay, therapy was initiated with rifampin, isoniazid, pyrazinamide, ethambutol (RIPE) and corticosteroids for presumed tuberculosis.

At the time of presentation, she had completed RIPE therapy and was taking mycophenolate mofetil with resolution of her ocular manifestations. She complained of new bilateral lower extremity weakness with associated lower back pain. Clinical examination results revealed a wide-based Trendelenburg gait with right foot drop and diminished strength of lower extremity flexors. Laboratory testing results showed a normal white blood cell count, vitamin B12, angiotensin-converting enzyme, lysozyme, C-reactive protein, and antinuclear antibody levels. She had negative test results for Lyme disease, syphilis, and HIV. Computed tomography showed small supraclavicular and mediastinal lymph nodes and an enlarged spleen. Magnetic resonance imaging demonstrated mild lacrimal gland prominence and low thoracic/high lumbar spine and cauda equina nerve roots hyperintensity and abnormal enhancement. Cerebrospinal fluid analysis revealed a lymphocytic pleocytosis with an elevated protein level, negative syphilis antibody results, and an absence of organisms on acid-fast bacilli smear, Gram stain, and culture.

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Article Information

Corresponding Author: Nahyoung G. Lee, MD, Massachusetts Eye and Ear, 243 Charles St, Boston, MA 02114 (grace_lee@meei.harvard.edu).

Published Online: December 23, 2021. doi:10.1001/jamaophthalmol.2021.3076

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank Sarkis Soukiasian, MD, Lahey Medical Center, for the fundus images. We also thank the patient’s mother for granting permission to publish this information.

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AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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