Bilateral Panuveitis and Myeloradiculopathy in a 12-Year-Old Girl | Adolescent Medicine | JN Learning | AMA Ed Hub [Skip to Content]
[Skip to Content Landing]

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.

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

Pediatric neurosarcoidosis

B. Perform a lacrimal gland biopsy

A right orbitotomy with lacrimal gland biopsy was performed (choice B) allowing for histopathologic confirmation of sarcoidosis (Figure 2). Sarcoidosis is a protean multisystem inflammatory disorder classically defined histologically by well-formed, non-necrotizing, “naked” epithelioid granulomas. Focal histologic necrosis can be seen, particularly in the lacrimal gland.1 Neurosarcoidosis can manifest anywhere in the nervous system including as intramedullary spinal cord lesions.2 Neurologic manifestations occur in approximately 5% of adults with sarcoidosis and is even rarer in children.3 Fifty-five pediatric cases have been reported of which 10 presented as isolated neurosarcoidosis.4 The average age of presentation of pediatric neurosarcoidosis is 12 years and the most common manifestations are seizure, cranial neuropathies, hypothalamic dysfunction, and optic nerve swelling.4 Spinal cord involvement is unusual in children, and to our knowledge, this is the third case reported in the literature.4 Symptoms present subacutely, with varying combinations of pain, limb paraparesis, paresthesias, and bowel or bladder dysfunction.2 Cerebrospinal fluid (CSF) studies typically show a lymphocytic pleocytosis and elevated protein level.5

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

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.

References
1.
Rosen  Y .  Four decades of necrotizing sarcoid granulomatosis: what do we know now?   Arch Pathol Lab Med. 2015;139(2):252-262. doi:10.5858/arpa.2014-0051-RAPubMedGoogle ScholarCrossref
2.
Junger  SS , Stern  BJ , Levine  SR , Sipos  E , Marti-Masso  JF .  Intramedullary spinal sarcoidosis: clinical and magnetic resonance imaging characteristics.   Neurology. 1993;43(2):333-337. doi:10.1212/WNL.43.2.333PubMedGoogle ScholarCrossref
3.
Stern  BJ , Krumholz  A , Johns  C , Scott  P , Nissim  J .  Sarcoidosis and its neurological manifestations.   Arch Neurol. 1985;42(9):909-917. doi:10.1001/archneur.1985.04060080095022PubMedGoogle ScholarCrossref
4.
Rao  R , Dimitriades  VR , Weimer  M , Sandlin  C .  Neurosarcoidosis in pediatric patients: a case report and review of isolated and systemic neurosarcoidosis.   Pediatr Neurol. 2016;63:45-52. doi:10.1016/j.pediatrneurol.2016.05.018PubMedGoogle ScholarCrossref
5.
Zajicek  JP , Scolding  NJ , Foster  O ,  et al.  Central nervous system sarcoidosis—diagnosis and management.   QJM. 1999;92(2):103-117. doi:10.1093/qjmed/92.2.103PubMedGoogle ScholarCrossref
6.
Urbain  F , Labeyrie  C , Herbrecht  A ,  et al.  Cervical spinal sarcoidosis mimicking compressive cervical myelopathy: Poor prognosis after surgery.   Rev Med Interne. In French. 2020;41(2):78-88. doi:10.1016/j.revmed.2019.11.006PubMedGoogle ScholarCrossref
7.
Mombaerts  I , Rose  GE , Garrity  JA .  Orbital inflammation: biopsy first.   Surv Ophthalmol. 2016;61(5):664-669. doi:10.1016/j.survophthal.2016.03.002PubMedGoogle ScholarCrossref
8.
Sharma  OP .  Markers of sarcoidosis activity.   Chest. 1986;90(4):471-473. doi:10.1378/chest.90.4.471PubMedGoogle ScholarCrossref
9.
Mochizuki  M , Smith  JR , Takase  H , Kaburaki  T , Acharya  NR , Rao  NA ; International Workshop on Ocular Sarcoidosis Study Group.  Revised criteria of International Workshop on Ocular Sarcoidosis (IWOS) for the diagnosis of ocular sarcoidosis.   Br J Ophthalmol. 2019;103(10):1418-1422. doi:10.1136/bjophthalmol-2018-313356PubMedGoogle ScholarCrossref
10.
Millet  JP , Shaw  E , Orcau  A , Casals  M , Miró  JM , Caylà  JA ; Barcelona Tuberculosis Recurrence Working Group.  Tuberculosis recurrence after completion treatment in a European city: reinfection or relapse?   PLoS One. 2013;8(6):e64898. doi:10.1371/journal.pone.0064898PubMedGoogle Scholar
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Close
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close

Name Your Search

Save Search
Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Close

Lookup An Activity

or

Close

My Saved Searches

You currently have no searches saved.

Close

My Saved Courses

You currently have no courses saved.

Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close