Cranial nerve VI palsy as an initial presentation of necrotizing sarcoid granulomatosis in a 14-year-old female: case report and literature review

Semin Arthritis Rheum. 2015 Feb;44(4):456-60. doi: 10.1016/j.semarthrit.2014.07.004. Epub 2014 Jul 17.

Abstract

Objectives: We describe a case of pediatric necrotizing sarcoid granulomatosis (NSG) presenting with right cranial nerve VI palsy and multiple lung nodules, successfully treated with corticosteroids.

Methods: This is a descriptive case report of one patient with review of the literature.

Results: A 14-year-old Caucasian female presented with complaints of pain on inspiration and dyspnea on exertion, as well as diplopia that was worse with right gaze. The patient presented to our emergency department with persistent diplopia and was found to have stable right cranial nerve VI palsy. CTA showed multiple pulmonary nodules. Despite continued extensive multispecialty work-up, the patient׳s cranial nerve VI palsy had not resolved, thus tissue confirmation via lung biopsy was performed. Pathologic diagnosis revealed necrotizing sarcoid granulomatosis. The patient was subsequently started on intravenous corticosteroids, which led to the rapid resolution of her presenting symptoms.

Conclusions: Necrotizing sarcoid granulomatosis is a multisystem organ disease that is rare in children. Pathology commonly reveals epithelioid noncaseating granuloma and granulomatous vasculitis with necrosis. We report an unusual presentation involving sixth nerve palsy in a 14-year-old girl. Diagnosis was determined and confirmed by histopathology of a pulmonary nodule biopsy. This is the first case to our knowledge of NSG presenting with cranial nerve palsy in a pediatric patient.

Keywords: Cranial nerve; Necrotizing sarcoid granulomatosis (NSG); Pediatric; Pulmonary nodules.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abducens Nerve Diseases / diagnosis*
  • Abducens Nerve Diseases / drug therapy
  • Abducens Nerve Diseases / etiology*
  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Biopsy
  • Female
  • Humans
  • Lung / pathology
  • Multiple Pulmonary Nodules / pathology
  • Sarcoidosis, Pulmonary / complications*
  • Sarcoidosis, Pulmonary / diagnosis*
  • Sarcoidosis, Pulmonary / pathology
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones