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