Neurosarcoidosis: unusual presentations and considerations for diagnosis and management

Abstract

Background: Sarcoidosis is a chronic, multisystem disease characterised by non-necrotising granulomatous inflammation of unknown aetiology. Most commonly, the lungs, lymph nodes, skin and eyes are affected in sarcoidosis; however, nervous system involvement occurs in approximately 5%-15% of cases. Any part of the nervous system can be affected by sarcoidosis.

Cases: Herein we describe three unusual patient presentations of neurosarcoidosis, one with optic neuritis, a second with hydrocephalus and a third with cervical myelopathy.

Conclusions: We include pertinent details about their presentations, imaging findings, pathology, management and clinical course.

Keywords: hydrocephalus; neurosarcoidosis; optic neuritis.

Figure 1

Case 1: T2-weighted sagittal view demonstrated a slightly hyperintense intradural extramedullary mass at C5–C6 level (A). T1-weighted sagittal view demonstrated an isointense intradural extra-axial mass at the C5–C6 level (B). T1-weighted Gd-enhanced sagittal view demonstrated contrast enhancement of the mass (C). T2-weighted axial view of the brain shows a hyperintense mass involving the left orbital nerve canalicular region and left prechiasmatic region with sparing of the optic nerve and no enhancement of the optic nerve (D). Sections from the intradural-extramedullary mass, revealed non-necrotising granulomatous inflammation with prominent multinucleated giant cells, some of which contain asteroid bodies (original magnification ×200). Stains for acid-fast and fungal organisms (not shown) were negative (E).

Figure 2

Case 2: Non-contrast CT of brain axial view revealed hydrocephalus (A). T1-weighted Gd-enhanced coronal view demonstrated contrast enhancement along the third ventricle and foramen of Monroe, it was difficult to distinguish the mass from the choroid plexus (B). Sections from the intraventricular biopsy demonstrate non-necrotising granulomatous inflammation involving the choroid plexus (bottom left; original magnification ×40). As in case 1, stains for acid-fast and fungal organisms were negative (C).

Figure 3

Case 3: T2-weighted sagittal view demonstrated a hyperintense intramedullary mass from C1 to C3 and around the lower medulla (A). T1-weighted sagittal view demonstrated an isointense mass from C1 to C3 (B). T1-weighted Gd-enhanced sagittal view demonstrated contrast enhancement of the intramedullary mass (C). Thoracic CT scan demonstrated bilateral pulmonary hilar mass (D). T1-weighted sagittal view 2 weeks following corticosteroid treatment showed near total resolution of the mass (E).