A Hemorrhagic Clue: Intracerebral Hemorrhage as the First Sign of Neurosarcoidosis

Introduction: Sarcoidosis is a multisystem granulomatous disorder of unclear etiology, most commonly affecting the lungs and lymphatics. Neurosarcoidosis occurs in 5-15% of cases and can involve the cranial nerves, meninges or brain parenchyma. Intracerebral hemorrhage (ICH) is a rare and atypical manifestation. We present a case of a young male with neurosarcoidosis who presented with ICH, highlighting an atypical neurologic onset with implications for early recognition and treatment.


Description: A 32-year-old healthy male with a history of tobacco and marijuana use presented with a 2-3 week history of progressive confusion, slurred speech and unsteady gait. He denied recent trauma, infections or illicit drug use. Head CT revealed possible left temporal intraparenchymal hemorrhage versus subarachnoid hemorrhage. CT angiography ruled out aneurysms, AVMs or dural fistulas. EEG was unrevealing. Brain MRI revealed a small focal hemorrhage in the left temporal lobe with diffuse nodular leptomeningeal enhancement, raising concern for an infiltrative, infectious or inflammatory process. Chest CT showed bilateral hilar lymphadenopathy and upper lobe peribronchial nodular infiltrates. Lumbar puncture revealed elevated protein, lymphocytic pleocytosis, positive oligoclonal bands, an elevated IgG index, and markedly increased soluble IL-2 receptor levels. Extensive infectious, neoplastic and autoimmune etiologies were negative. Biopsy of mediastinal lymph nodes confirmed non-caseating granulomas consistent with sarcoidosis. He received high dose IV steroids, followed by oral taper with steady clinical improvement. Rheumatology follow up was arranged for consideration of Infliximab.


Discussion: ICH is a rare and atypical presentation of neurosarcoidosis. Parenchymal hemorrhage with diffuse leptomenal enhancement prompted further workup as neurosarcoidosis may mimic CNS infection or malignancy. Diagnosis was supported by MRI findings and biopsy proven non-caseating granulomas. Favorable response to corticosteroids further supported the diagnosis and long-term immunosuppression may be necessary for disease control. ICH as the sentinel presentation was a rare finding yet, this case highlights the importance of considering it in the differential for unexplained cerebral hemorrhage.