Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature
Article first published online: 9 JUN 2012
© 2012 The Author(s) European Journal of Neurology © 2012 EFNS
European Journal of Neurology
Volume 20, Issue 1, pages 71–78, January 2013
How to Cite
O'Dwyer, J. P., Al-Moyeed, B. A., Farrell, M. A., Pidgeon, C. N., Collins, D. R., Fahy, A., Gibney, J., Swan, N., Dempsey, O. J., Kidd, D. P., Reid, J. M., Smyth, S. and McCabe, D. J. H. (2013), Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature. European Journal of Neurology, 20: 71–78. doi: 10.1111/j.1468-1331.2012.03783.x
- Issue published online: 22 DEC 2012
- Article first published online: 9 JUN 2012
- Manuscript Accepted: 4 MAY 2012
- Manuscript Received: 26 JAN 2012
- intracranial haemorrhage;
- systematic review
Background and purpose
Intracranial haemorrhage in neurosarcoidosis (NS-ICH) is rare, poorly understood and the diagnosis of NS may not be immediately apparent.
The clinical features of three new NS-ICH cases are described including new neuropathological findings and collated with cases from a systematic literature review.
Cases: (i) A 41-year-old man with headaches, hypoandrogenism and encephalopathy developed a cerebellar haemorrhage. He had neuropathological confirmation of NS with biopsy-proven angiocentric granulomata and venous disruption. He responded to immunosuppressive therapy. (ii) A 41-year-old man with no history of hypertension was found unconscious. A subsequently fatal pontine haemorrhage was diagnosed. Liver biopsy revealed sarcoid granulomas. (iii) A 36-year-old man with raised intracranial pressure headaches presented with a seizure and a frontal haemorrhage. Hilar lymph node biopsy confirmed sarcoidosis, and he was treated successfully. Systematic review: Twelve other published cases were identified and collated with our cases. Average age was 36 years and M:F = 2.3:1; 46% presented with neurological symptoms and 31% had CNS-isolated disease. Immediate symptoms of ICH were acute/worsening headache or seizures (60%). ICH was supratentorial (62%), infratentorial (31%) or subarachnoid (7%). Forty percent had definite NS, 53% probable NS and 7% possible NS (Zajicek criteria). Antigranulomatous/immunosuppressive therapy regimens varied and 31% died.
This series expands our knowledge of the pathology of NS-ICH, which may be of arterial or venous origin. One-third have isolated NS. Clinicians should consider NS in young-onset ICH because early aggressive antigranulomatous therapy may improve outcome.