Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature.
dc.contributor.author | O'Dwyer, J P | |
dc.contributor.author | Al-Moyeed, B A | |
dc.contributor.author | Farrell, M A | |
dc.contributor.author | Pidgeon, C N | |
dc.contributor.author | Collins, D R | |
dc.contributor.author | Fahy, A | |
dc.contributor.author | Gibney, J | |
dc.contributor.author | Swan, N | |
dc.contributor.author | Dempsey, O J | |
dc.contributor.author | Kidd, D P | |
dc.contributor.author | Reid, J M | |
dc.contributor.author | Smyth, S | |
dc.contributor.author | McCabe, D J H | |
dc.date.accessioned | 2012-12-05T12:36:26Z | |
dc.date.available | 2012-12-05T12:36:26Z | |
dc.date.issued | 2012-06-09 | |
dc.identifier.citation | Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature. 2012:notEur J Neurol | en_GB |
dc.identifier.issn | 1468-1331 | |
dc.identifier.pmid | 22681045 | |
dc.identifier.doi | 10.1111/j.1468-1331.2012.03783.x | |
dc.identifier.uri | http://hdl.handle.net/10147/254547 | |
dc.description.abstract | BACKGROUND AND PURPOSE: Intracranial haemorrhage in neurosarcoidosis (NS-ICH) is rare, poorly understood and the diagnosis of NS may not be immediately apparent. METHODS: The clinical features of three new NS-ICH cases are described including new neuropathological findings and collated with cases from a systematic literature review. RESULTS: 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%). 40% had definite NS, 53% probable NS and 7% possible NS (Zajicek criteria). Antigranulomatous/immunosuppressive therapy regimens varied and 31% died. CONCLUSIONS: 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. | |
dc.language | ENG | |
dc.language.iso | en | en |
dc.rights | Archived with thanks to European journal of neurology : the official journal of the European Federation of Neurological Societies | en_GB |
dc.title | Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature. | en_GB |
dc.type | Article | en |
dc.contributor.department | Department of Neurology, The Adelaide and Meath Hospital, Dublin, incorporating the National Children's Hospital, Trinity College, Dublin, Ireland. | en_GB |
dc.identifier.journal | European journal of neurology : the official journal of the European Federation of Neurological Societies | en_GB |
dc.description.province | Leinster | en |
html.description.abstract | BACKGROUND AND PURPOSE: Intracranial haemorrhage in neurosarcoidosis (NS-ICH) is rare, poorly understood and the diagnosis of NS may not be immediately apparent. METHODS: The clinical features of three new NS-ICH cases are described including new neuropathological findings and collated with cases from a systematic literature review. RESULTS: 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%). 40% had definite NS, 53% probable NS and 7% possible NS (Zajicek criteria). Antigranulomatous/immunosuppressive therapy regimens varied and 31% died. CONCLUSIONS: 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. |