Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature

Eur J Neurol. 2013 Jan;20(1):71-8. doi: 10.1111/j.1468-1331.2012.03783.x. Epub 2012 Jun 9.


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.

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.

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.

Publication types

  • Case Reports
  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Central Nervous System Diseases / complications*
  • Humans
  • Intracranial Hemorrhages / diagnosis*
  • Intracranial Hemorrhages / etiology*
  • Magnetic Resonance Imaging
  • Male
  • Sarcoidosis / complications*
  • Tomography, X-Ray Computed

Supplementary concepts

  • Neurosarcoidosis