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



METHODS
(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.
Department of Neurology, The Adelaide and Meath Hospital, Dublin, incorporating the National Children's Hospital, Trinity College, Dublin, Ireland.
J P
JP
O'Dwyer
B A
BA
Al-Moyeed
M A
MA
Farrell
C N
CN
Pidgeon
D R
DR
Collins
A
A
Fahy
J
J
Gibney
N
N
Swan
O J
OJ
Dempsey
D P
DP
Kidd
J M
JM
Reid
S
S
Smyth
D J H
DJ
McCabe
D000328:Adult; D002493:Central Nervous System Diseases / Q000150:complications*; D006801:Humans; D020300:Intracranial Hemorrhages / Q000175:diagnosis* / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D012507:Sarcoidosis / Q000150:complications*; D014057:Tomography, X-Ray Computed