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Primary histiocytic sarcoma should be considered in the differential diagnosis of this rare syndrome. This case highlights the importance of a broad search in the investigation of new onset raised intracranial pressure headache, including imaging of the lower spinal cord. Primary histiocytic sarcoma should be considered in the differential diagnosis of this rare syndrome.Brain arteriovenous malformations (bAVMs) are relatively rare, although their potential for secondary intracranial haemorrhage (ICH) makes their diagnosis and manageme

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