Fulminant recurrent intracerebral hemorrhage in neurofibromatosis type 1: A case report with clinical insights

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder associated with cerebrovascular abnormalities, including an increased risk of intracranial hemorrhage. We present a 44-year-old male with confirmed NF1 who developed fulminant recurrent bilateral frontal intracerebral hemorrhage, requiring three emergency craniotomies over a 22-day period. Despite aggressive surgical and medical management, he developed recurrent hemorrhages and refractory cerebral edema, ultimately leading to death after the family elected to transition to comfort measures only. Serial coagulation profiles showed dynamic changes consistent with acute hemorrhage, postoperative reparative responses, and terminal consumptive coagulopathy. This case highlights the complex management challenges in NF1 patients with intracranial hemorrhage, where underlying vascular dysplasia may contribute to refractory hemorrhage and edema, requiring specialized approaches. Early recognition of NF1-associated cerebrovascular complications, aggressive multidisciplinary care, and serial coagulation monitoring are crucial for optimizing outcomes in these high-risk patients.