Case Report: Fulminant recurrent intracerebral hemorrhage in neurofibromatosis type 1

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 25-day period. Despite aggressive surgical and medical management, he had recurrent hemorrhages and refractory cerebral edema, ultimately leading to death after the family chose comfort measures only. Serial coagulation profiles showed dynamic changes consistent with acute hemorrhage, postoperative reparative responses, and terminal changes that suggested a possible consumptive coagulopathy. This case illustrates the difficult management of NF1 patients with intracranial hemorrhage, where underlying vascular dysplasia can lead to refractory bleeding and edema. Early recognition of NF1-related cerebrovascular problems, coordinated multidisciplinary care, and repeated coagulation monitoring are important for these high-risk patients.