Hématome sous-dural aigu spontané : rechercher la maladie de Willebrand

Acute spontaneous subdural hematoma is a frequent, although poorly known clinical situation. No standardized diagnostic approach has ever been proposed for this disease. Some classical causes, such as dural fistula or spontaneous intracranial hypotension are consensually searched, whereas others, less known by neurologists, could be forgotten. Herein, we report the case of a middle-aged woman who presented with uncommon headache, leading to the diagnosis of subacute spontaneous subdural hematoma. She had no significant medical history, except for heavy menstrual bleeding. Initial investigations, including computed tomography, MRI, arteriography, full blood count and a standard hemostasis exploration showed no abnormalities. Despite normal international normalized ratio (INR) and partial thromboplastin time (PTT), we performed a platelet function assay (PFA-100) and a search for von Willebrand disease. These explorations led to the diagnostic of a type 2 von Willebrand disease. Von Willebrand disease is the most frequent hemorrhagic disease worldwide. It is linked to a quantitative (type 1 and 3) or functional (type 2) constitutional deficit in von Willebrand factor. As many mutations in various genes have been evidenced, clinical presentations of von Willebrand disease are very heterogeneous, ranging from a menorrhagia to a dramatic pediatric hemorrhagic syndrome. Generally, treatment consists in repeated administration of von Willebrand factor. The present case of a late diagnostic of constitutional hemostasis disease, revealed by an acute spontaneous subdural hematoma in which common hemostasis exploration was normal, underlines the importance of a precise clinical interview searching for any other hemorrhagic symptoms in these patients. It also recalls the need to know the basis of hemostasis physiology; especially primary hemostasis which is not explored by common hemostasis exams (INR and PTT)