We report a case of spontaneous intracerebral hemorrhage occurring in a young woman and revealing Addison’s disease. This autoimmune primary adrenocortical insufficiency was associated with premature ovarian failure. Exhaustive research for nonhypertensive causes of intracerebral hemorrhage was negative. Initial coagulation studies disclosed severe hypofibrinogenemia and prolonged prothrombin time related to vitamin K-dependent coagulation factor deficit. Clotting abnormalities cleared at 4 months under treatment with hydrocortisone. Glucocorticoids are potent regulators of fibrinogen biosynthesis, increasing fibrinogen secretion. We conclude that primary adrenocortical insufficiency induced this hemorrhagic diathesis leading to spontaneous intracerebral hemorrhage. This latter has never been reported in Addison’s disease. Primary adrenocortical insufficiency should be considered as a rare potential cause of nonhypertensive intracerebral hemorrhage.

Keywords:
Intracerebral hemorrhage, Addison’s disease, Hepatocellular fibrinogen synthesis, Vitamin K deficiency
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