Idiopathic intracranial hypertension and hemophilia A

A patient with hemophilia A and long-standing recurrent symptoms of idiopathic intracranial hypertension is described. During his relapses, he experienced headache, and attention and language disturbance, but no visual symptoms.

Hemophilia A is a rare inherited coagulation disorder secondary to factor VIII deficiency. Idiopathic intracranial hypertension has been reported in association with prothrombotic conditions and iron deficiency anemia, but not in patients with hemophilia A. Recurrent or chronic headache is not a typical symptom of hemophilia, but headache is a presenting sign of intracranial bleed in persons with hemophilia.

Medical history review, clinical neurologic examination, brain magnetic resonance imaging, computed head tomography, and electroencephalogram were performed.

Neurologic examination revealed bilateral papilledema during relapses of idiopathic intracranial hypertension. Multiple lumbar punctures preceded by the intravenous administration of factor VIII early in the course of the illness confirmed the presence of elevated cerebrospinal fluid pressures and absence of subarachnoid blood. He had no complications from lumbar punctures. Initial electroencephalograms showed background slowing but later normalized. Magnetic resonance imaging of the brain and computerized tomography of the head were normal. Relapses of idiopathic intracranial hypertension were eventually controlled with the administration of acetazolamide.

Idiopathic intracranial hypertension may develop in patients with hemophilia A in the absence of visual symptoms. Therapeutic and diagnostic lumbar punctures were safe to perform on this patient, following the administration of factor VIII.