Hemorrhagic transformation of ischemic stroke in a patient with Fahr’s disease

Abstract

BackgroundFahr’s disease is a very rare inherited neurodegenerative disorder characterized by diffuse and symetric intracranial calcification of the bilateral basal ganglia and cerebellum. Although the disease is slowly progressive, several acute forms have been described. We would like to present a very rare case of Fahr’s disease with an ischemic stroke and its hemorrhagic transformation.Case presentationA 70-year-old woman presented to our emergency department with rapid cognitive decline, dysarthria and right limb weakness. Cranial computed tomography showed diffuse and symmetric hyperdense areas at the level of the bilateral dentate nuclei of cerebellar hemispheres and basal ganglia, suggestive of calcification consistent with Fahr's disease on a background of thyroidectomy history, and effacement in the left caudate region. The patient was admitted to the intensive care unit with suspected left cerebral ischemic stroke. Because of clinical deterioration with increasing drowsiness and right hemiplegia, a control computed tomography scan was performed. Upon detection of a 6 * 8 cm lesion corresponding to an intracerebral hematoma in the left temporoparietal area with intraventricular component, she underwent surgery. The patient did not become hemodynamically stable and died on post-operative day 3.ConclusionsThe calcium deposits in the walls of the cerebral vessels in Fahr’s disease may exacerbate inflammatory processes leading to disruption of the blood-brain barrier. In addition, peripheral blood extravasation into the disrupted blood brain barrier due to ischemic stroke may lead to hemorrhagic transformation.