Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Brain infarction and cerebral venous thrombosis in paroxysmal nocturnal hemoglobinuria: case report

Keiko Maezono, M.D.1j, Eijirou Tanaka, M.D.1j, Shinji Ashida, M.D., Ph.D.1j, Shiori Ogura, M.D.1j, Yoshikazu Nakahara, M.D., Ph.D.2j and Yoshinari Nagakane, M.D., Ph.D.1j

1) Department of Neurology, Kyoto Second Red Cross Hospital
2) Department of Neurosurgery, Kyoto Second Red Cross Hospital

A 65-year-old woman with a six-year history of paroxysmal nocturnal hemoglobinuria (PNH) was admitted due to weakness in the right leg following a seven-day history of fever and upper respiratory infection. MRI revealed several high-intensity areas in bilateral frontal lobe cortices and the left cerebellum on diffusion-weighted imaging, and signal hypointensity along the course of the cortical vein in the left frontal lobe on T2-weighted imaging. We diagnosed cerebral venous thrombosis and brain infarction, and commenced heparin infusion. She developed right-sided dens hemiparesis on hospital day 6, when brain CT showed subcortical hemorrhage in the left frontal lobe. Despite eculizumab administration and decompressive craniectomy for hematoma, she died on hospital day 26. Thrombosis in PNH has been recognized as a life-threating complication, and intensive treatment including emergent administration of eculizumab is warranted if this situation arises.
Full Text of this Article in Japanese PDF (1646K)

(CLINICA NEUROL, 62: 27|32, 2022)
key words: paroxysmal nocturnal hemoglobinuria, cerebral venous thrombosis, eculizumab

(Received: 22-Jun-21)