脳卒中で発症したreversible cerebral vasoconstriction syndromeの3例

Reversible Cerebral Vasoconstriction Syndrome Associated with Stroke: Three Case Reports

Ⅰ．はじめに

　Reversible cerebral vasoconstriction syndrome（RCVS）は，2007年にCalabreseら²⁾によって新たに疾患概念として提唱されたものであり，これまでにさまざまな名称（isolated benign cerebral vasculitis¹⁶⁾，acute benign cerebral angiopathy¹⁴⁾，reversible cerebral segmental vasoconstriction¹¹⁾，Call-Fleming syndrome¹¹⁾，CNS pseudovasculitis¹²⁾，benign angiopathy of the CNS¹⁾，post-partum angiopathy¹⁷⁾，migraine angiitis⁷⁾，migrainous vasospasm¹⁶⁾，primary thunderclap headache⁹⁾，cerebral vasculopathy¹⁹⁾）で散在性に報告されてきた疾患群を包括するものである．RCVSは雷鳴頭痛と頭蓋内血管攣縮を呈するのが特徴であり，この血管攣縮は通常3カ月以内に自然軽快するといわれている．しかしながら，ときに虚血性あるいは出血性の脳卒中を伴うことが知られており，脳神経外科診療においても遭遇する機会が多く，特に若年者の脳卒中の原因として知っておくべき重要な疾患群と考えられる．

　本稿では，自験例の中でさまざまなタイプの脳卒中で発症し，RCVSと診断された3例を提示するとともに，文献的考察を交えて報告する．

　Reversible cerebral vasoconstriction syndrome（RCVS）is characterized by severe headache and diffuse segmental constriction of cerebral arteries that resolves spontaneously within a few months. Although manifestations of stroke are not included in diagnostic criteria of RCVS, it is known that some cases may be associated with stroke, including intracerebral hemorrhage, subarachnoid hemorrhage, or cerebral infarction. We present three cases of RCVS associated with various types of stroke, and then review the literature. Case 1:A 49-year-old woman presented with a headache followed by left hemiparesis and dysarthria. One month before the onset, she was transfused for severe anemia caused by uterus myoma. CT images revealed intracerebral hemorrhages in the right putamen and right occipital lobe. Angiography revealed multiple segmental constrictions of the cerebral arteries. One month after the onset, these vasoconstrictions improved spontaneously. Case 2:A postpartum 38-year-old woman who had a history of migraine presented with thunderclap headache. Imaging revealed a focal subarachnoid hemorrhage in the right postcentral sulcus and segmental vasoconstriction of the right middle cerebral artery. One week after the onset, this vasoconstriction improved spontaneously. Case 3:A 32-year-old woman who had a history of migraine presented with headache followed by left homonymous hemianopsia. Imaging revealed a cerebral infarction of the right occipital lobe and multiple constrictions of the right posterior cerebral artery. These vasoconstrictions gradually improved spontaneously.