Zou Ning, Guo Guixiang, Wan Fangchao, Li Xin
Department of Neurology, The First People's Hospital of Changde City, Changde, China.
Front Neurol. 2021 Sep 16;12:696056. doi: 10.3389/fneur.2021.696056. eCollection 2021.
Moyamoya disease is a rare cerebrovascular occlusive disease, which is characterized by stenosis and gradual occlusion of the internal carotid arteries, causing the progression of characteristic collateral vessels. To date, most studies investigating moyamoya disease have focused on medical implications, and the potential implications for neurocognitive and/or neuropsychiatric functioning were inconclusive. we present a case of a 26-year-old Chinese postpartum woman who presented to the emergency department with a 19-h history of cognitive decline, vomiting, and convulsions. Blood pressure, heart rate, and respiration rate were 200/120 mmHg, 115 beats/minute, and 30 breaths/minute, respectively, on arrival. The Glasgow Coma Scale, modified RANKIN scale (mRS), and National Institute of Health stroke scale (NIHSS) scores were 3, 5, and 18, respectively. Moyamoya disease was diagnosed using cerebral angiography and digital subtraction angiography. The cognitive functions of orientation, use of language, ability to calculate, and memory significantly improved after 11 days of treatment (Glasgow Coma Scale: 15; mRS: 0; NIHSS: 0). This patient was diagnosed with reversible posterior leukoencephalopathy syndrome related to moyamoya disease. This case highlights that atypical posterior reversible encephalopathy syndrome can occur in patients with moyamoya disease, and should be considered for the differential diagnosis of cerebral infarcts and hemorrhage in a postpartum female.
烟雾病是一种罕见的脑血管闭塞性疾病，其特征是颈内动脉狭窄并逐渐闭塞，导致特征性侧支血管形成。迄今为止，大多数关于烟雾病的研究都集中在医学方面，而对神经认知和/或神经精神功能的潜在影响尚无定论。我们报告一例26岁的中国产后女性病例，该患者因认知功能下降、呕吐和抽搐19小时就诊于急诊科。入院时血压、心率和呼吸频率分别为200/120 mmHg、115次/分钟和30次/分钟。格拉斯哥昏迷量表、改良Rankin量表（mRS）和美国国立卫生研究院卒中量表（NIHSS）评分分别为3分、5分和18分。通过脑血管造影和数字减影血管造影诊断为烟雾病。治疗11天后，定向、语言运用、计算能力和记忆等认知功能显著改善（格拉斯哥昏迷量表：15分；mRS：0分；NIHSS：0分）。该患者被诊断为与烟雾病相关的可逆性后部白质脑病综合征。该病例强调，烟雾病患者可能发生非典型后部可逆性脑病综合征，在产后女性脑梗死和脑出血的鉴别诊断中应予以考虑。
