Department of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, India.
Department of General Medicine, Burdwan Medical College and Hospital, Burdwan, India.
J Stroke Cerebrovasc Dis. 2022 Jun;31(6):106432. doi: 10.1016/j.jstrokecerebrovasdis.2022.106432. Epub 2022 Mar 31.
An ambiguous definition of "asymptomatic" Moyamoya Angiopathy(aMMA) of absence of ischemic/hemorrhagic episodes in MMA patients, has led to its variable adaptation in the limited past-studies.
To observe the clinic-radiological characteristics and prospective follow-up of apparently "asymptomatic" MMA, and to determine if it is truly asymptomatic or not.
An observation, cohort study of 122 angiographically proven MMA over 6 years was undertaken from a single, tertiary-care-center to observe the clinico-radiological characteristics, prospective follow-up of apparently aMMA. Amongst them, 6 had an initial diagnosis of aMMA following evaluation by atleast one post-graduate doctor, which were further scrutinized by 3 different neurologists for epidemiological, clinical, radiological characteristics and subsequent follow-up. Data were analyzed using descriptive statistics.
Mean age was 23.7 ± 13.14 years. 3 of 6 underwent brain-imaging for evaluation of non-migraine-like headache, 1 for dizziness, 2 as part of familial screening for MMA. 4 of 6 patients had specific-triggers for aggravation of symptoms. Brain-imaging revealed old vascular insults and ivy sign in 5 of 6 each (83.3%), mean suzuki staging was 3.6±0.82. 4 of 6 underwent cerebral perfusion study, all had hypoperfusion. Revascularization surgery was done in 2 of 6, rest were managed conservatively. None had any new-onset neurological deficit or radiological progression over a mean follow-up period of 22.3 ± 20.22 months.
Apparently aMMA may not be truly asymptomatic and often have subtle "paroxysmal events" precipitated by specific-triggers, indicative of transient ischemic symptoms. Thus, warrants for a more precise definition to avoid misclassification of aMMA.
由于缺乏对“无缺血性/出血性事件”的烟雾病（Moyamoya Angiopathy，MMA）患者的“无症状”的明确定义，过去的有限研究中对其进行了不同的定义。
观察表现为“无症状”的烟雾病的临床-影像学特征和前瞻性随访，并确定其是否真的无症状。
对来自单一三级护理中心的 122 例经血管造影证实的 MMA 患者进行了为期 6 年的观察、队列研究，以观察临床表现为“无症状”的烟雾病的临床-影像学特征和前瞻性随访。其中，有 6 例在至少一位研究生医生评估后被诊断为“无症状”烟雾病，然后由 3 位不同的神经科医生进一步仔细评估其流行病学、临床、影像学特征和后续随访情况。数据采用描述性统计进行分析。
平均年龄为 23.7 ± 13.14 岁。6 例中有 3 例因非偏头痛样头痛进行脑成像评估，1 例因头晕，2 例因 MMA 家族筛查而进行脑成像评估。6 例中有 4 例患者的症状加重有特定诱因。5 例（83.3%）的脑成像显示陈旧性血管损伤和 ivy 征，6 例的平均铃木分期为 3.6±0.82。6 例中有 4 例行脑灌注研究，均显示灌注不足。6 例中有 2 例行血运重建手术，其余患者接受保守治疗。平均随访 22.3 ± 20.22 个月后，无患者出现新发神经功能缺损或影像学进展。
表现为“无症状”的烟雾病可能并非真正无症状，且常由特定诱因引起的隐匿性“阵发性事件”，提示短暂性缺血症状。因此，需要更精确的定义来避免烟雾病的误诊。
