Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Institute for Neurological Research, FLENI, Buenos Aires, Argentina.
JAMA Neurol. 2014 Jan;71(1):68-73. doi: 10.1001/jamaneurol.2013.4639.
Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches and evidence of vasoconstriction with subsequent resolution. The clinical course of RCVS is traditionally considered monophasic and benign. However, recurrent episodes of focal neurological symptoms have been described after initial presentation.
To define the frequency, timing, and consequences of clinical worsening in patients with diagnosis of RCVS.
DESIGN, SETTING, AND PARTICIPANTS: Retrospective observational study of consecutive patients with RCVS at 2 referral institutions for neurological disease.
Clinical worsening after diagnosis of RCVS. We defined clinical worsening as new permanent or transient neurological deficits (compared with presenting signs and symptoms) or new onset of seizures. We performed a logistic regression analysis to assess associations between patient characteristics and clinical worsening. Functional outcome was assessed at 1 to 3 months using the modified Rankin score.
We identified 59 patients (median age, 47 years; interquartile range, 32-54 years) with RCVS. Twenty patients (34%) experienced clinical worsening after a median of 2.5 days (range, several hours to 14 days). Eight of the 20 patients who worsened had permanent deficits, including 4 who died. We did not find an association between age, sex, smoking, migraine, acute or chronic hypertension, peripartum state, or use of serotonergic drugs with clinical worsening. Clinical worsening was associated with radiological infarction (P = .001) and worse functional outcome (P < .004). Functional outcome was favorable (modified Rankin score 0-2) in 51 patients (86.4%).
Clinical worsening after diagnosis is common in patients with RCVS. Thus, RCVS is self-limited but not strictly monophasic. Most patients have a very favorable outcome, but clinical worsening may result in permanent deficits.
可逆性脑血管收缩综合征（RCVS）的特征是反复发作的霹雳头痛和随后缓解的血管收缩证据。RCVS 的临床病程传统上被认为是单相和良性的。然而，在初始表现后已经描述了反复发作的局灶性神经症状。
定义 RCVS 诊断后临床恶化的频率、时间和后果。
设计、设置和参与者：对 2 家神经病学转诊机构的连续 RCVS 患者进行回顾性观察研究。
RCVS 诊断后临床恶化。我们将临床恶化定义为新的永久性或短暂性神经功能缺损（与现有体征和症状相比）或新发癫痫发作。我们进行了逻辑回归分析，以评估患者特征与临床恶化之间的关联。使用改良 Rankin 评分在 1 至 3 个月评估功能结局。
我们确定了 59 例 RCVS 患者（中位年龄 47 岁；四分位距 32-54 岁）。20 例患者（34%）在中位时间 2.5 天（范围为数小时至 14 天）后出现临床恶化。20 例恶化的患者中有 8 例出现永久性缺损，其中 4 例死亡。我们没有发现年龄、性别、吸烟、偏头痛、急性或慢性高血压、围产期状态或使用 5-羟色胺能药物与临床恶化之间存在关联。影像学梗死（P = .001）和更差的功能结局（P < .004）与临床恶化相关。51 例患者（86.4%）的功能结局良好（改良 Rankin 评分 0-2）。
RCVS 患者诊断后临床恶化很常见。因此，RCVS 是自限性的，但并非严格的单相。大多数患者的预后非常好，但临床恶化可能导致永久性缺损。
