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Original Investigation |

Clinical Worsening in Reversible Cerebral Vasoconstriction Syndrome

Brian S. Katz, MD1; Jennifer E. Fugate, DO1; Sebastián F. Ameriso, MD2; Virginia A. Pujol-Lereis, MD2; Jay Mandrekar, PhD3; Kelly D. Flemming, MD1; David F. Kallmes, MD4; Alejandro A. Rabinstein, MD1
[+] Author Affiliations
1Department of Neurology, Mayo Clinic, Rochester, Minnesota
2Institute for Neurological Research, FLENI, Buenos Aires, Argentina
3Department of Biostatistics, Mayo Clinic, Rochester, Minnesota
4Department of Radiology, Mayo Clinic, Rochester, Minnesota
JAMA Neurol. 2014;71(1):68-73. doi:10.1001/jamaneurol.2013.4639.
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Importance  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.

Objective  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.

Main Outcome and Measure  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.

Results  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%).

Conclusions and Relevance  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.

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Figure 1.
A 52-Year-Old Woman Presented With Recurrent Thunderclap Headaches, Visual Changes, and Mild, Transient Left Hemiparesis

A, Axial, non–contrast-enhanced computed tomography obtained at initial presentation demonstrated no evidence for acute or chronic ischemia. B, Oblique anteroposterior view of the left internal carotid artery angiogram showed subtle narrowing of distal middle cerebral artery and anterior cerebral artery branches (arrows). C, One week later, the patient developed recurrent, persistent left hemiparesis. Axial diffusion-weighted magnetic resonance imaging performed at that time demonstrated bilateral areas of restricted diffusion within the cortex and associated sulcal effacement. D, Oblique anteroposterior view from a time-of-flight magnetic resonance angiogram of the left intracranial circulation showed widespread, progressive narrowing of proximal and distal anterior cerebral artery and middle cerebral artery branches.

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Figure 2.
A 33-Year-Old Woman Who Was 3 Weeks Post Partum Presented With Recurrent Headaches, Altered Mentation, and Left-Sided Numbness and Weakness

A, Non–contrast-enhanced computed tomography demonstrated mild subcortical hypodensity in the right parietal region. Hemorrhagic infarction of the left caudate head was present as well (not shown). Magnetic resonance angiogram of the brain showed widespread moderate narrowing of all vascular territories. Over the following 24 hours, she worsened rapidly to the point of developing spastic quadriparesis and requiring intubation for airway protection. B, An emergent conventional angiogram (anterior-posterior view of the right internal carotid artery injection shown) showed severe, near-occlusive, widespread stenoses, which were markedly worse than in the previous magnetic resonance angiogram. C, The patient was treated with angioplasty and intra-arterial injection of calcium channel blocker, but her clinical condition continued to deteriorate. Magnetic resonance imaging of the brain confirmed the presence of new infarctions, and the axial fluid-attenuated inversion recovery sequence showed new bilateral frontoparietal cortical infarctions. She became comatose after developing severe cerebral edema. After failure of aggressive medical therapy, the family requested withdrawal of life support measures.

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