Reversible Cerebral Vasoconstriction Syndrome With Typical and Atypical Symptoms: A Case Series

Abstract

Introduction Reversible cerebral vasoconstriction syndrome (RCVS) is most commonly characterized by focal or diffuse severe thunderclap headache with or without focal neurological deficits and associated transient focal vasoconstriction of the intracerebral arteries lasting up to three months. We present six patients diagnosed as RCVS, with three patients presenting with focal neurological deficits without headache and the remaining three with severe headache alone. Neuroimaging revealed focal subarachnoid bleed with or without segmental intracerebral vasospasm, which resolved over three months. Despite thunderclap headache being the most prevalent symptom associated with RCVS, the absence of this symptom should not preclude the diagnosis, especially in the presence of cortical subarachnoid hemorrhage (SAH) or focal segmental intracerebral arterial narrowing. Methods This case series is a retrospective analysis of all patients diagnosed with RCVS between 2018 and 2022, focusing on clinical symptoms, imaging findings, and management. Results Six patients (three males and three females) were diagnosed with RCVS between 2018 and 2022. Three patients presented with typical symptoms, while the remaining three presented with atypical symptoms. Neuroimaging findings ranged from normal to focal SAH with or without arterial narrowing. Conclusion This case series underscores the diverse clinical presentations of RCVS, emphasizing that while thunderclap headache is the predominant symptom, its absence should not exclude the possibility of RCVS, especially when accompanied by focal neurological deficits or cortical SAH. Neuroimaging played a crucial role in identifying the spectrum of findings. These findings highlight the importance of comprehensive evaluation and consideration of RCVS in patients presenting with neurological symptoms, even in the absence of typical headache features.

Keywords: ct; ct angio; mri; primary angiitis of the central nervous system; reversible cerebral vasoconstriction syndrome; subarachnoid haemorrhage; thunderclap headache.

Figure 1. A 54-year-old man with sudden onset of left-sided paresthesia and facial numbness. (A) Unenhanced CT appears normal. There was no SAH. (B) FLAIR MRI on the same day shows focal SAH (blue arrow) in the right central sulcus. (C) MR angiogram was normal. Follow-up CT angiogram (not shown) was also normal

CT: computed tomography; SAH: subarachnoid hemorrhage; FLAIR: fluid-attenuated inversion recovery; MRI: magnetic resonance imaging.

Figure 2. A 60-year-old man with a sudden, severe frontal headache accompanied by vomiting. Unenhanced CT shows focal SAH (blue arrows) in bilateral precentral sulci. CT angiogram (not shown) was normal.

CT: computed tomography; SAH: subarachnoid hemorrhage

Figure 3. A 45-year-old woman with sudden, severe occipital headache, photophobia, and nausea without any focal neurological deficits. Unenhanced CT shows focal SAH in the left posterior parietal region (blue arrow). CT angiogram (not shown) was normal.

CT: computed tomography; SAH: subarachnoid hemorrhage

Figure 4. A 52-year-old woman with sudden onset of severe headache. (A) Unenhanced CT shows focal SAH in both frontal regions (right side - blue arrow). (B-C) CT angiogram shows narrowing (blue arrows) in the right middle cerebral artery (B) and right A1 segment of the anterior cerebral artery (C). Follow-up CT angiogram after five months (not shown) shows resolution of the narrowing.

CT: computed tomography; SAH: subarachnoid hemorrhage

Figure 5. A 48-year-old man with sudden onset of left-sided incoordination and left-sided facial numbness intermittently over two weeks. The initial non-contrast CT (not shown) revealed focal SAH in the right central sulcus. The CT angiogram (not shown) was initially normal. (A-B) FLAIR MRI shows focal SAH in the right central sulcus and the parietooccipital region (blue arrow). (C) MRI angiogram demonstrated focal stenosis in the right middle cerebral artery. (D) Follow-up 3D CT angiogram after six months shows resolution of the right middle cerebral artery stenosis.

CT: computed tomography; SAH: subarachnoid hemorrhage; FLAIR: fluid-attenuated inversion recovery; MRI: magnetic resonance imaging