Reversible cerebral vasoconstriction syndrome: strategies to early diagnosis and the role of transcranial color-coded doppler ultrasonography (TCCD)

Abstract

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular transitory condition characterized by severe headache, possible concomitant acute neurological symptoms, evidence of diffuse multifocal segmental constriction of cerebral arteries, and usually spontaneously resolving within 3 months. Putative causes and/or precipitating factors are vasoactive drugs-e.g., antidepressants, α-sympathomimetics, triptans-post-partum, and immunosuppressants.

Case presentation: We report the case of a middle-aged woman referred to the emergency room (ER) with a 7-day long intense headache and vomit. Cerebral non-contrast computed tomography (CT) was negative for acute ischemic lesions or intracranial bleedings. She was again referred to ER 7 days later with additional fluctuating episodes of weakness in left arm and both lower limbs. A new brain CT was negative. Due to worsening headache, a transcranial color-coded Doppler (TCCD) was performed, which showed diffuse multifocal blood flow acceleration in all principal intracranial vessels, and particularly on the right hemisphere. These findings were subsequently confirmed at MR angiogram and digital subtraction angiography.

Conclusion: TCCD imaging is a non-invasive and relatively inexpensive tool which provides real-time information on cerebrovascular function, blood flow velocities, and hemodynamic changes. TCCD may be a powerful tool in the early detection of acute infrequent cerebrovascular conditions, as well as in monitoring their course and the therapeutic response.

Keywords: Cerebrovascular diseases; Reversible cerebral vasoconstriction syndrome; Thunderclap headache; Transcranial color-coded Doppler.

Fig. 1

Transcranial color-coded Doppler (TCCD) images showing focal acceleration, diffuse aliasing, and flow turbulence in the principal intracranial vessels. A Right distal middle cerebral artery (M1) with peak systolic velocity (PSV) of ≈ 380 cm/s, B right posterior cerebral artery (P1) with PSV ≈ 300 cm/s, and C basilar artery with PSV ≈ 180 cm/s and flow turbulence

Fig. 2

Cerebral angiography (A) and magnetic resonance angiography (MRA) (B) depicting remarkable smooth multifocal widespread narrowing and irregularities in the vessels of both anterior and posterior intracranial circulation