Neurointervention.  2021 Mar;16(1):78-82. 10.5469/neuroint.2020.00416.

Lessons Learned from a Fulminant Case of Reversible Cerebral Vasoconstriction Syndrome: Past Medical History Misleads the Diagnosis and Intra-Arterial Milrinone Offers Diagnostic Utility

• Alapatt PJ
• Panwar A
• Kuruttukulam GV
• Sundar K

• KMID: 2513348
• DOI: http://doi.org/10.5469/neuroint.2020.00416

Abstract

A 34-year-old post-partum female having dermatomyositis developed headache and became comatose after a seizure episode. Magnetic resonance imaging of brain showed a massive left ganglio-capsular bleed for which decompressive surgery was done. Computed tomographic angiography showed multiple foci of narrowing and irregularities in distal cerebral vessels. In view of dermatomyositis, the diagnosis of vasculitis was considered and pulse therapy of intravenous methylprednisolone was started. The patient, however, showed no improvement and developed new brain infarcts. She was subsequently taken up for a diagnostic cerebral angiography which showed multifocal severe narrowing in bilateral major cerebral arteries. These angiographic abnormalities showed excellent reversibility to intra-arterial milrinone and hence, reversible cerebral vasoconstriction syndrome (RCVS) was diagnosed. Normal angiographic findings in the first week do not rule out the disease and a repeat angiography should be considered if the clinical suspicion of the RCVS is high. Intra-arterial milrinone has a high diagnostic utility.

Keyword

Reversible cerebral vasoconstriction syndrome; Milrinone; Diagnostic cerebral angiography; Vasculitis

Figure

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  Fig. 1. (A) Magnetic resonance imaging of brain showing large intra-parenchymal hematoma involving left ganglio-capsular and frontotemporal regions with significant midline shift. (B) Post-operative computed tomography (CT) scan of brain showing hematoma evacuation. (C) CT angiography demonstrating multifocal luminal irregularities and narrowing (white arrowheads) of distal branches of bilateral middle and posterior cerebral arteries. (D) CT brain on twelfth postoperative day showing multiple acute infarcts (white arrows) in right middle cerebral arteries (MCA) territory. (E) Digital subtraction angiography performed in the second week showing severe proximal narrowing of left MCA (black thick arrow) and the anterior cerebral arteries (ACA) (black thin arrow). (F) Post intra-arterial milrinone injection of left internal carotid artery showing excellent reversibility of MCA and ACA abnormalities.

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