Fatal reversible cerebral vasoconstriction syndrome: An unusual presentation of a rare disease

Abstract

The reversible cerebral vasoconstriction syndrome is a variable, segmental, and multifocal constriction of brain arteries, usually with a benign course. We describe the case of a 49-year-old woman who presented with headaches, visual symptoms, and seizures. Three days after admission, vasoconstriction areas were found in at least two vascular territories in two segments of the same arteries. The patient was admitted to the intensive care unit where her blood pressure was monitored and she received medical treatment. Surprisingly, the patient presented an unpredicted evolution in developing malignant cerebral edema on the seventh day after admission. She then suffered brain death and was taken to organ donation. A guided nervous system necropsy was later performed. The pathology discarded vasculitis and exhibited hemorrhage areas in the cerebral convexity. Herein, we discuss the most relevant aspects of cases with fulminant evolution reported in the literature. The reversible cerebral vasoconstriction syndrome is usually associated with fatal outcomes when patients exhibit focalization, their first neuroimaging typically shows disturbances, and a rapid clinical deterioration occurs. It is crucial to identify factors linked to poor prognosis and set intervention strategies and early prevention.

Keywords: stroke; brain hemorrhage; vasoconstriction; mortality; prognosis.

Figura 1. Estudios de neuroimágenes. A. Tomografía computarizada cerebral simple, reportada como normal. B-C. Resonancia magnética cerebral en la que se evidenció una hemorragia subaracnoidea en la convexidad de ambos hemisferios, sin cambios arteriales o venosos, ni aneurismas. D. Angiografía por resonancia cerebral con hemorragia subaracnoidea frontal bilateral y parietal izquierda y hallazgos de leucoencefalopatía posterior reversible. E. Tomografía computarizada de control en que se evidenció una nueva hemorragia intraparenquimatosa parieto-occipital izquierda. F-G. Panangiografía cerebral en la que se observó áreas de adelgazamiento con dilatación de los vasos sanguíneos, principalmente de la circulación posterior y anterior. H. Tomografía computarizada de control con nueva área de sangrado occipital izquierdo más superior, con drenaje a ventrículos, importante edema de las cisuras y compromiso principalmente de la fosa posterior

Figura 2. Resultados de la autopsia, microscopía simple. A. Cortes de la arteria cerebral posterior que no muestran vasculitis. Hematoxilina y eosina, 10X. B. Arteria vertebral normal. Hematoxilina y eosina, 20X. C. Vasos arteriales leptomeníngeos sin vasculitis, pero con foco vecino de hemorragia cortical (*). Hematoxilina y eosina, 10X. D. Corte de la duramadre con fondo hemorrágico (¤). Hematoxilina y eosina, 20X.