Case Report and Review of the Literature: Fatal Reversible Cerebral Vasoconstriction Syndrome

Background: A fatal outcome occurs in 2% of patients with Reversible Cerebral Vasoconstriction Syndrome (RCVS). Due to its rarity, guidelines for the management of the most severe forms of RCVS are lacking.Case presentation: Here, we describe the case of a 55 year-old woman who died from complications of RCVS and reviewed patients with fatal outcome reported in the literature. In our patient, the first episode of neurological deterioration was preceded by an increase of cerebral blood flow velocities assessed with transcranial Doppler. A fatal evolution could not be prevented despite therapeutic escalation consisting of multiple non-invasive and invasive treatments including cervical sympathetic bloc and continuous arterial infusion of nimodipine at the site of severe vasoconstriction.Conclusion: This case and the review of literature illustrate the challenges in the management of patients with severe RCVS. We describe here how monitoring of cerebral blood flow might help anticipate clinical worsening at the beginning of the disease and propose novel invasive and non-invasive therapeutic strategies based on monitoring of neurophysiological parameters.

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Three paediatric patients with reversible cerebral vasoconstriction syndromes

Cephalalgia ◽

10.1111/j.1468-2982.2009.01955.x ◽

2009 ◽

Vol 30 (3) ◽

pp. 354-359 ◽

Cited By ~ 26

Author(s):

H-Y Liu ◽

J-L Fuh ◽

J-F Lirng ◽

S-P Chen ◽

S-J Wang

Keyword(s):

Blood Flow ◽

Cerebral Blood Flow ◽

Magnetic Resonance Angiography ◽

Neurological Deficits ◽

Channel Blockers ◽

Cerebral Vasoconstriction ◽

Paediatric Patients ◽

History Of ◽

Blood Flow Velocities ◽

Secondary Causes

Reversible cerebral vasoconstriction syndromes (RCVS) occur predominantly in middle-aged women. Only one child with RCVS has ever been reported. We report three boys (aged 10, 16 and 10 years) who had multiple thunderclap headaches, including one boy with bath-related attacks. None of them had secondary causes. All their magnetic resonance angiography demonstrated reversible cerebral vasoconstrictions of the major arteries with increased cerebral blood flow velocities on transcranial colour-coded sonography studies. Two of the three had hypertensive surges during headache attacks. None of the three boys had seizure, stroke or other neurological deficits. However, a left occipital subcortical lesion of unknown nature was noted in one boy. The efficacy of calcium channel blockers was variable in these three boys. This report suggests that, although rare, RCVS should be considered in paediatric patients if they present with a history of multiple attacks of thunderclap headache.

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Patterns of Cerebral Blood Flow and Systemic Hemodynamics During Arithmetic Processing

Journal of Psychophysiology ◽

10.1027/0269-8803.22.2.81 ◽

2008 ◽

Vol 22 (2) ◽

pp. 81-90 ◽

Cited By ~ 21

Author(s):

Natalie Werner ◽

Neval Kapan ◽

Gustavo A. Reyes del Paso

Keyword(s):

Blood Pressure ◽

Blood Flow ◽

Cerebral Blood Flow ◽

Time Course ◽

Doppler Sonography ◽

Transcranial Doppler Sonography ◽

Systemic Hemodynamics ◽

Heart Period ◽

Blood Flow Velocities ◽

Flow Velocities

The present study explored modulations in cerebral blood flow and systemic hemodynamics during the execution of a mental calculation task in 41 healthy subjects. Time course and lateralization of blood flow velocities in the medial cerebral arteries of both hemispheres were assessed using functional transcranial Doppler sonography. Indices of systemic hemodynamics were obtained using continuous blood pressure recordings. Doppler sonography revealed a biphasic left dominant rise in cerebral blood flow velocities during task execution. Systemic blood pressure increased, whereas heart period, heart period variability, and baroreflex sensitivity declined. Blood pressure and heart period proved predictive of the magnitude of the cerebral blood flow response, particularly of its initial component. Various physiological mechanisms may be assumed to be involved in cardiovascular adjustment to cognitive demands. While specific contributions of the sympathetic and parasympathetic systems may account for the observed pattern of systemic hemodynamics, flow metabolism coupling, fast neurogenic vasodilation, and cerebral autoregulation may be involved in mediating cerebral blood flow modulations. Furthermore, during conditions of high cardiovascular reactivity, systemic hemodynamic changes exert a marked influence on cerebral blood perfusion.

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Non-invasive assessment of cerebral blood flow using selective MR-Arterial Spin Labeling (ASL): First Experience

Klinische Neurophysiologie ◽

10.1055/s-0030-1250925 ◽

2010 ◽

Vol 41 (01) ◽

Author(s):

R Wiest ◽

K Jann ◽

J Gralla ◽

M Hauf ◽

J Wang ◽

...

Keyword(s):

Blood Flow ◽

Cerebral Blood Flow ◽

Arterial Spin Labeling ◽

Spin Labeling ◽

Non Invasive

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Double-pulse laser illumination method for measuring fast cerebral blood flow velocities in the deep brain using a fiber-bundle-based endomicroscopy system

Biomedical Optics Express ◽

10.1364/boe.9.002699 ◽

2018 ◽

Vol 9 (6) ◽

pp. 2699 ◽

Cited By ~ 2

Author(s):

Minkyung Kim ◽

Jinki Hong ◽

Hyun-joon Shin

Keyword(s):

Blood Flow ◽

Cerebral Blood Flow ◽

Pulse Laser ◽

Fiber Bundle ◽

Double Pulse ◽

Laser Illumination ◽

Illumination Method ◽

Blood Flow Velocities ◽

Double Pulse Laser ◽

Deep Brain

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RENAL VERSUS CEREBRAL BLOOD FLOW VELOCITIES IN PRETERM INFANTS WITH PATENT DUCTUS ARTERIOSUS

Pediatric Research ◽

10.1203/00006450-198911000-00037 ◽

1989 ◽

Vol 26 (5) ◽

pp. 505-505

Author(s):

T Bõrnelburg ◽

G Jorch

Keyword(s):

Blood Flow ◽

Cerebral Blood Flow ◽

Patent Ductus Arteriosus ◽

Preterm Infants ◽

Ductus Arteriosus ◽

Blood Flow Velocities ◽

Patent Ductus ◽

Flow Velocities

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Longitudinal changes in cerebral blood flow velocities in different clinical courses of migraine

Cephalalgia ◽

10.1177/0333102416658715 ◽

2016 ◽

Vol 37 (10) ◽

pp. 927-937 ◽

Cited By ~ 5

Author(s):

Mi Ji Lee ◽

Min Kyung Chu ◽

Hanna Choi ◽

Hyun Ah Choi ◽

Chungbin Lee ◽

...

Keyword(s):

Blood Flow ◽

Cerebral Blood Flow ◽

Cerebral Artery ◽

Posterior Cerebral Artery ◽

Clinical Course ◽

Episodic Migraine ◽

Longitudinal Changes ◽

Blood Flow Velocities ◽

Headache Clinic ◽

Flow Velocities

Objective To assess longitudinal changes in cerebral blood flow velocities (ΔCBFVs) according to the clinical course of migraine. Methods We retrospectively included migraine patients with two or more attacks per month at baseline who were followed up within 2 years with transcranial Doppler in a tertiary headache clinic. ΔCBFVs were analyzed in relation to clinical courses, defined as remission (0–1 headache days/month), persistence (2–14/month), or progression (≥15/month) in episodic migraine (EM), and conversion to EM (<15/month) and persistence (≥15/month) in chronic migraine (CM). Results A total of 166 patients (90 EM and 76 CM) were included. In EM, the remission group ( n = 30) showed a decrease in CBFV in the middle cerebral artery (MCA) and the basilar artery (BA). The progression group ( n = 10) showed increasing CBFVs in the bilateral MCAs. Patients with the persistence course ( n = 50) showed generally unchanged CBFVs. In CM, ΔCBFVs decreased in the BA and increased in the posterior cerebral artery (PCA) after conversion to EM ( n = 61), whereas they remained unchanged in the persistence group ( n = 15). In all patients, % change in headache days was positively correlated with the %ΔCBFVs of the bilateral MCAs and the BA. Conclusions CBFV changes are associated with the different clinical courses of migraine. The association is more prominent in EM than CM.

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