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Syndrome de vasoconstriction cérébrale segmentale réversible ou angéite primitive du Systeme nerveux central?

Published online by Cambridge University Press:  02 December 2014

Philip Gerretsen  and
Ralph Z. Kern
Philip Gerretsen
Affiliation:
Division of Neurology, Mount Sinai Hospital, University Health Network, and the University of Toronto, Toronto, Ontario, Canada
Ralph Z. Kern
Affiliation:
Division of Neurology, Mount Sinai Hospital, University Health Network, and the University of Toronto, Toronto, Ontario, Canada
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Abstract

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Background:

Reversible Cerebral Vasoconstriction Syndrome (RCVS) may present as thunderclap headache (TCH), accompanied by reversible cerebral vasospasm and focal neurological deficits, often without a clear precipitant. RCVS may be mistaken for Primary Angiitis of the Central Nervous System (PACNS) due to the presence of similar angiographic features of segmental narrowing of cerebral arteries. We discuss the clinical features of a young female migraine patient who developed TCH and was found to have RCVS following initial treatment with corticosteroids for PACNS, in the context of a systematic review of the available medical literature.

Methods:

A Medline™ search was performed to identify all case reports since 1966 describing RCVS and PACNS that provide sufficient clinical detail to permit diagnostic classification according to published criteria. RCVS included case studies in which there was angiographic or transcranial Doppler ultrasound evidence of near-to-complete resolution of cerebral vasoconstriction in the absence of a well-recognized secondary cause. PACNS included reports of histologically confirmed PACNS either through biopsy or necropsy.

Results:

Reversible Cerebral Vasoconstriction Syndrome occurs primarily in females and is characterized by sudden, severe headache at onset, normal CSF analysis, vasoconstriction involving the Circle of Willis and its immediate branches, and angiographic or TCD ultrasound evidence of near-to-complete vasospastic resolution within 1-4 weeks. It occurs typically in the context of vasoconstrictive drug use, the peripartum period, bathing, and physical exertion.

Conclusion:

Initial and follow-up (within 4 weeks) non-invasive angiographic studies are indicated in patients who present with TCH or who have clinical presentations that could be consistent with RCVS or PACNS in the absence of a well-recognized secondary cause, such as subarachnoid haemorrhage. Early reversibility of cerebral vasospasm is the key neuroradiological feature that supports the clinical diagnosis of RCVS.

Résumé:

RÉSUMÉ: <span class='italic'> <span class='bold'>Contexte</span></span>:

Une céphalée en coup de tonnerre (CCT) peut etre la manifestation initiale du syndrome de vasoconstriction cérébrale segmentaire réversible (SVCSR), accompagnée par un vasospasme cérébral réversible et des déficits neurologiques focaux, souvent sans facteur précipitant évident. Le SVCSR peut etre confondu avec l'angéite primitive du système nerveux central (APSNC) à cause de la présence de manifestations angiographiques similaires dans ces deux pathologies, soit des rétrécissements segmentaires au niveau d'artères cérébrales. Nous discutons des manifestations cliniques observées chez une jeune femme migraineuse qui a consulté pour une CCT et chez qui on a diagnostiqué un SVCSR après un traitement initial par des corticostéroi'des pour une APSNC. Nous présentons également une revue systématique de la littérature médicale actuelle sur ce sujet.

<span class='italic'> <span class='bold'>Méodes</span></span>:

Nous avons effectué une recherche dans la base de données Medline™ afin d'identifier toutes les observations qui décrivent le SVCSR et l'APSNC depuis 1966 et qui fournissent suffisamment de détails cliniques pour permettre une classification diagnostique selon les critères publiés. Nous avons relevé des études de cas de SVCSR comportant des données d'angiographie ou d'échographie Doppler transcränienne (DTC) sur la régression complète ou presque complète de la vasoconstriction cérébrale, en l'absence d'une cause secondaire évidente. Nous avons identifié des comptes-rendus d'APSNC confirmées en anatomopathologie sur des spécimens obtenus soit par biopsie ou par autopsie.

<span class='italic'> <span class='bold'>Réltats</span></span>:

Le SVCR survient principalement chez les femmes et il se caractérise par une céphalée subite, sévère d'amblée, une analyse du LCR normale, une vasoconstriction impliquant l'hexagone de Willis et ses branches immédiates et une régression complète ou quasi complète du vasospasme en 1 à 4 semaines constatée à l'angiographie ou à l'échographie DTC. Il survient typiquement dans le contexte de la prise de médicaments vasoconstrictifs, en période péripartum, lors d'un bain ou lors de l'activité physique.

<span class='italic'> <span class='bold'>Conclusions</span></span>:

On devrait effectuer des études angiographiques non effractives au départ et au cours du suivi chez les patients qui consultent pour une CCT ou dont le tableau clinique est compatible avec un SVCSR ou une APSNC en l'absence d'une cause secondaire évidente, telle une hémorragie sous-arachnoi'dienne. La réversibilité précoce du vasospasme cérébral est la manifestation neuroradiologique clé qui étaie le diagnostic clinique du SVCSR.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 34 , Issue 4 , November 2007 , pp. 467 - 477
Copyright
Copyright © The Canadian Journal of Neurological 2007

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