DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY CLINICAL INSIGHT

In vivo evidence of arterial wall inflammation in childhoodvaricella-zoster virus cerebral vasculopathy

ST�EPHANE DARTEYRE1 | ANNA HUBERT2 | ST�EPHANE CHABRIER1 | SOPHIE BESSAGUET3 | MARIE-ANGENGUYEN MOREL4

1 Department of Paediatric Physical Medicine and Rehabilitation, CHU Saint-�Etienne, Hopital Bellevue, Saint-�Etienne; 2 Department of Paediatric Neurology, CHULyon, Hopital Femme-M�ere-Enfant, Lyon; 3 Department of Paediatric Radiology, CHU Grenoble, Hopital Couple-Enfant, Grenoble; 4 Department of Paediatrics, CHUGrenoble, Hopital Couple-Enfant, Grenoble, France.

Correspondance to Dr St�ephane Chabrier, Service de M�edecine Physique et R�eadaptation P�ediatrique, Hopital Bellevue, CHU Saint-�Etienne, 42055 Saint-�Etienne Cedex2, France. E-mail: stephane.chabrier@chu-st-etienne.fr

doi: 10.1111/dmcn.12329

A previously healthy 4-year-old male was admitted to ourinstitution with acute headache, vomiting, and drowsiness5 months after varicella infection. Brain imaging revealed aright sylvian infarct due to an intracranial arterial lesion(see Fig. 1). Apart from detection of varicella-zoster virusDNA in cerebrospinal fluid by polymerase chain reactionassay, all aetiological investigations were normal. After 3

months on aspirin, there was no recurrence and his physi-cal examination remained normal. Magnetic resonanceangiography showed partial revascularization of the regionof the infarct.

This report fits the clinical definition of post-varicellaarteriopathy. The proposed mechanism for this frequentcause of childhood stroke is focal viral invasion of the

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Figure 1: 1.5-T brain magnetic resonance imaging/angiography with a 12-channel head-coil (Siemens Avanto, Erlangen, Germany). (a) Axial fluid-attenu-ated inversion recovery and (b) diffusion-weighted images: high intensity signal in the inferior division of the right median cerebral artery consistentwith acute ischemic lesions. (c) Time-of-flight images: inferior view of a maximum intensity projection of the circle of Willis and (d) 3D surface renderedanterior view, showing severe right carotid trifurcation stenosis and absence of visibility of the right median cerebral artery. Post-gadolinium axialT1-weighted images: (e) linear and (f) concentric/eccentric enhancement of the right (solid arrows) supraclinoid internal carotid artery associated withluminal narrowing compared to the left (open arrows) side, and no periarterial enhancement on the left side.

© 2013 Mac Keith Press 1

arterial wall, through a trigeminal and/or meningeal(observed in the present case) pathway, inducing acute vas-culitis. Only two neuropathological reports in immuno-competent children have been published.1,2 Yet these caseswere unusually severe and it is questionable if a similarmechanism is involved in the most common forms of thedisease.

Recent adult stroke literature shows that post-contrastmagnetic resonance imaging helps to distinguish vasculitis(in which enhancement is detected) from non-inflammatorycerebral arteriopathies (demonstrating parietal thickeningand a lack of enhancement).3 Moreover, in the former case,gadolinium-enhanced patterns contribute to the under-standing of the pathogenic process, as they are consistentwith neuropathological data. Hence, smooth, diffuse, andcircumferential parietal enhancement confined to the

vessels supplying the area of infarction is suggestive ofvasculitis.3

Our report suggests that such a mechanism could alsooccur in childhood varicella-zoster virus arteriopathy. Thepresent images are indeed clearly different from the physi-ological periarterial 1.5-T post-contrast appearance, whichis typically (1) thin, linear, and noncircumferential;(2) bilateral; and (3) does not involve the supraclinoidportion of the carotid artery.4 Further research is neededto assess the validity of this potential imaging diagnostictool to see if it has a prognostic or therapeutic value.

DISCLOSUREAll authors state that they have no interests which mightbe perceived as posing a conflict or bias.

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2 Developmental Medicine & Child Neurology 2013