EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY 10 (2006) 152–153 Official Journal of the European Paediatric Neurology Society Case Study Post-varicella arteriopathy: Benefits of using serial transcranial Doppler examinations Christelle Rougeota,*, Christian Boissierb, Stéphane Chabriera a Services de pédiatrie, hôpital nord, 42055 Saint-Etienne Cedex 02, France et de médecine vasculaire, hôpital nord, 42055 Saint-Etienne Cedex 02, France b A R T I C L E I N F O A B S T R A C T Article history: A 28/12-year old boy suffered from hemiplegia secondary to a post-varicella arteriopathy. His Received 30 October 2005 clinical status improved and after 4 years of follow-up he had no recurrent stroke or Received in revised form transient ischemic attack. Regular improvement of arterial lesions, demonstated by serial 9 April 2006 transcranial Doppler investigations, excluded a progressive arteriopathy. Aspirin therapy Accepted 16 April 2006 was initiated and continued during 2,5 years. Transcranial Doppler is a portable, noninvasive tool that can be easily used on a regular basis to follow-up children with postvaricella arteriopathy. Keywords: Q 2006 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved. Childhood stroke Post-varicella arteriopathy Transcranial Doppler 1. Introduction Post-varicella arteriopathy is one of the leading causes of arterial ischaemic stroke in childhood. This basal cerebral vasculopathy generally shows a monophasic course with progressive improvement or complete regression of the lesions and no restenosis. 1,2Arterial follow-up, generally with MR angiography, is however mandatory as postvaricella arteriopathy can worsen and because other vasculopathies may mimic the disease at the beginning. 3,4 The method is non-invasive but requires complete immobility. We report the case of a child suffering from post-varicella arteriopathy followed-up with transcranial Doppler examinations. 2. Case study A previously well 2 8/12-year old right-handed boy was admitted to hospital with vomiting, somnolence and hemiplegia, which had progressively developed over 2 days. He had no personal or family history of thrombosis. His development was normal. He had suffered from varicella 1 month before. On admission, he had a complete right hemiplegia and an important decrease level of consciousness. He was afebrile and the general examination was normal. Cranial CT scan demonstrated a left fronto-parietal hypodensity. Laboratory investigations and cytochemical analysis of CSF were normal. Cranial MRI, performed on day 2 under general anaesthesia, confirmed an infarct in the deep and superficial left middle cerebral artery territory. MR angiography showed an interruption of the flow in the left middle cerebral artery, 1 cm distal to its origin. Other cerebral arteries were normal. * Corresponding author. Tel.: C33 4 77 82 83 01; fax: C33 4 77 82 81 08. E-mail address: stephane.chabrier@chu-st-etienne.fr (C. Rougeot). 1090-3798/$ - see front matter Q 2006 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.ejpn.2006.04.001 EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY 153 10 (2006) 152–153 Table 1 – Longitudinal peak systolic (PSV) and end diastolic (EDV) velocities measured by transcranial Doppler in the right and left middle cerebral arteries Time after admission (days) Right middle cerebral artery (cm sK1) 2 3 8 115 315 535 919 PSV Left middle cerebral artery (cm sK1) EDV PSV EDV 90 89 36 33 78 82 85 88 97 38 37 39 38 41 No flow Velocity not measurable 12 28 39 45 87 3 9 12 17 28 A bedside performed transcranial Doppler (Vivid 3, General Electric) did not visualise the left middle cerebral artery. Chest radiogram, EKG, transthoracic echocardiography and Doppler of the cervical carotid arteries were normal. Blood count, prothrombin time, activated partial thromboplastin time, protein C, S and antithrombin levels and homocysteinemia were normal. Activated protein C resistance, G20210A prothrombin gene mutation, lupus anticoagulant, anticardiolipin, anti-b2GPI and antinuclear antibodies were absent. Varicellazoster virus antibodies were detected in the serum without intrathecal production. PCR did not detect varicella-zoster virus DNA in the CSF. The child was treated with oral aspirin 100 mg daily. In order to assess the vascular outcome and the risk of recurrence, the follow-up was realised both clinically and by serial transcranial Doppler, which was repeated on days 3 and 8 and on months 4, 10, 18 and 30. On day 3, color Doppler detected a minimal revascularisation. Then, velocities in the left middle cerebral artery increased regularly (see Table 1). At 30 months, only a 10% asymmetry of the peak systolic velocity rates persisted. Aspirin was discontinued. During clinical follow-up of 4 years, the child experienced no recurrent stroke or transient ischemic attack. His neurological deficit progressively regressed to expressive aphasia and moderate right upper limb weakness and neglect. PSV asymmetry (%) 85 66 54 49 10 invasive but needs complete immobility, which requires sedation or general anaesthesia in young children. On the contrary transcranial Doppler is a portable method with no need for sedation, contrast material or radiation that can be easily used to provide reliable information about the intracranial vessels. It is the method of choice for screening children at high risk of cerebral infarct in sickle cell disease.5 In this report, the arterial lesion was located distally to the target of the transcranial Doppler. This explains the reduction of the velocities, in spite of increased peak flow velocities, which are classically observed at the site of significant stenosis of intracranial arteries. Thus, after an initial MR angiography, transcranial Doppler may be an interesting and non-invasive alternative to follow children suffering from post-varicella arteriopathy. Its interest should be weighted against its potential limitations, such as decreased sensibility in case of minimal arterial stenoses or in case of lesions located in other arterial segments than M1. 4. Disclosure The authors have reported no conflicts of interest. R E F E R E N C E S 3. Discussion According to the criteria defined by Lanthier and coll., the child had a stroke consistent with post-varicella arteriopathy.1 Antithrombotic therapy is frequently used in the acute stage as the stroke spontaneously recurs in about one-third of cases during the first months. The optimal duration of the treatment is a matter of debate. Arterial follow-up can help the decision since restroke rarely occurs once the stenosis begins to regress.1 Moreover, to our knowledge, the latest delay of recurrence in non-progressive post-varicella arteriopathy reported in the literature is 33 weeks.3 The children are generally followed with MR angiography. The method is non- 1. Lanthier S, Armstrong D, Domi T, de Veber G. Post-varicella arteriopathy of childhood: natural history of vascular stenosis. Neurology 2005;64:660–3. 2. Chabrier S, Rodesch G, Lasjaunias P, Tardieu M, Landrieu P, Sébire G. Transient cerebral arteriopathy: a disorder recognized by serial angiograms in children with stroke. J Child Neurol 1998;13:27–32. 3. Ganesan V, Kirkham FJ. Mechanisms of ischaemic stroke after chickenpox. Arch Dis Child 1997;76:522–5. 4. Häusler MG, Ramaekers VTh, Reul J, Meilicke R, Heimann G. Early and late onset manifestations of cerebral vasculitis related to varicella zoster. Neuropediatrics 1998;29:202–7. 5. Adams JR, Pavlakis S, Roach ES. Sickle cell disease and stroke: primary prevention and transcranial Doppler. Ann Neurol 2003;54:559–63.