Transient ischemic attacks in a child with post-varicella arteriopathy and MTHFR homozigotic mutation C677T

Abstract

acidenTes isquÊmicos TransiTórios em criança com arTeriopaTia pós-varicela e muTação mThFr c677T homozigóTica. Chickenpox is the primary etiologic agent involved in 60% of cases and usually carries a good prognosis 2 . We report on a patient with multiple transients ischemic attacks (TIA) who presented a post-varicella artheriopathy (PVA) associated with MTHFR homozigotic mutation C677T. Moreover the diagnosis criteria and therapy of PVA is discussed. caseA three year-old boy was admitted to the emergency room (ER) with sudden onset of transient motor deficit of both left limbs. The neurological and general examination was normal. His past medical history was remarkable for primary varicella infection occurring five months before. In the ER he underwent blood tests, brain CT and EEG which displayed normal results. While performing EEG, he presented another deficit episode lasting for five minutes and similar to the one previously described. In the light of multiple TIA's, anticoagulation with heparin was initiated. However, he had three more TIA's in the same territory, and at the fifth day after admission had sudden onset of a left pyramidal syndrome with discrete hemiparesis.The heart evaluation including electrocardiogram and echocardiogram was normal. Noteworthy, concearning the analytical work-up, we underscore the serologic evidence of a non-recent VVZ infection (IgG+, IgM-) and the presence of a MTHFR homozigotic mutation C677T with normal homocystein and folic acid. All the remaining study was normal or negative and included hemogram, biochemistry, immunologic study, metabolic, protrombotic and CSF (cytochemistry, serologic, microbiologic and virus PCR including VVZ). The MRI with Angio-MRI performed at the second day after admission showed a recent stroke in the territory of right lenticulostriate arteries and a decreased flow on the M1-M2 transition of right MCA (Fig 1). A brain conventional angiography was performed to better study all the brain vascular system including the distal vessels. The intracranial es-

Vascular Complications of Varicella

“…In the literature varicella vasculopathy includes arterial ischemic stroke 2-4,9,10 as well as DVT. [5][6][7][8] The risk for both arterial and venous occlusion as complication of a varicella infection is significantly elevated in patients with congenital disorders of coagulation, such as protein S or protein C deficiency, and homozygous carriers of factor V Leiden or Methylene Tetrahydrofolate Reductase mutation; 5,[11][12][13][14] as well as in patients with concomitant sepsis. 15 Other risk factors for thrombotic complications associated with varicella infection include diabetes mellitus type 1, patent foramen ovale, severe iron deficiency anemia, Down syndrome and AIDS.…”

Common Features Between Stroke Following Varicella in Children and Stroke Following Herpes Zoster in Adults

“…To further define features of varicella-related stroke in children, we reviewed over 60 case reports of stroke following varicella. These reports span the decades before varicella vaccination was commonly administered, so all cases are considered to be wild-type varicella virus (Losurdo et al 2006;Eda et al 1983;Liu and Holmes 1990;Caekebeke et al 1990;Bodensteiner et al 1992;Ichiyama et al 1990;Shuper et al 1990;Tucciarone et al 1992;Yilmaz et al 1998;Hausler et al 1998;Silverstein and Brunberg 1995;Hayman et al 2001;Okanishi et al 2009;Rougeot et al 2006;Danchaivijitr et al 2006;Ueno et al 2002;Kimura et al 2002;Gomez-Gosalvez et al 2003;Beleza et al 2008;Hayes et al 2007;Lopez Medina et al 2002;West et al 2006;Tiah et al 2004;Guillot et al 2005;Morino et al 2009;Yaramis et al 2009;Singhal et al 2001;Aydin et al 2006;Cadavid et al 1999;Leis and Butler 1987;Kramer et al 1999;Darling et al 1995;Wieting et al 1997;Frank et al 1989;Spiegel et al 2010;Walker et al 1973;Hansen et al 2006;Nguyen et al 1994;Alehan et al 2002;…”

Acidente vascular encefálico isquêmico pós-varicela em crianças: série de sete casos com evolução de sequelas após quatro anos e revisão sistemática de literatura

“…Uma possível explicação para isso é a insuficiente literatura sobre o assunto em crianças, uma vez que a maioria dos estudos são relatos de casos isolados nos quais a coleta de líquor não foi realizada , conforme tabela 3. Beleza e cols 2008 103 Yaramis e cols 2009 104 Okanishi e cols 2009 105 Requero e cols 2009 106 Ciccone e cols 2010 93 Neuteboom…”