Stroke in children: need for increased awareness.
Despite being rare, stroke in children is one of the leading causes of severe long term disabilities in this age group. Fifty to 80% of children surviving stroke will have permanent neurological deficit, more frequently hemiparesis or hemiplegia, but also sensory, visual and cognitive impairment, and seizures. (1)
Additionally, stroke is one of the top 10 causes of death in children. Stroke in children is rare, with a reported incidence of 1.3 to 13.0 cases /100,000. (2) Stroke poses a significant toll for the patients and their families.
Clinical presentation can involve non-focal neurological signs, such as headache, altered mental status, seizures, or a transient neurological deficit. Absence of focal signs results in delayed diagnosis with increased recurrence, mortality and complications. Delay in diagnosis of stroke up to 72 hours has been reported in children, with significant fraction of this time occurring in hospitals. (3)
In this paper we present a pediatric patient with stroke with non focal presentation.
A previously healthy 11 year-old female experienced the new onset of severe headaches for one week. The pain occurred multiple times during the day, lasting less than one hour. She went to the local ER where a brain CT demonstrated a sub-acute ischemic lesion in the right caudate nucleus (figure 1, A). She was discharged home and referred to child neurology clinic. When she was seen five days later, her symptoms had not changed, and her exam remained non focal. In order to investigate underlying causes of her CT finding, she was admitted. General laboratory investigations including CBC and metabolic panel were normal. A hypercoagulable workup (protein C, protein S, factor II mutation, antithrombin III level, lupus anticoagulant, MTHFR mutation, cryoglobulin, homocystine, cardiolipin antibody, lipoprotein (a), beta-2 glycoprotein) showed abnormality of Factor V Leiden with heterozygousity for R506Q polymorphism. MTHFR showed homozygousity for C667T. Homocystine was within normal limits. TTE was unremarkable. Intra-and extra-cranial Magnetic Resonance Angiography (MRA) was normal. MRI of the brain demonstrated region of restricted diffusion in the head of right caudate nucleus (figure 1, B). During her hospital stay she became asymptomatic. She was started on aspirin and folic acid, and was discharged home. She is doing well 18 months later.
Approximately 55% of children's strokes are ischemic in nature, and 45% are hemorrhagic. (4)
Congenital heart disease and sickle cell disease (SCD) are the most common predisposing factors for arterial ischemic stroke in children. Children's stroke recurs in up to 25% of cases; mortality is higher after recurrence. (5) Children with hypercoagulability or vascular causes (vasculopathies and cerebral arteriopathies) are at increased risk of recurrence. (6)
Our patient was discharged home despite the identification of a subacute ischemic lesion on brain CT. The incomplete workup prior to discharge hindered early recognition of treatable risk factors and put her at risk of recurrence.
In order to minimize the risk of recurrence, a comprehensive evaluation of a child with stroke should at least exclude vascular and cardiac causes (by MRA or CTA and cardiac echo), and include a complete hypercoagulablility work-up.
Recommendations for management of stroke in infants and children have recently been published. (4) Specific treatment is suggested for different etiologies including aspirin or anticoagulation for prothrombotic conditions, anticoagulation for arterial dissection, and exchange transfusion for SCD. Safety of anticoagulation has been demonstrated in neonates with CVST. (7) Although tPA is not currently recommended in children, a multicentric clinical trial to evaluate safety of this drug is under way.
With treatment options becoming available in children, it is critical to consider stroke in the differential diagnosis for every child presenting with new onset of acute neurological deficit, severe headache, altered mental status, or seizures, and to obtain urgent and complete evaluation in the emergency room including a brain MRI. The same holds true for any infant experiencing seizures after cardiac surgery, or following prolonged or complicated delivery. (8) Referral to pediatric neurology for thorough evaluation of possible risk factors, including cardiac, vascular, infectious and hyper-coagulable factors for accurate diagnosis and correct management in this age group is strongly suggested.
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Despite improved availability of rapid, non-invasive diagnostic techniques for diagnosing stroke, rapid access to care and transfer to specialized service are often delayed particularly in remote, underserved areas of the state.
Increased awareness is needed among primary care and ER physicians, as well as among the general public, about occurrence of stroke in children. A high index of suspicion is necessary in this age group, as accurate and timely diagnosis and treatment, and age-appropriate rehabilitation can minimize death and disability.
We thank Dr. Laurie Gutmann for her inputs and suggestions and for her final review of this paper.
(1.) Nelson KB, Lynch JK. Stroke in newborn infants. Lancet Neurol 2004;3(3):150-8.
(2.) Mallick AA, O'Callaghan FJ. The epidemiology of childhood stroke. Eur J Paediatr Neurol 2010;14(3):197-205.
(3.) Mekitarian Filho E, Carvalho WB. Stroke in children. J Pediatr (Rio J) 2009;85(6):46979.
(4.) Roach ES, Golomb MR, Adams R, Biller J, Daniels S, Deveber G, Ferriero D, Jones BV, Kirkham FJ, Scott RM, Smith ER. Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke 2008;39(9):2644-91.
(5.) DeVeber G. In pursuit of evidence-based treatments for paediatric stroke: the UK and Chest guidelines. Lancet Neurol 2005;4(7):432-6.
(6.) Fullerton HJ, Wu YW, Sidney S, Johnston SC. Risk of recurrent childhood arterial ischemic stroke in a population-based cohort: the importance of cerebrovascular imaging. Pediatrics 2007;119(3):495-501.
(7.) Moharir MD, Shroff M, Stephens D, Pontigon AM, Chan A, MacGregor D, Mikulis D, Adams M, deVeber G. Anticoagulants in pediatric cerebral sinovenous thrombosis: a safety and outcome study. Ann Neurol 2010;67(5):590-9.
(8.) Jordan LC. Assessment and treatment of stroke in children. Curr Treat Options Neurol 2008;10(6):399-409. Amy Kathryn Gessford, DO
P. Pergami, MD, PhD
N. Seemaladinne, MD
Department of Pediatrics, Child Neurology, West
Virginia University, Morgantown, WV.
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|Title Annotation:||Scientific Article|
|Author:||Pergami, P.; Seemaladinne, N.|
|Publication:||West Virginia Medical Journal|
|Article Type:||Case study|
|Date:||Nov 1, 2012|
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