The younger the child, the more nonspecific their symptoms may be.
Received Jul 6; Accepted Sep This article has been cited by other articles in PMC. It has an increasingly recognised impact on child mortality along with its outcomes and effects on quality of life of patients and their families. Clinical presentation and risk factors of paediatric stroke are different to those of adults therefore it can be considered as an indipendent nosological entity.
The relative rarity, the age-related peculiarities Management pediatric stroke the variety of manifested symptoms makes the diagnosis of paediatric stroke extremely difficult and often delayed. History and clinical examination should investigate underlying diseases or predisposing factors and should take into account the potential territoriality of neurological deficits and the spectrum of differential diagnosis of acute neurological accidents in childhood.
Neuroimaging in particular diffusion weighted magnetic resonance is the keystone for diagnosis of paediatric stroke and other investigations might be considered according to the clinical condition.
Despite substantial advances in paediatric stroke research and clinical care, many unanswered questions remain concerning both its acute treatment and its secondary prevention and rehabilitation so that treatment recommendations are mainly extrapolated from studies on adult population.
We have tried to summarize the pathophysiological and clinical characteristics of arterial ischemic stroke in children and the most recent international guidelines and practical directions on how to recognise and manage it in paediatric emergency.
Paediatric stroke, Arterial ischemic stroke, Cerebral arteriopathy, Sickle cell disease, Moyamoya, Antithrombotic treatment, Thrombolysis Background Stroke is defined as a sudden loss of brain function caused by a decreased cerebral blood flow.
While many efforts and important clinical trials have been allocated to increase knowledge about adult stroke, paediatric stroke often remains an under-recognized nosological entity even among paediatricians, although it is an important cause of lifelong disability with a human and economic impact on families and on the society [ 1 — 3 ].
The estimated incidence of paediatric stroke is 1—6 perchildren per year [ 4 — 6 ]. Perinatal stroke differs from paediatric stroke in some clinical and pathophysiological aspects, mainly overlapping with global hypoxic ischemic encephalopathy and presenting with more aspecific and generalised signs such as apnea, hypotonia, poor feeding, seizures and irritability [ 7 — 11 ].
Differently, in children focal neurological deficits such as hemiplegia are the most frequently reported signs and the most frequently affected artery is middle cerebral artery MCA.
In this review we have tried to focus on risk factors and underlying mechanisms, diagnosis, treatment, rehabilitation, knowledge translation and knowledge needs about paediatric arterial ischemic stroke AIS.
Risk factors for paediatric stroke Presumptive risk factors for paediatric stroke differ in children compared with adults. Whereas adult risk factors are primarily related to arrhythmias, obstructive atherosclerotic arteriopathies and socioeconomic status, these are rarely found to be related to stroke in children [ 1213 ].
In several studies, such as the International Paediatric Stroke Study IPSSa wide range of underlying systemic factors were reported in the setting of childhood stroke, in particular: However, in the majority of the children, no underlying systemic disease was found [ 14 ].
Furthermore, it represents the greatest predictor of recurrence, emphasising its role as a treatment target for secondary stroke prevention [ 1315 ]. The most common established arteriopathy in paediatric stroke is an acquired unilateral intracranial arteriopathy associated with basal ganglia stroke, characteristically involving the junction of the distal internal carotid artery, the proximal MCA and the proximal anterior cerebral artery ACA.
This condition was originally designed as a transient cerebral arteriopathy TCA and characterised by its time course, by an unilateral location, and by the absence of a long-term progression [ 1617 ]. On initial traditional imaging, TCA may be not distinguishable from a progressive arteriopathy such as Moyamoya disease MMD or from a vasculitis that presents unilaterally, with a different course and prognosis.
Few clinical and radiological parameters may help predict the course of childhood unilateral intracranial arteriopathy. It has been described that patients with progressive arteriopathy more often show arterial occlusion, ACA involvement and abnormal collateral vessels and that a mainly cortical localization is associated with poor functional outcome.
These arteriopathies are clinically important because their presence indicates an increased risk for recurrent stroke and vascular imaging of intracranial and neck vessels are imperative to detect them.
The underlying mechanisms for these unilateral, monophasic arteriopathies are poorly understood. Investigators originally proposed that the basis for this disorder was inflammatory, but currently the possible role of infection is the most plausible hypothesis.
It has been reported that in young children with AIS there is a 3-fold increase in preceding varicella infection compared with population rates and this prevalence was higher than the annual prevalence of chickenpox reported in the regional population [ 19 ].
The fact that post-varicella angiopathy, consisting in a non-progressive arteriopathy, has similar arterial imaging but a presumed infectious cause suggests that TCA might sometimes have a link to infectious mechanisms [ 17 ].
Furthermore, acute infection has been shown to be a risk factor and a potential trigger for spontaneous cervical artery dissection and upper respiratory infections URI have recently been related to transient arteriopathy.
In conclusion, although many underlying diseases have been reported in the setting of childhood AIS, emerging research demonstrates that non-atherosclerotic intracerebral arteriopathies in otherwise healthy children are prevalent. In particular, these conditions are related to parainfectious aetiology, inflammatory reaction and altered inflammation signalling [ 13 ].
Genetical factors and Moyamoya syndrome Genetically determined arteriopathies are increasingly recognised as a cause of childhood AIS. It is characterized by a usually bilateral progressive stenosis or occlusion of the intra-cranial internal carotid apices, involving the anterior and medial cerebral arteries.
Ina genetic arteriopathy caused by a deficiency of adenosine deaminase 2 ADA2 was reported with clinical features that included intermittent fever, lacunar strokes beginning in early childhood and livedoid rash; histopathological changes included compromised endothelial integrity, endothelial cellular activation and inflammation [ 1625 ].
Most paediatric stroke experts believe that coagulopathy represents a potential risk factor for stroke usually working in combination with other factors, rather than being an independent causative mechanism [ 2030 — 33 ].
Thus, it is reasonable to search for the more common prothrombotic states in patients with another identified stroke risk factor and in patients with a history of ischemic or thrombotic stroke in this case, oral contraceptives may be discontinued in adolescents.
If homocysteine is found to be elevated, aspecific diet or supplementation with folate, vitamin B6, or vitamin B12 might be considered and, in general, patients who are found to have a prothrombotic tendency should be referred to a haematologist [ 133435 ].
Sickle cell disease Children with SCD represent another important group of patients with a high risk of arteriopathies and stroke. Intranscranial Doppler TCD was found to be effective in identifying SCD patients at a high risk for stroke and currently primary prevention is possible through chronic red blood cell transfusions in SCD patients with elevated TCD velocity.
Metabolic diseases Metabolic infarctions are a rare but important condition for the paediatric population. Energy depletion leads to ischemic lesions in mitochondrial disorders.Nurses caring for children, whether in the emergency department, inpatient pediatric or the intensive-care unit, are likely to encounter a child who could experience an acute stroke-like event.
Nurses caring for children, whether in the emergency department, inpatient pediatric or the intensive-care unit, are likely to encounter a child who could experience an acute stroke-like event. Describe management of pediatric stroke, including treatment, course, recurrent stroke risk, and rehabilitation Disclosures As an organization accredited by the ACCME, Medscape, LLC, requires everyone who is in a position to control the content of an education activity to disclose all relevant financial relationships with any commercial .
Sickle cell disease (SCD) is a very common cause of pediatric stroke. 11% of patients with Sickle cell disease will have a clinically apparent stroke by age of 20 years.
Strokes may occur as early as 18 months of age, but most children present after five years of age. 25% of patients with Sickle cell disease will have a stroke by age Describe management of pediatric stroke, including treatment, course, recurrent stroke risk, and rehabilitation Disclosures As an organization accredited by the ACCME, Medscape, LLC, requires everyone who is in a position to control the content of an education activity to disclose all relevant financial relationships with any commercial interest.
Primary stroke centers established for adults provide an example of the multidisciplinary approach that can be applied to the evaluation and treatment of children who present with acute stroke. Guidelines for Urgent Management of Stroke in Children - Pediatric .