Overview
Definition:
Pediatric stroke refers to the onset of neurological deficits caused by ischemic or hemorrhagic cerebrovascular events in children up to 18 years of age
It is a critical condition with significant short-term and long-term morbidity
Differentiating between causes, particularly Sickle Cell Disease (SCD)-related stroke and non-SCD arteriopathies, is crucial for effective management.
Epidemiology:
The incidence of pediatric stroke is approximately 1.7-13 per 100,000 children per year, with ischemic stroke being more common than hemorrhagic
In children with SCD, the cumulative risk of stroke by age 18 is about 11%, with a high recurrence rate
Non-SCD arteriopathies encompass a broad range of conditions including vasculitis, Moya Moya disease, and fibromuscular dysplasia, each with varying prevalence.
Clinical Significance:
Pediatric stroke can lead to permanent neurological disability, including motor deficits, cognitive impairment, seizures, and visual disturbances
Early and accurate diagnosis, followed by appropriate management tailored to the underlying etiology, is vital to minimize brain injury, prevent recurrence, and optimize long-term outcomes for affected children
DNB and NEET SS examinations frequently assess the understanding of stroke etiologies in this age group.
Clinical Presentation
Symptoms:
Sudden onset of focal neurological deficits
Hemiparesis or hemiplegia
Seizures, often focal
Altered consciousness or lethargy
Aphasia or dysarthria
Visual field defects
Vomiting or headache (more common in hemorrhagic stroke)
In infants, irritability, feeding difficulties, or developmental regression.
Signs:
Focal neurological deficits on examination, including motor weakness, sensory loss, abnormal reflexes, or gait abnormalities
Cranial nerve palsies
Funduscopic examination may reveal retinal hemorrhages or exudates in SCD
Signs of systemic illness may be present in cases of vasculitis.
Diagnostic Criteria:
Diagnosis of pediatric stroke is primarily based on clinical presentation and neuroimaging confirmation
Specific diagnostic criteria for underlying causes like SCD (e.g., transcranial Doppler findings, MRI evidence of infarcts in context of known SCD) or arteriopathies (e.g., imaging patterns of Moya Moya, evidence of inflammation in vasculitis) are used to guide further management.
Diagnostic Approach
History Taking:
Detailed birth history (hypoxia, birth trauma)
Family history of stroke, SCD, or clotting disorders
History of previous transient ischemic attacks (TIAs)
Recent infections or inflammatory conditions
Immunization status
Presence of sickle cell trait or disease
Medications
Trauma history.
Physical Examination:
Complete neurological examination, focusing on motor function, sensation, coordination, cranial nerves, reflexes, and gait
Assessment for signs of systemic illness, including fever, rash, joint swelling, or lymphadenopathy
Cardiovascular examination to detect murmurs or arrhythmias
Examination for signs of SCD (e.g., pallor, jaundice, hepatosplenomegaly).
Investigations:
Neuroimaging: MRI brain with diffusion-weighted imaging (DWI) is the gold standard for detecting ischemic stroke
MRI angiography (MRA) or CT angiography (CTA) for vessel assessment
Transcranial Doppler (TCD) ultrasonography is crucial for identifying children with SCD at high risk of stroke, often showing increased flow velocities
Echocardiography to rule out cardiac embolic sources
Laboratory tests: Complete blood count (CBC) with peripheral smear (}$HbSS$ on electrophoresis confirms SCD)
Coagulation profile (PT, aPTT, INR)
Antiphospholipid antibodies
Inflammatory markers ($ESR$, $CRP$)
Genetic testing for thrombophilias if indicated
Lumbar puncture if meningitis or encephalitis is suspected.
Differential Diagnosis:
Migraine with aura
Todd's paresis
Intracranial hemorrhage from trauma or bleeding disorders
Seizures without stroke
Brain tumors
Metabolic disorders
Intracranial infections (abscess, encephalitis)
Functional neurological deficits.
Management Pediatric Stroke
Initial Management:
Immediate assessment of airway, breathing, and circulation
Stabilization of vital signs
Rapid neuroimaging (MRI brain) to confirm stroke and determine type (ischemic/hemorrhagic) and extent
If ischemic, consider thrombolytic therapy (e.g., alteplase) within the therapeutic window in select cases, adhering to pediatric protocols
Management of seizures with anticonvulsants (e.g., levetiracetam, phenytoin).
Medical Management Scd:
For SCD-related ischemic stroke: Exchange transfusion to reduce HbS levels to <30% is a cornerstone therapy to prevent recurrence
Long-term management involves chronic transfusion therapy or hematopoietic stem cell transplantation
Hydroxyurea may be used to increase fetal hemoglobin, reducing sickling episodes.
Medical Management Arteriopathy:
Management depends on the underlying arteriopathy
Vasculitis: Immunosuppressive therapy (corticosteroids, cyclophosphamide, IVIG)
Moya Moya disease: Antiplatelet agents (aspirin) and management of risk factors
Consider surgical revascularization for progressive disease
Other arteriopathies: Management of hypertension, hyperlipidemia, and risk factor modification.
Supportive Care:
Multidisciplinary team approach involving pediatric neurologists, hematologists, neurosurgeons, rehabilitation specialists, and social workers
Intensive monitoring for neurological deterioration and complications
Nutritional support
Physical, occupational, and speech therapy for rehabilitation
Psychosocial support for the child and family.
Complications
Early Complications:
Cerebral edema
Hemorrhagic transformation of ischemic infarcts
Increased intracranial pressure
Status epilepticus
Hydrocephalus
Extension of infarct or new infarcts.
Late Complications:
Long-term neurological deficits (hemiparesis, cognitive impairment, speech disorders, visual impairment)
Epilepsy
Behavioral and emotional problems
Learning disabilities
Recurrent stroke
Developmental delay.
Prevention Strategies:
For SCD: Regular TCD screening to identify children at high risk
Prompt initiation of transfusion therapy
Adherence to hydroxyurea or chronic transfusion protocols
For non-SCD arteriopathies: Early diagnosis and treatment of underlying inflammatory or genetic conditions
Aggressive management of cardiovascular risk factors
Prompt evaluation of neurological symptoms.
Prognosis
Factors Affecting Prognosis:
Age at stroke onset (younger age generally has better recovery potential)
Etiology of stroke (SCD-related strokes can have high recurrence)
Severity of initial neurological deficit
Extent of brain injury on imaging
Promptness and adequacy of treatment
Presence of complications
Access to rehabilitation services.
Outcomes:
Outcomes vary widely
Some children have complete recovery, while others may have significant long-term disabilities
Recurrence is a major concern, especially in SCD
Early and aggressive management improves functional outcomes and reduces the risk of recurrence.
Follow Up:
Long-term, regular follow-up with pediatric neurology and hematology (if SCD is present) is essential
This includes monitoring for recurrent strokes, managing sequelae, ongoing rehabilitation, educational support, and neurodevelopmental assessments
Periodic neuroimaging and TCD may be indicated based on the etiology and clinical course.
Key Points
Exam Focus:
Distinguishing features of SCD stroke (high recurrence, TCD findings, exchange transfusion management) from non-SCD arteriopathies (vasculitis, Moya Moya, fibromuscular dysplasia)
Management of acute ischemic stroke in children (thrombolysis, antiplatelets)
Role of neuroimaging and TCD in diagnosis and risk stratification
Long-term rehabilitation and secondary prevention strategies.
Clinical Pearls:
Always consider stroke in a child presenting with sudden focal neurological deficits
TCD is a critical screening tool in children with SCD
Exchange transfusion is life-saving for SCD-related ischemic strokes
A multidisciplinary approach is paramount for optimal care
Educate parents about warning signs and the importance of adherence to treatment protocols.
Common Mistakes:
Delaying neuroimaging in suspected stroke
Underestimating the risk of recurrence in SCD
Inadequate management of seizures
Failing to consider non-SCD arteriopathies in older children and adolescents
Inappropriate use of anticoagulation in certain types of pediatric stroke without clear indication
Not initiating prompt rehabilitation.