Pediatric STROKEPATRICIA TAN M.D, FAAMRPEDIATRIC PHYSIATRIST

Learning objectives

Review epidemiology and risk factors Patho-anatomy and pathophysiology Discuss clinical presentation and differential diagnosis Disease progression: natural history, disease stages, disease

trajectory (clinical features and presentation over time) Secondary or associated conditions and complications Essentials of assessment Rehabilitation management and treatments Cutting edge concepts and practice

Definition

NEUROLOGICAL INSULT OCCLUSION OR RUPTURE OF CEREBRAL

BLOOD VESSELS

ISCHEMIC, HEMORRHAGIC, COMBINATION

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Historical Aspect

Cerebral apoplexy, acute infantile hemiplegia, acute hemiplegia of childhood, congenital hemiplegia, hemiplegic cerebral palsy

Thomas Willis (1621-1675) in first documented case of pediatric stroke in 17th

century

Geovanni Battista Morgagni’s proposed lesions occur in the brain opposite the side of hemiplegia (1682-1771)

Matthew Baillie (1761- 1823) first described cerebral hemorrhage as consequence of disease of the blood vessels of the brain

ETIOLOGY

HEMORRHAGIC: Intracranial vessels

rupture and bleed into cerebral tissues

• Malformation• Traumatic injury• Bleeding diathesis

ISCHEMIC: Interruption in blood flow

• Inadequate systolic cardiac function

• Inappropriate vasoconstriction

• thromboembolic occlusion of the vessel

Epidemiology

Incidence: 2-13 per 100,000 children per year Hemorrhagic stroke 1.4 per 100,000 children

Intracerebral hemorrhage 1.1-5.2 per 100,000

Subarachnoid 0.4 per 100,000 children

Arterial ischemic stroke (AIS) 0.6-7.9 per 100,000 children, infants and children; more common in boys than in girls

55% of pediatric strokes are ischemic vs in adults, greater than 80%

Basilar artery stroke 0.037 per 100,000 children per year

Epidemiology: Risk Factors

Ethnicity: African Americans : 11% of patients with sickle cell disease will have a stroke before the age of 20

Arteriovenous malformation (AVM): the most common cause of hemorrhagic stroke after infancy, but can cause thrombotic stroke

Trauma: head and neck trauma at risk for ischemic event due to dissection of the carotid or vertebral arteries, hyperextension or rotational injuries

Dehydration

Genetic: Neurofibromatosis type 1, connective tissue disorders, Disorders of metabolism

Metabolic

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

Fabry disease

Homocystinuria

Menkes” disease

Mitochondrial encephalopathy and lactic acidosis and stroke-like episodes (MELAS)

Causes of ischemic stroke in children and young adults

Vascular: Noninflamamatory Vascular: Inflammatory

Arterial dissectionSpontaneousTraumaticConnective tissue (Marfan, Ehler Danlos)Fibromuscular dysplasiaTransient cerebral arteriopathyHypertensionRadiation vasculopathyMoyamoya: primary; secondary to SCD, NF I, Down, Williams, Cranial radiation, FMD, smoker, OC, Vasculitis, postinfectious vasculopathy, viral or bacterial infection, congenital heart disease

Takayasy arteritisGiant cell arteritisKawasaki diseasePolyarteritis nodosaVasculitis related to rheumatological Primary CNS angitisInfectious/postinfectious vasculitis: HIV, Varicella, bacterial meningitis,SyphilisTuberculosisfungal

Vasospasm following subarachnoid hemorrhageCongenital vessel hypoplasia

Risk Factors Migraine with aura, overuse of ergot alkaloids (childbearing,

Oral contraceptives) Substance abuse : amphetamines, ecstasy, cocaine,

phencyclidine (PCP), glue sniffing. Stimulants and heroin: vasculitis predisposing to infarction

Cardiac Congenital heart disease

Patent femoral ovale

Atrial sept; aneurysm

Atrial myxoma

Atrial fibrillation

Other arrythmia

Cardiomyopathy

Myocarditis

Myocardial infection

Cardiac surgery

Cardiac catheterization

Extracorporeal membrane oxygenation (ECMO)

Endocarditis

Rheumatic heart disease

Prosthetic valve

Causes of ischemic stroke in children and young adults

Hematologic: Inherited prothrombic states

Hematologic:AcquiredProthrombic state

Protein C deficiency Antiphospholipid antibody syndrome

Protein S deficiency L-asparaginaseAntithrombin III deficiency Pregnancy

Factor V Leiden gene mutation Oral contraceptives

Prothrombin gene mutation Protein losing enteropathyElevated homocysteine Disseminated intravascular

coagulationElevated lipoprotein LeukemiaPolycythemia vera Malignancy

Patho-anatomy/physiology

Cellular level: ischemic changes precipitate a series of events that result in tissue necrosis

Cell injury: activation of enzymes that degrade the cell’s proteins, genetic material, and lipids

Activation of N-methyl-D-aspartate receptors and formation of reactive oxygen species

Influx of inflammatory cells to site of injury

Further changes take place in area of ischemic focus over hours to days

Patho-anatomy/physiology

Middle cerebral artery most commonly affected in ischemic strokes

Hemorrhagic strokes are supratentorial

Cerebral venous sinus thrombosis : superficial venous system than within deep veins

Predisposing factors for AIS: mineralizing angiopathy and mild trauma immediately or shortly preceding stroke onset

Differential Diagnosis for hemorrhagic stroke in children

Disease Progression

Acute Subacute Chronic/stable

Abrupt onset focal neurological deficits or rapid decline in neuro function

Previously unrecognized or misdiagnosed strokes

Over 50% with lasting or permanent sequelae

Altered mental status or seizure: may be the only presenting symptom. Subtle signs, especially in infants

Stepwise or progressive decline in function over time

Majority of ped stroke achieve indepin ADLs. Children more likely to regain function in previously learned domain than achieve a new skill. Younger age is a risk factor that limits progress in ADL independence

Intracranial hemorrhage: nausea, vomiting, headache

Regression in function is rare. Recurrent stroke should be suspected if child develops new deficits

Morbidity and Mortality

10-25% leads to death

Secondary conditions and complicationsSeizure disorder

Hemiplegia/quadriplegia

Spasticity

Muscle contractures

Visual deficits

Speech and language impairment

Dysphagia

Cognitive impairment

Essentials of Assessment History

Time of symptom onset

Specific sensory or motor deficits, vision changes, dysphagia

Headache

Trauma

Birth and developmental history

Family history of clots, miscarriages, drug exposure

Screening for potential risk factors, including recent infections

Physical Examination

Closely monitor vital signs acutely Document seizure activity Signs of increased intracranial pressure, such as

papilledema Full neurological exam:

Mental status

Cranial nerves

Motor function

Sensory testing

Reflexes

Coordination

Later stages: ADL skills, transfer skills, functional mobility

Laboratory studies

Complete blood count (anemia, platelet disorders). If abnormal, peripheral smear

Metabolic panel (electrolyte abnormalities, dehydration)

Coagulation panel ( APTT,PT, lupus anticoagulant, anti-cardiolipin antibodies, ANA, anti B2GP1 Antibodies, platelet aggregation, factor VIII activity, d-dimers, thrombin time, factor V Leiden, Protein C antigen, Protein S antigen, plasminogen activator inhibitor, homocysteine, coagulation factor assays)

Inflammatory markers: ESR, C-Reactive protein

Maternal labs for syphilis testing (VDRL, toxicity screen)

Imaging

Cranial ultrasound:

safe and available may miss superficial and ischemic lesions

cannot be done after fontanelles

closed

primarily used to evaluate cranial

blood vessels

The optimal imaging depends on the child’s clinical stability

Imaging

• Quick and accurately depicts superficial or hemorrhagic lesions

• Confirms lesion location• Venous thrombosis and AIS may

be missed

Head CT

• Parenchymal abnormalities, non-ischemic lesions that mimic arterial or venous state

MRI, MRA MRV (magnetic

venography) may more accurately

define site of arterial or venous

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Supplemental assessment tools

EKG and echocardiogram: arrhythmia, thrombus, congenital cardiac defects leading to acute ischemic stroke

EEG: generalized activity suppression or subtle presentation

Neuropsychological testing: cognitive and memory problems

Predictors of outcomes:

Ischemic strokes: a low PedNIHSS is the best indicator of having no symptoms or mild symptoms in 3-6 monthsHemorrhagic strokes cause higher mortality in acute phase; inhouse mortality rate 6-9% ; larger volume of hemorrhage correlates to worse 30-day outcomes

ABCs, obtain IV, blood work Neuroprotective: head of

bed flat, NPO, IV fluids, normothermia, normotensive,

normoglycemic

• MRI/MRA head and neck to confirm stroke

If concerns for hemorrhage or increased

ICP, Head CT

• IV thrombolysis in select patients within 4.5 hours of last seen well, or mechanical thrombectomy within 24 hours of last seen well

Pediatric stroke code: early management of the child with stroke

Admit for close observation,

neuro checks 48-72 hours

Institute therapy for secondary

prevention (ASA or heparin)

Complete stroke work up (Echo,

hypercoagulable work up)

Pediatric stroke code: early management of the child with stroke

Management:

Surgical evacuation of hemorrhage not typically indicated, but may be useful for persistently increased ICP refractory to medical management

Correction of low platelet counts and factor deficiencies

Vitamin K deficiency: infants with biliary atresia, maternal ingestion of warfarin, phenytoin, barbiturates during pregnancy

Recombinant tissue plasminogen activator-less common for children

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Management: subacute,chronic

Sickle cell patients: long term red blood cell transfusion for secondary prevention; hydroxyurea for those who are unable to receive ongoing transfusion

Revascularization in patents with moyamoya disease

Heparin therapy for extracranial arterial dissection or cardioembolism; transitioned to warfarin , duration 3-6 months for dissection and one year or longer for cardio-embolic causes

Surgery for congenital vascular abnormalities, endovascular repair, radiosurgery

Discontinue oral contraceptives, consider other forms of birth control

Management: subacute,chronic

Elevated homocysteine: diet changes and/or folate, Vitamin B6, Vitamin B12

Long term use of prophylactic therapies LMWH in neonates: little information ; individuals with prothrombic conditions plus other risk factors: complex congenital heart disease, dehydration prolonged bedrest, methylene tetrahydro-folate reductase (MTHFR) mutation to normalize homocysteine

Individualized education plan, 504 plan to specify educational adaptations, educationally related therapy services, nursing services provided by school

Orthotics, adaptive equipment, medical management of spasticity: Baclofen, Zanaflex, Dantrolene

REHABILITATION MANAGEMENT AND TREATMENT

Class I recommendations

Early dysphagia screening : prevent pneumonia, malnutrition, dehydration and other complications

Enteral feedings initiated within 7 days who cannot swallow safely; NGT short term (2-3 weeks) for nutritional support

Enriched environments to increase cognitive activities

Treatment of aphasia: communication partner training

Targeted injection of Botulinum toxin into localized upper limb muscles or phenol to reduce spasticity

EXERCISES

Comprehensive inpatient rehabilitation: intensive repetitive

Constraint induced therapy or bilateral training therapy for upper extremity function

Range of motion

Strengthening

Balance: fall risk

Gait training

Developmental stimulation

Speech and language for communication and cognition

Swallowing therapy

REHABILITATION MANAGEMENT AND

TREATMENT Brace management: AFO with remediable gait impairments (foot drop) to improve mobility; wrist hand orthosis for upper extremity

Goal-directed upper limb training: motivation is the only individual characteristic unrelated to health state, that influences motor change and functional outcomes

Persistence with object-oriented tasks as well as manual abilities need to be considered when undertaking upper limb intervention

REHABILITATION MANAGEMENT AND TREATMENT

Class II Recommendations: Nutritional supplements Incorporating principles of

neuroplasticity into dysphagia rehab strategies

Strategy training or gesture training for apraxia

Music therapy for improving verbal memory

Compensatory strategies to improve memory functions internalized strategies (visual

imagery, semantic organization, spaced practice)

external memory assistive technology (notebooks, paging systems, computers)

REHABILITATION MANAGEMENT AND TREATMENT: Class II Recommendations

GROUP TREATMENT ACROSS

CONTINUUM OF CARE, USE OF

COMMUNITY-BASED APHASIA GROUPS

TELEREHABILITATION WHEN FACE TO

FACE TREATMENT NOT POSSIBLE OR

IMPRACTICAL

PHYSICAL MODALITIES (NMES OR VIBRATION ) TO

DECREASE SPASTICITY

TEMPORARILY AS AN ADJUNCT TO REHAB THERAPY

POSTURAL TRAINING AND TASK-ORIENTED

THERAPY FOR ATAXIA

CARDIOVASCULAR EXERCISES AND

STRENGTHENING INTERVENTIONS FOR RECOVERY OF GAIT

CAPACITY AND GAIT RELATED

MOBILITY TASKS

ROBOT ASSISTED MOVEMENT TRAINING TO

IMPROVE MOTOR FUNCTION AND MOBILITY AFTER

STROKE IN COMBINATION WITH

CONVENTIONAL THERAPY

REHABILITATION MANAGEMENT AND TREATMENT: Class III Recommendations

Drug therapy NMES Pharyngeal electrical stimulation Physical stimulation Transcranial direct-current

stimulation: anodal tDCS over left dorsolateral prefrontal cortex to improve language based complex attention (working memory)

Transcranial magnetic stimulation

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Prognosis

Hemiparesis recovers proximally to distally. Hand and foot/ankle function often have poor recovery, orthotics frequently required.

Cognitive and language deficits may remain after motor recovery.

ADL function may be more impaired in children who had stroke in a younger age, before they initially learn the skill

In a long term follow up study, all children finish high school, and many went to college.

About 60% of older than 16 were employed but only a few were financially independent

Early intervention and intensive therapy techniques should be encouraged

Care Coordination

In rehab setting, physiatry, pediatrician, physical therapy, occupational therapy, speech therapy, neuropsychology, nutrition, child life therapies, recreational therapies,

care coordinators, social workers, nursing staff

In acute care: critical care team, neurology, hematology, trauma, neurosurgery, physiatry

Multidisciplinary teams remain the standard of care for pediatric stroke patients

Patient and Family Education

CAUTIOUS OPTIMISM GOOD BALANCE OF HONESTY ABOUT PROBABLE LONG-TERM DEFICITS BUT

ALSO STRONG POTENTIAL FOR RECOVERY

CHILDREN’S HEMIPLEGIA AND STROKE ASSOCIATION: SUPPORT GROUP,

RESOURCES TO LEARN ABOUT STROKE REHAB, FIND EVENTS AND ACTIVITIES FOR

CHILDREN WHO HAVE HAD A STROKE

Emerging/unique interventions

Pediatric stroke outcomes measure Pediatric stroke recurrence and

recovery questionnaire PedNIHSS Remember to keep stroke in the

differential diagnosis of children with neurologic complaints: Headache Vomiting

Prevent misdiagnosis or delayed diagnosis

Cutting edge therapies and unique concepts and practice

Robotic therapy: used in adult stroke and children with cerebral palsy: expansion to pediatric stroke is a logical next step

Cutting edge therapies and unique concepts and practices: constraint induced therapy

•Any child who can understand simple directions •Does not have significant contractureMinimal hand function

•Adjunct interventions, functional electrical stimulation and botulinum toxin type A

•Primed, low frequency repetitive transcranial magnetic stimulation (Rtms) is a novel method to ramp up the potential for activity dependent plasticity in older children: no serious side effects

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