Pediatric STROKE - CHSLI · 2020-01-22 · Incidence: 2-13 per 100,000 children per year...
Transcript of Pediatric STROKE - CHSLI · 2020-01-22 · Incidence: 2-13 per 100,000 children per year...
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
occlusionThis Photo by Unknown Author is licensed under CC BY-NC
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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