MOHAMMAD EZADEEN

House paediatrecianUmdurman pediatrics Hospital-SUDAN

MOHEZDNSR@HOTMAIL.COM

STROKE IN CHILDHOOD

WHO DEFINITION OF STROCK 2004

• “A clinical syndrome in which there is rapidly developing signs of focal or global disturbance of cerebral functions, lasting more than 24 hours or leading to death, with no apparent causes other than of vascular origin”

INCIDENCE

• One of the top 10 causes of death in childhood

• Hemiplegia secondary to vascular disorders occurs in children with an incidence of 1–3/100,000 per year

Neonatal stroke: 28/100,000 live births

Several studies have found that pediatric ischemic stroke is more common in boys than in girls

Questions should be answered

• WHAT IS THE LESION?• WHERE IS THE LESION?• WHAT IS THE CAUSE?

WHAT IS THE LESION

• FOCAL• SYSTEMIC• DISSEMINATED

Where is the lesion

• CORTEX• CORONA RADIATA• INTERNAL CAPSULE• BRAIN STEM• SPINAL CORD

Case 1

10 YEARS RT.HANDED BOY PRESENTED WITH SUDDEN ATTACH OF ATTAXIA VERTIGO .OE LEFT HORNER , HORIZONTAL NYSTAGMUS,ABSENT GAG REFLEX,LOSS OF PINPRINK SENSATION OF LEFT .FACE AND LOSS OF TEMP AND PAIN SENSATION OF THE RT.BODY WITH NO APPARENT MOTOR DYSFUNCTION.WHAT COULD BE THE LESION ?

WHAT IS THE CAUSE

• 1-CONGENITAL• 2- TRAUMA• 3-VASCULAR• 4-INFLAMMATORY• 5-NEOPLASM• 6-METABOLIC & DEGENERATIVE

CASE2

• 13 YEAR OLD GIRL PRESENT WITH TENITUS VERTIGO AND DEAFNESS. O/E THERE IS LEFT SIDE LOWER MOTOR FACIAL NERVE PALSY AND LOSS OF CORNEAL REFLES.

• WHERE IS THE LESION? • WHAT IS THE CAUSE ?

COMMONCAUSES OF ACUTE STROKE SYNDROM IN CHILDREN

• The most common causes are congenital heart disease (cyanotic), sickle cell anemia (SS), meningitis, and hypercoagulable states.

• The cause of stroke in children is established in approximately 75% of cases

CASE

• 3 yeas old well being boy presented with fever, headache, confusion the mother has found him lieing in the front of the door with no trauma .He developed partial seizures with secondary generalisation and a right parieto-temporal syndrome consisting of left hemiparesis with hypoesthesia, left homonymous hemianopia, topographical isorientation and sensorineural deafness of acute onset .PH and FH unremarkable.anti vrus has given but with no improvment.Cerebrospinal fluid was normal. Brain Magnetic Resonance showed cortical and subcortical hyperintensities located unilaterally in the right parietal and temporo-occipital lobes and diffuse atrophy of the cerebellar cortex .

• BLOOD CHOLESTROL FOUND TO BE ELEVATED 275mg/dl

MELAS

• Mitochondrial Encephalopathy and Lactic Acid Strok like syndrome.

• Pathophysiology.• Eeg• Csf• A3243G levels • Treatment:

GENERAL CAUSES OF ACUTE STROKE SYNDROM IN CHILDREN

• GENERAL CAUSES:• Arterial thrombosis• Arterial embolism• Venous thrombosis• AVM• Vasculitis.

Arterial thromboembolism

• Sickle cell diseas• Cyanotic heart disease / mainly of MCA oxygen saturation is significantly decreased

together with a viral illness or dehydration-cardiac procesures• Trauma :Thrombosis of the internal carotid

artery.

• Hypercoagulablity syndrom.• Moyamoya• deficiencies in protein C, protein S, and

antithrombin III, as well as antiphospholipid syndrome.

• Cardiac causes: AF, DCM,Myxoma, IE,prosthetic valave and RHD.

• dissection• TTP• DIC• IBD

Venous thrombus

• Septic :encephalitis and bacterial meningitis

-Aseptic: severe dehydration in infancy, may cause

thrombosis of the superior sagittal sinus

hypercoagulopathy, cyanotic congenital heart diseases, and leukemic infiltrates of cerebral veins

vasculitis

• Arteritis: • Kawasaki arteritis• Homocystinurea• SLE• Wegner granulomatosis • juvenile RA• PAN • Behchets Dsease• Sjogren syndrom

Other causes

• Migrain vasospasm.• Focal cerebral arteriopathy of childhood (FCA) is the term

used by the International Pediatric Stroke Study (IPSS) group to describe an unexplained focal arterial stenosis in a child with CVA

• Arterial tortuosity syndrome • Fibromuscular dysplasia • Vasospasm resulting from subarachnoid hemorrhage

MET AND DEG

• CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is caused by a mutation in the Notch3 gene

• progressive degeneration of smooth muscle cells in the vessel wall

• may present with migraine, TIA, or ischemic stroke in late childhood or early adulthood

• Fabry disease, an X-linked lysosomal storage disorder due to deficiency of a-galactosidase A, may result in vessel narrowing and infarction in affected young adult males and carrier females

• Menkes' disease, a rare X-linked condition resulting in impaired copper transport, is associated with cerebral vessel tortuosity and stroke

-MELAS

DIFERENTIAL DIAGNOSIS

• TODDS PALSY• Alternating hemiplegia of childhood is

occasionally associated with migraine• encephalitis (particularly herpes)• demyelinating conditions such as acute

disseminated encephalomyelitis,

• A retropharyngeal abscess• idiopathic intracranial hypertension• drug toxicity• postinfectious cerebellitis• PSYCHOLOGICAL

INVESTIGATIONS FOR STROKE

• In children, head CT is generally considered inadequate to diagnose stroke.

• Brain MRI is more sensitive for acute ischemia than CT.it should be obtained ASAP.

• brain MRI provides better visualization of the posterior fossa.*

Current UK guidelines from the Royal College of Physicians (RCP) recommendation

• Magnetic resonance angiography (MRA) of the head to evaluate the intracranial large arteries. Computed tomography angiography (CTA) can be substituted

• MRA of the neck to evaluate the extracranial large arteries. CTA can be substituted.

• Axial T1 MRI of the neck to evaluate for dissection• Transcranial Doppler when MRA or CTA are

nondiagnostic and there is a high index of clinical suspicion for intracranial large artery disease

LABORATORY• Electrocardiogram • Complete blood count including platelets • Electrolytes, urea nitrogen, creatinine • Serum glucose • Prothrombin time (PT) and international normalized ratio (INR) • Partial thromboplastin time (PTT) • HB Electrophoresis.

• Cardiac enzymes and troponin if there is clinical suspicion of myocardial ischemia

• Ooxygen saturatio• Electroencephalogram if seizures are

suspected• Echo• Transesophageal echocardiography (TEE) if

TTE is nondiagnostic• Holter monitor if there is suspicion for cardiac

arrhythmia, particularly atrial fibrillation

• Liver function tests

• Toxicology screening

• Blood alcohol level

• Lumbar puncture, if there is clinical suspicion for subarachnoid hemorrhage and head CT scan is negative for blood, or if there is suspicion for an infectious etiology of stroke

• Hypercoagulable evaluation :• Protein C and protein S , antithrombin

III,lipoprotein , homocystin,anticardiolipin antibody,lupus anticoagulant tests.

• Vasculitis evaluation :• ESR,CRP,ANA,HIV, VDRL

MANAGMENT

• SUPPORTIVE:• Airway an respiration managment.• Circulation and bp managment.• Care of the skin• Care of nutrition• Care of the bladder • Care of the bowel• Physiotherapy

TREATMENT

• No randomized controlled trials of treatment in acute childhood stroke have been performed

• In general, treatment of pediatric stroke is largely adapted from treatment of adult stroke.

• Thrombolysis — Alteplase (rt-PA) is not approved for use in children less than 18 years of age with ischemic stroke

Initial antithrombotic

• there are no randomized controlled trials examining the effectiveness of antiplatelet or anticoagulation therapy for the treatment of acute arterial ischemic stroke in children

• The American Academy of Chest Physicians (ACCP) recommends either unfractionated heparin or low molecular weight heparin (LMWH) or aspirin as initial therapy until dissection and embolic causes have been excluded

• The American Heart Association Stroke Council guideline states that it may be reasonable to initiate anticoagulation with LMWH or unfractionated heparin in children with arterial ischemic stroke pending completion of the diagnostic evaluation

• UPTODATE RECOMENDATION:• suggest aspirin 3 to 5 mg/kg per day rather than

anticoagulation as initial therapy for most children with acute arterial ischemic stroke of unknown etiology

NICE GUIDELINES 2008for adults

SICKLER MANAGMENT

• For children with arterial ischemic stroke and sickle cell disease, UPTODATE suggest urgent intravenous hydration with intravenous normal saline rather than hypotonic saline .

• also recommend urgent exchange transfusion.• The goal of exchange transfusion is to achieve

a hemoglobin S fraction <30 percent of total

PROGNOSIS

• A study of national registry data from the United States reported that in-hospital mortality after ischemic stroke in children ages one to 17 years was 3.4 percent

• In young adults, mortality is approximately 4 to 6 percent in the first year after ischemic stroke

DISABILITY

• Despite the neural plasticity present in children, the majority of children with stroke have persistent disability

RECURRENCY• Recurrent cerebral ischemia, including stroke

and TIA, is common ranging from 6.6 to 20 percent

PREVENTION

• the American College of Chest Physician (ACCP) guideline for antithrombotic therapy in children recommends daily aspirin (1 to 5 mg/kg daily) for a minimum of two years

• NO GUIDELINE supprt use of adding with asprin clopedogril.

• limited data suggest that combined treatment with aspirin and clopidogrel is associated with an increased risk of intracranial bleedin

• For stroke secondary to a cardioembolic cause, the ACCP guideline recommends anticoagulant therapy with LMWH or warfarin for at least six weeks, with ongoing treatment dependent upon radiologic assessment but asprin not recommended.

• For children with ischemic stroke due to arterial dissection, uptodate suggest anticoagulation with warfarin or low molecular weight heparin for three to six months after stroke onset, followed by long-term therapy with aspirin

• For children with ischemic-type moyamoya: surgical revascularization at a center with expertise in the surgical treatment of moyamoya

• For children with sickle cell disease, uptodate suggest chronic transfusion therapy to maintain hemoglobin S less than 30 percent of total hemoglobin

• For children with stroke related to vasculitis, treatment of the underlying condition

Recurrent ischemia despite aspirin

• If in aspirin therapy: changing therapy to either clopidogrel or

anticoagulation (with low molecular weight heparin or warfarin)

STROK IN NEONATES

Stroke is more common in the newborn period than at any other time in childhood and carries the risk of significant long-term neurodevelopmental morbidity.

acutely in the neonatal period

later when the child develops a hemiparesis or symptomatic epilepsy syndrome

CAUSES

• congenital heart disease• placental pathology • Thrombophilia

INVESTIGATION OF CHOICE MRI

Wayne State University, School of Medicine, Detroit, MI, USA

RECENT RESEARCHES

• Stem cell therapy for stroke• In the past 10 years there has been an explosion of research

interest in how a variety of stem cell populations respond in animal models of stroke

• IDEA/ stem cells may improve aspects of cellular and functional recovery following largely ischemic models of stroke

• TRIAL STILL ONGOING

(Journal of Pediatric Neurology 2010(

Role of Chlamydia pneumoniae in pediatric acute ischemic stroke

• Several studies have shown that Chlamydia pneumoniae accelerates atherothrombosis by cytokine-mediated process with increased risk of cerebral ischemia in adults.

• ONE study has proved that in children.

• Journal of Pediatric Neurology 2010

QUIZ1

• A dilated and unreactive pupil indicates the compression of what structure?

Quiz 2

• Pinpoint pupils and respiratory changes indicate the compression of what structure?

Quiz 3

• How does the presentation of stroke differ between infants and older children?

• Infants usually have a seizure, whereas older children have acute hemiplegia.

Calder K, Kokorowski P, Tran T, Henderson S: Emergency department presentation of stroke. Pediatr Emerg Care 19:320-328, 2003

Quiz 4

• A child who develops weakness, incontinence, and ataxia 10 days after a bout of influenza likely has what diagnosis?

Acute disseminated encephalomyelitis

• Any portion of the white matter • small foci of perivenular inflammationand demyelination• mumps, measles, rubella, varicella-zoster, influenza,

parainfluenza, mononucleosis, and immunization• CSF examination shows mild increase of pressure

and up to 250 cells/mm3, with a lymphocyte predominance

• steroids

CT scan V/S MRI

• CT scan without contrast • head trauma for skull fractures• acute strokes • subarachnoid hemorrhages• ventricular shifts caused by masses • edema or increased ICP

CT scan with contrast

• better identification of disruptions in the blood-brain barrier or of highly vascular structures

• tumors, edema, focal inflammation, hemangiomas, and arteriovenous malformations.

MRI

• image in three dimensions• subacute and chronic HG• tumors or masses

• MRI with contrast is helpful for defining brain metastases

• Magnetic resonance angiography arterial stenosis ,hemangiomas, arteriovenous

malformations, and vascular aneurysms

THANK YOU

• References• Uptodate • Nelson• NICE guidelines for strok managment • Prof Farook Yaseen notes• Said Elwan neurology• E-medicine web site• Pediatric journal of neurology• International journal of neurology• Netter atlas of neuroanatomy• Pediatric secrets.• Dr.Mamdooh mahfoooz lectures