supervised by: dr.amani done by : eman al-otibi darin al –radadi dina al-amam ebtehal al-yami

Supervised by: Dr.amaniDone by :Eman Al-otibiDarin Al –radadiDina Al-amamEbtehal Al-yami

An 18 m. old baby is admitted to the hospital B/C he is suffering from fever and convulsion.In the P.H : he was doing well until 4 days of his admission when he had fever, running nose & dry cough. His mother brought him to PHC where the family physician diagnosed him as acute URTI and he prescribed antibiotic, cough syrup and paracetamole. But the fever didn’n subside & in the 4th day he developed convulsion.F.H : his brother developed fever & convulsion when he was 3 y old.

DEFINITIONS

Seizures: Are transient disturbances in brain function

manifesting as episodic impairments in consciousness in association with abnormal autonomic activity.

Epilepsy:

Is a group of neurologic conditions , the common and fundamental characteristic of

which is the liability to recurrent, usually

unprovoked epileptic seizures.NB:

A persone with a single or recurrent seizures due to correctable circumstances dose not necessarily have epilepsy

ETIOLOGYEpilepsy:

Idiopathic: 70-80% Secondary:

Cerebral malformation: hydrocephalus Cerebral damage: congenital infection, asphyxia,

intraventricular hge/ischemia Cerebral tumor.

Degenerative disorders: Alzheimer’s disease Neurocutaneous disorders: tuberous sclerosis

Non-epileptic: Febrile convulsions

Metabolic: Hypoglycemia

Hypocalcaemia/hypomagnesemia Hypo/hypernatraemia

Inborn error of metabolism Head trauma

Meningitis/encephalitis Poisons/toxins: chemicals, cocaine,

isonized, antidepressant

Metabolic

Phenylketonuria

Biotinidase deficiency

Maple syrup urine disease

Isovalericacidemia

Ornithine accumulation

Nonketotic hyperglycinemia

Pyridoxine dependency

Hypoglycemia

Lipidosis

Developmental malformations

Polymicrogyria

Lissencephaly

Schizencephaly

Down syndrome and other chromosomal disorders

Aicardi syndrome

Organoid nevus syndrome

Neurocutaneous syndromes

Tuberous sclerosis

Sturge-Weber syndrome

Congenital infections

Toxoplasmosis

Cytomegalovirus

Syphilis

Encephalopathies

Post-asphyxia

Post-traumatic

Posthemorrhagic

Postinfectious

CAUSES OF PEDIATRIC SEIZURES

Infants (0-1yr)CNS infectionMalformation

Drug withdrawal or toxicity

GeneticMetabolic

Hypoxia

Young Children (1-12)

CNS infection Degenerative disorder

Fever Genetic Trauma

Idiopathic

RISK FACTOR•History of seizure.

•Family history.•Fever.

•Stress .•Lack of sleep.

•Missed meal•Flashing of light .

Classification of Seizures and Epileptic SyndromesPartial Seizures

Simple partial (consciousness not impaired)

Complex partial (consciousness impaired)

Secondarily generalized seizures

Generalized Seizures

Absence

  Typical

  Atypical

Tonic

Clonic

Tonic-clonic

Minor motor

  Atonic

  Myoclonic

Epileptic Syndromes

Benign focal epilepsy (benign rolandic epilepsy, benign centrotemporal epilepsy)

Juvenile myoclonic epilepsy

Infantile spasms (West syndrome)

Lennox-Gastaut syndrome

Acquired epileptic aphasia (Landau-Kleffner syndrome)

Benign neonatal convulsions

Types of seizures :

SEIZURE OUTCOMESInjury/Death15%

Head contusions/Lacerations (Common)Mortality

1.2% of all seizures3% to 26% in Status Epilepticus

10X higher in adults (Vs..... Children)Highest with hypoxic or ischemic.

TYPES OF PEDIATRIC SEIZURES

“Generalized” Seizure Partial Seizure

PEDIATRIC SEIZURES ARE DIFFERENT

Immature nervous systemCannot sustain organized seizuresPoorly developed connections

Less capable of repetitive high-frequency firing

MECHANISM OF SEIZURENormally functioning

cortex>>have recurrent &collaterals inhibitory circuits (GABA)

Also we have large number of excitatory neurotransmitters like ACH,aspartate and glutamate normally there is balance between this circuits.

case of seizure >>cerebral cortex hypersyncrous repetitive discharge involving large group of

neurons,, Intracellular :rapid action

potential firing with reduction of transmembrane potential so

(excessive excitation &reduction inhibitory system)

Repetitive discharge lead to morphological &physiological change which produce abnormal discharge

ONE OF THE MECHANISMmechanism for some forms of inherited

epilepsy are mutations of the genes that code for sodium channel proteins; these defective sodium channels stay open for too long, thus making the neuron hyper-excitable. Glutamate, an excitatory neurotransmitter

Another possible mechanism involves mutations leading to ineffective GABA

Partial SeizuresStart by activation of group of neuron in one part of one hemisphereThree main types

)1Simple partial Seizures. motor, sensory, behavioral, or autonomic)

)2Complex partial seizures.

)3Partial Seizures evolving to generalized. 2nd generalized 

*pause activity

*confusion•Temporary

memory loss

•*change in autonomic behavior

SIMPLE PARTIAL SEIZURES:

Consciousness is preserved Simple partial seizures arise from a

specific anatomic focus. Clinical symptoms include

.1Motor : Location and direction of spread of the seizure focus determine the clinical symptoms.

.2 Sensory : tingling or parasthesia on the face or lomp , visual , aoditory, olfactory disturbance.

.3Psychic ( behavioral ) : DEJA, JAMIAS..4Autonomic abnormalities : sweating or

flusing.

Complex partial consciousness is impaired.

Commonly origenated from temporal lope.It is associated with alterned consiousness without the pt. collapsed to the ground . The pt. stop what he is doing & stares blinkly, often macking rhythmic smacking movment of lips

or pickling at their cloth .

. Partial Seizures evolving to generalizedWhen partial seizures spread to involve the

whole brain and produce a generalized tonic-clonic seizure, they show secondary generalization ("jacksonian" seizures).

Generalized seizer

GENERALIZED SEIZER: It starts by activation of

neurones in large area of both hemisphere.

GENERALIZED SEIZER: It consists of:

1-Generalized--Tonic

--Clonic,- and Tonic-Clonic Seizures (grandmal).

2-Absence Seizures ( petit mal ).3-Myoclonic.

4-Atonic seizure

TONIC-CLONIC SEIZURES (GRANDMAL)

Phases of TCS:Prodromal phase : houres or days before

attack. Aura : specific feeling or occurance of seizure.

e.g olfactory hallusination, epi. Discomfort .

Consciousness and control of posture are lost followed by:Tonic phase :Rapid discharg of motor cortix cell causing tonic contraction of muscle ( stiffness )children do not breath and become cyanosed Upward deviation of the eyes.Pooling of secretion , pupillary dilation ,diaphoresis, hypertension , and

piloerection . Clonic phase :

Less rapid, gradually & slowing of discharge of cortical cells causes alternating contraction and relaxation ( Jerking of limbs). Irregular breathing & cyanosis persists, saliva may accumulate in mouth, tongue bitting and incontinence . Last for 2 – 3 min.

Post-ictal phase :Deep unconsciousness .Flaccid limb & jaw.Loss of corneal reflex.Headache, confusion & malaise.Todd paralysis .Amnesia.

EEG During attack shows :repetitive synchronous bursts of spike activity followed by periodic paraxysmal discharges.

ABSENCE SEIZURES 2 types :Typical absence seizureAtypical absence seizure :

Typical absence seizure :*(Disorder of childhood (Usually begin

between 4 and 6 y Characterized : Brief loss of enviromental awareness

accompanied by eye fluttering or simple automatisms ,such as head bobbing and lip smacking .

The attack lasts for a few second (15-30 sec)

Induced by hyperventilation . TYPICAL EEG >>>> 3 hz. SPIK AND

WIEVES .

MYOCLONIC SEIZURES: It consist of single or multiple myoclonic jerk

involving one part of the body or entire.Not proceded by aura.

Atonic Seizures

-Sudden loss of postural tone, with sagging of the head or falling.

-Rarely loose consciousness.

EPILEPTIC SYNDROMES

Epilepsy : Is a group of neurologic conditions, the common and fundamental characteristic of which is : the liability to recurrent , unprovoked seizures.

EPILEPTIC SYNDROMES

1-Benign focal epilepsy (benign rolandic epilepsy, benign centrotemporal epilepsy)

2-Juvenile myoclonic epilepsy3-Infantile spasms (West syndrome)

4-Lennox-Gastaut syndrome5-Acquired epileptic aphasia (Landau-

Kleffner syndrome)6-Benign neonatal convulsions

Benign focal epilepsy, also known as rolandic epilepsy

-usually begins between ages 5 and 10 years.In the central sulcus.Good prognosis.They are usually focal motor seizures involving the face and arm and tend to occur only during sleep or on awakening in more than half of patients .

Symptoms commonly include abnormal movement or sensation around the face and mouth with drooling and a rhythmic guttural sound .Speech and swallowing are impaired .

A family history of similar seizures is found in 13% of patients.

The disorder is called benign because 1 -seizures usually respond promptly to

anticonvulsant therapy;2 -intellectual outcome and brain imaging

are normal, and epilepsy resolves after puberty. Continued treatment is not needed.EEG : centrotemporal spike

INFANTILE SPASMS (WEST SYNDROME)

INFANTILE SPASMS (WEST SYNDROME)

are brief contractions of the neck, trunk, and arm muscles, followed by a phase of sustained musclecontraction lasting 2 to 10 seconds 3 patterns :

1. Extensor : extension of trunk and extrimities . least common .

2. Flexor : flexion of neck , arm and leg onto the trunk .

3. Mixed : most common . May occur during sleep or waking .

Classified in to : 1-Cryptogenic : unknown cause .

-10-20% . -Good prognosis .

2-Symptomatic : -related to prenatal or perinatal or pos natal causes .-Prognosis 80_90 % mantal retardation , but the nature of

the disease determine the outcomes. Tuberous sclerosis is the most common recognized

cause.

The EEG during the waking state,

hypsarrhythmia ,Treatment of infantile spasms includes adrenocorticotropic hormone, oral corticosteroids ,benzodiazepines ,

and valproic acid

JUVENILE MYOCLONIC EPILEPSY

occurs in adolescence and is an autosomal dominant disorder The patient may have absence, generalized tonic or clonic, and myoclonic seizures .

The hallmark is morning myoclonus occurring predominantly within 90 minutes of awakening .

Seizures usually resolve promptly with therapy with valproic acid, but therapy must be maintained for life .

Benign neonatal convultions Outosomal dominant Generalized clonic seizures occur toward the end of first week of life.

Lennox gastaut syndrome multiple seizure types Poorly response to treatment

Acqured epliptic aphasia (landau-kleffner syndrome )Characterized by the abrupt loss of previously acqured language in young childrin

SummaryseizureAbsent seizure

Characterized :

Brief loss of enviromental awareness accompanied by eye fluttering or simple automatisms ,such as head bobbing and lip smacking .

Induced by hyperventilation. TYPICAL EEG >>>> 3 hz. SPIK AND WIEVES .

Epileptic Syndromesrolandic epilepsy

EEG : centrotemporal spike

Infantile spasms (West syndrome)

are brief contractions of the neck, trunk, and arm muscles, followed by a phase of sustained muscle

The EEG during the waking state,hypsarrhythmia ,

FEBRILE CONVULSIONS Seizures associated with sudden onset of high fever in

absence of other causes of seizures & not due to intracranial infections )should be extra cranial infection).

Viral infection of the URT , roseola infantum , shigellosis (most important 2 infections)&acute otitis media are most frequently the causes of Febrile convulsion

Affect 4-6% of children. Commonest seizure in childhood. Genetic predisposition. Strong family history “1st degree relative” Gastroenteritis may cause febrile convulsion especially with

campylobacter Giovanni

TYPES Typical (simple): Occur in 1st 24 h of fever. Age group (6 ms – 6 ys) More than half occur between ages 1

and 2 years (mean age 22 months). Single. No neurological or developmental

abnormality. Negative family history of epilepsy. Generalized tonic clonic. Persist < 15 min.

CONT.…Atypical (complex): Occur after 1st 24 h of fever. Age group (<6 ms & >6 ys) Multiple Focal convulsion . Persist > 15 min. Repeated convulsion for several hours or days

in the febrile illness. Neurodevelopmental abnormality may be

present Positive family history of epilepsy may be

present 4-10% of these cases may develop epilepsy

later .

INVESTIGATION Lumbar puncture should be done at

first time of diagnosis. Glucose level CBC EEG Urine analysis

TREATMENT ABC Left lateral position O2 i.v or rectal diazepam Antipyretic & cold compressor Search for the cause and treat it . We should exclude meningitis and encephalitis before treatment. But in general no need treatment. Prophylaxis: During attack of fever give oral or rectal diazepam

3 times daily.Advise family to keep paracetamol at home.

Phenobarbital not given b\c lead to ADHD except in neonatal convulsion.

PROGNOSIS Good prognosis. Risk of recurrence:

50% first febrile seizure at younger than 1 year of age .

30% first seizure at older than 1 year of age 10% have three or more recurrence . 7% with complex having complicated

febrile seizure.Intellectual achievements are normal.

Simple febrile seizures do not cause brain damage.

Risk of epilepsy development:is 2% if one risk factor present,Presence of more than one risk increase to 10%:

1. +ve family history2. Atypical febrile convulsion3. Neurological abnormalities4. Developmental

abnormalities

DIFFERENTIAL DIAGNOSIS Encephalitis & meningoencephalitis Metabolic:

• Hypoglycemia• Hypocalcaemia/ hypomagnesaemia• Hypo/ hypernatraemia

C.V.A. Toxic: e.g. drugs/ aminophilline Neoplastic: e.g. neuroblastoma

NEONATAL SEIZURESNeonate are at particular risk for the develoment of seizure.

-different from child or adult in that generalized tonic-clonic convulsion not occur in first month of life.

CONT..…Neonatal seizures may be difficult to

recognize clinically . There are seizures and none epileptic

activitiesAutonomic changes (tachycardia,increase

BP ) in seizure .but not in non epileptic.None epileptic movements are

suppressed by gentle restraint and enhanced by sensory stimuli . But not in seizure.

Also in seizure coarse, fast slow clonic movement.but in non epileptic there fine rapid movement.

Clinical characteristics of neonatal seizures

Characteristics Designation

Repetitive,rhythmic contractions of muscle groups of the limbs,face,trunk

1-Focal clonic

2-multifocal clonic

Rigid Posturing of single limbs,asymmetric posturing of the trunk ,sustained eye deviation, cannot provoked by stimulation

3-Focal tonic

Not repetitive or recur at a slow rate , may generalized or focal, may provoked by stimulation

4-Myoclonic

Symmetric posturing of limbs,trunk May flexor, extensor or both and provoked by stimulation

5-Generalized tonic

6-Subtle seizure-:1-Ocular signs:

Random and roving eye movement or nystagmus

2-Orobuccolingual movements:Sucking, chewing, tongue protrusions,excesive salivation

3-Progression movement:Swimming movements of the arms, bicycling or pedaling movements of the legs.Also , there alteration in the respiratory rate including apnea. And change in color.

EEG CLASSIFICATION OF NEONATAL SEIZURESA-Clinical seizures with a consistent

EEG event.clinical seizure occures in relashionship to

seizure activity recorded on EEG (focal clonic , focal tonic, some myoclonic) respond to anticonvulsants

B-Clinical seizures with inconsistent EEG event.

(tonic , subtle , some myoclonic)C-Electrical seizures with absent

clinical seizure.comatosed pts who are not on anticonvulsants

ETIOLOGY OF NEONATAL SEIZURESAge 1-4 days:

-hypoxic-ischemic encephalopathy -drug withdrawal,maternal drug use of

narcotics or barbiturates.-drug toxicity,lidocaine,penicilline.

-intraventricular heamorrhage.-metabolic disorders:

1-hypocalcemia:* perinatal asphyxia *maternal

diapetes* sepsis

*hypo/hyperthyroidism.

2-hypoglycemia3-hypomagnesemia

4-hypo/hypernatremia:*inappropriate antidiuretic hormone

secretion. -inborn error of metabolism

-pyridoxine deficiency.

Age 4-14 days:-infections:

*meningitis, encephalitis-metabolic disorders:

1-hypocalcemia:*diet , milk formula.

2-hypoglycemia:*anterior pituitary hypoplasia

*pancreatic islet cell tumors.

-drug withdrawal-benign neonatal convulsion

-kernicterus , hyperbilirubenemia.

Age 2-8 weeks:-infections:

*hrpes simplex, encephalitis.-head injury:

*subdural haemorrhage , child abuse.-inherited disorder of metabolism.

-malformation of cortical development.*focal cortical dysplasia.

-tuberous sclerosis.-sturge – weber syndrome.

BENIGN FAMILIAL NEONATAL SIEZURE

-Autosomal dominant , begin on 2nd -3rd day of life with a seizure frequency of 10-20/day.Patients are normal between seizures.Which stop in 1-6 months .

Fifth day fits occur (4-6) days in small appearing neonatesSizure are multifocal. Present for less than 24 hours. Prognosis is good.

DIAGNOSTIC EVALUATIONCareful neorologic examinationsFamily history of IEMExamination of retina.(chorioretinitis

seuggsets TORCH ).Inspection of skin (hypopigmented lesions

:tuberus sclerosis)An unusual body odor :inborn error of

metabolism.

Blood ..lower serum Ca associated +birth trauma or a CNS insult in the perinatal period

hypomagmesemia+Hypocalcemia occur in infants of malnourished mothers ( Im magnesium)

Serum ammonia : urea cycle abnormalities (orinthine transcarbamylase ) present with increasing lethargy progression to coma,vomiting.

CSF : infections , blood.

TREATMENT The treatment is specific such as treatment of meningitis or the correction of hypoglycemia,hypocalcemia, hypomagnesemia.Therapy should involve anticonvulsant agent:

Phenobarbital 20to40 mg/kg or 10 to 20mg/kg phynetoin or diazepam.

STATUS EPILEPTICS Neurological emergency which defined as

ongoing seizure activity for greater than 20 min or repetitive seizures without return of consciousness for greater than 30 min.

50% of this group ,status is triggered by fever. 25% have acute brain injury.(meningitis m

encepelitis ,electrolyte disorder). Risk factors:1. Abrupt withdrawl. )Sudden cassation of anti-

convulsant medication.)

2. cerebral haemorrhage.3. CNS inf. Or tumor.

INVESTIGATIONSBlood glucose.Blood calcium and magnesium.CBC.Electrolytes.ABG.CSF analysis.Blood and CSF cultures.EEG: not diagnostic, and it is normal in about

one-third of cases .Transfontanellar ultrasound for hemorrhage.CT scan: to diagnose cerebral malformations and

hemorrhage.

TREATMENT Goal : stop seizures as soon as possible.At onset : ABC & O21: IV line with NS.2: Lorazepam )best chose) ,)0.1 mg/kg ) or diazepam ) 0.2

mg/kg ) over 2 minutes via second IV line.3: Phenytoin 20 mg/kg IV.

4:EEG monitoring unless status ended and patient waking up.

5: Phenobarbital 20mg/kg.

If persist : artificial ventilation. Muscle relaxant.

EVALUATION full Carful History:

Take care about:(onset ,duration ,head injury,

previous attack, medication , neck stiffness , fever , family history , history of neurologic or developmental disorder) Physical examination:

Exclude any suspected cause , vital sign , meningitis test, neurologic & mental status

evaluation

Laboratory investigation : CBC , full blood chemistry , blood or

urine toxicology screening , analysis of CSF , blood ammonia , urine and stool culture

imaging MRI superior to CT in showing brain

pathology, but in emergency department setting ,CT may be desirable because it can be performed rapidly and shows acute intracranial hemorrhage more clearly than MRI

EEG

There is no special examination or tests to diagnose & evaluate seizure & epilepsy

The only way is by exclusion & interpretation of history , physical examination , imaging , laboratory tests , EEG

MANAGEMENT

Education .

Avoid RF.

Correct the underlying cause.

If there is no clear cause for the first seizure, wait before starting anticonvulsant.

Single drug therapy (minimum dose) is used where possible.

Sometimes, two or more anticonvulsants are required.

Most commonly used anticonvulsants: valproate, carbamazepine, lamotrigine.

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CHOICE OF ANTIEPILEPTIC 1Seizure type Drug of choice Alternatives

Partial simple &Partial complex

CarbamazepinePhenytoinValproate

LamotrigineGabapentinLevetiracetamTopiramateTiagabineOxcarbazepinePhenobarbital

81

CHOICE OF ANTIEPILEPTIC 2Seizure type Drug of choice Alternatives

Generalised tonic clonic

CarbamazepinePhenytoinValproate

LamotrigineTopiramatePhenobarbital

Absence EthosuximideValproate

LamotrigineClonazepam

Atypical absenceAtonic, myoclonic

Valproate Clonazepam

Some side-effects of anticonvulsantsSide effects Drug

Hepatotoxicity nausea & vomiting increased appetite and weight Transient hair loss.

Valproate

Liver dysfunction, Lupus erythematosus syndrome Dizziness, visual disturbance.

Carbamazepine

Rash, behavior disturbance, irritability.N & V Lamotrigine

Nausea and vomiting & abdominal dyscomfort Ethosuximide

drowsiness ,easy bruising or bleeding,irritability, confusion ,hyperactivity

Phenobarbital

Hirsutism, gum hypertrophy, ataxia, Steven.j.S headache, dizziness, nervousness, or sleep problems (insomnia), slurred speech

Phenytoin

Condition mimic seizure:1-breath holding attack2-benign paroxysmal vertigo3- Night terrors4- Syncope 5-peudosizure 6- prolonged QT interval 7- narcolepsy and cataplexy

BREATH HOLDING SPELLS

Occur in some toddler when they are upset . The child cries ,holds his breath and goes blue ,sometimes children briefly loss consciousness but rapidly recover fully.

- Cyanotic spells: predictable and provoked by upsetting and scolding. rare before 6 months and abate by 5 yrs

- Heralded by brief shrill cry followed by forced expiration and apnea ,followed by loss of consciousness & may be a.e repeated generalized clonic jerks, episotonous .

- Pallid spells :painful experiences such as falling & striking the head.the child stops breathing, loses consciousness, become hypotonic may have atonic seizure .in refractory cases oral atropine

● Drug therapy….. is unhelpful

● Attacks ….resolve spontaneously

BENIGN PAROXYSMAL VERTIGO

ToddlersSudden + ataxiaHorizontal nystygmus Consciousness not disturbedRotational sensation

NIGHT TERRORS

5- 7 ysMidnight and 2 A.MDilated pupil ,

tachycardia ,Tachypnea ,sweating ,hyperventilation

1/3 +somnambulism ( walking at night )Diazepam (short course)

PSUDOSEIZURES 10-18 yrs ,girls Past Hx of epilepsy Seizures are bizarre, no loss of

sphinctors A neurotic personality

SUMMARY

REFERENCE Nelson essential of pediatrics Nelson text book of pediatrics

Illustrated text book of pediatrics