EPILEPSY IN CHILDREN

WHAT IS EPILEPSY?

Epilepsy is a chronic neurological condition characterized by recurrent, unprovoked seizures, occurrence of at least 2 unprovoked seizures 24 hours apart.

Epileptic seizure → clinical manifestation of abnormal & excessive discharge of a set of neurons in the brain

CAUSES Idiopathic (70 – 80%) – cause unknown but

presumed genetic Secondary Cerebral dysgenesis /malformation Cerebral vascular occlusion Cerebral damage

- Antenatal: Congenital infections, drugs, alcohol

- Natal: HIE, birth trauma- Postnatal: IVH in prematurity, CNS infections, kernicterus, trauma, tumour

Cerebral tumours Neurodegenerative disorders Neurocutaneous syndrome

OLD CLASSIFICATION

GENERALISED SEIZURES:-(Discharge arises from both hemisphere)

Absence seizures Myoclonic seizure Tonic Tonic clonic Atonic seizures

FOCAL – SEIZURES ARISE FROM ONE

OR PART OF ONE HEMISPHERE

Frontal seizuresTemporal lobe seizuresOccipital seizuresParietal lobe seizures

ILAE 2010 CLASSIFICATION

GENERALIZED SEIZURES

CLASSIFICATION

TONIC CLONIC SEIZURESTonic phase The tonic phase begins with flexion of the trunk

and elevation and abduction of the elbows. Subsequent extension of the back and neck is followed by extension of arms and legs.

Piercing cry may be present due to passage of air through closed vocal cords.

Autonomic signs are common during this phase and include increase in pulse rate and blood pressure, profuse sweating

This stage lasts for 10-20 seconds.

Clonic phase tremor occurs at a rate of 8 tremors per

second, which may slow down to about 4 tremors per second. This is because phases of atonia alternate with repeated violent flexor spasms. Each spasm is accompanied by pupillary contraction and dilation. Some patients may have tongue or cheek bites.

The atonic period lasts about 30 sec.

The clonic phase lasts for 30 sec. to 1minute.

TONIC CLONIC SEIZURES

ABSENCE SEIZURES

Patient stares briefly and stop talking or ceases to respond.

Most of the patient are completely motionless while some feel some myoclonic movements in eye lids,facial muscles,fingers at a rate of 3 per sec..and this rate corresponds to the abnormality in EEG as generalized 3 per sec.spike & wave pattern.

Occurs at the age of 4-12 years Prognosis is good.95% remission in

adolescense

MYOCLONIC SEIZURES

These are brisque,brief muscular contractions some of them involve only single muscle or a part

of the muscle & some of them are so large that they include whole body or both the limbs.

Myoclonic jerks are common in the morning involving entire body both the limbs and sometimes absence seizures are common.

This is the most common form of idiopathic gen.epilepsy in childhood.it begins at adolescence (15 yr).

4 to 6 Hz irregular spikes have been noted in EEG.

PARTIAL SEIZURES ---FOCAL SEIZURES

PARTIAL SEIZURE - FOCAL SEIZURE

Begin in a relatively small group of dysfunctional neurones in one of the cerebral hemispheres.

Mayb e heralded by an aura which reflects the site of the origin

May or may not be associated with change in consciousness or more generalised motor jerking.

Frontal seizures

•Involve the motor cortex•May lead to clonic movements → travel proximally→ (Jacksonian March)•Asymmetrical tonic seizures → bizarre ,hyperkinetic & easily dismissed as non – epileptic events

Temporal lobe seizure•Most common •Strange warning feeling/ aura with smell , taste abnormalities & distortion of sound & shape •Automatism → spread to the premotor cortex•Deja-vu & Jamais-vu•Consiousness may be impaired, length of event is longer than a typical absence

Occipital

• Distortion of vision

Parietal

• Causes contralateral dysaesthesias (altered sensation )

• Distorted body image

SPECIAL EPILEPSY SYNDROME

1.INFANTILE SPASM:-

-Most cases appears in 1st yr of life. - Single brief recurrent gross flexion

movements of the limb …rarely extension movements

-EEG shows multifocal,multiple small spikes. -On maturity it disappears(4 to 5yr) -CT & MRI mostly normal. -Later progress to LENOX GASTAUT SYND.

HISTORY TAKING

HOPI:- Two unprovoked seizures >24 hr apart suggest the presence of

an epileptic disorder Any aura?change in the behaviour? Types of seizures -tonic clonic(tensing,then shaking,LOC) -atonic(drop attack) -absence(jus staring,not responding,blinking) -partial(maybe consciouss,only ½ limbs shaking/jerking How long did it last?frequency?time of day?precipatating factor? Any loss of consciousness?tongue bitting? What did you do for the child?(appropriate first aid measures) Post ictal:drowsy?sleeping?vomiting?

PERINATAL HISTORY Infection during pregnancy?TORCH Birth history - birth asphyxia , birth trauma Neonatal jaundice

POST NATAL HISTORY

Central nervous system (CNS) infection e.g. meningitis, encephalitis etc.

Head injury Lead contact (lead fumes from burning batteries, pica)

PAST HISTORY Age at 1st seizure?describe seizure? h/o febrile seizures When and how diagnosed?any event preceding

seizure?

DRUG HISTORY1. Anticonvulsant medications2. How many?3. Any increase in dosage?types?4. Compliance,how often dose missed?what to do if

missed?5. Side effect?6. Responding current medication or not

Outpatient review1. Frequency2. Test done3. Other investigations(eg.EEG to date)

Hospitalization 1. How many?reasons? Any identified medical problems associated with

seizures?1. Any history of trauma,meningitis?encephalitis?2. How are this problem managed? FAMILY HISTORY1. Of convulsion?inborn error metabolism?

SOCIAL HISTORY:- Impact on child:1. Schooling2. Athletic participation?3. Self esteem4. Does teacher know about the condition?5. On family:financial burden

CONTINGENCY PLAN:1. What to do in the event of a seizure?

PHYSICAL EXAMINATION

•Consciouss level•Posture•Deformity•movement•Dysmorphism,head size and shape•Cranial nerves•Gait•Spine

•Neurocutaneous signs1. café-au-lait spots2. Neurofibromata3. adenoma sebaceum

MANAGEMENT AND TREATMENT

DIAGNOSIS

The clinical diagnosis is more important then any tool… (H/O, Eye witnesses, substantiated by video if available)

# EEG:- EEG is most sensitive tool for diagnosis which

shows electrical activity changes in the brain but it also require clinical correlation

Many children with epilepsy may have normal EEG and many children who will never have epilepsy have EEG abnormalities

Done for dx, classification, selection of anti-epileptic drugs and prognosis

FOCAL DISCHARGES

GENERALIZED DISCHARGES

MRI and CT-not required routinely for childhood generalized

epilepsy. To identify a tumour,vascular lesion or area of

sclerosis.

PET and SPECT. To detect areas of hypometabolism in epileptogenic

lesions OTHER INVESTIGATIONS Blood test and metabolic investigations(seizures

related to feed and fasting). Genetic studies Lumbar puncture

PRINCIPLES OF ANTICONVULSANT THERAPY Treatment recommended if ≥ 2 episodes→ recurrence risk

80% Attempt to classify the seizure type & epileptic syndrome

Monotherapy as far as possible → most appropriate drug → increase dose gradually till epilepsy controlled, maximum dose reached / side effects occur

Alternative monotherapy (Add on the 2nd drug if 1st drug failed. Optimise 2nd drug, then try to withdraw 1st drug.

Rational combination therapy (usually 2 or maximum 3 drugs )

Combines drugs with different mechanism of action & consider their spectrum of efficacy, drug interactions & adverse affects.

Monitor drug levels (carbamazepine, phenytoin, phenobarbitone) to check compliance → if seizures not well controlled/in situations of polypharmacy where drug interaction is suspected.

When withdrawal of medication is planned → seizure free for 2 years, consideration should be given to epilepsy syndrome, likely prognosis & individual circumstances before attempting slow withdrawal of medication over 3-6 months (longer if clonazepam/ phenobarbitone)

If seizures recur → last dose reduction is reversed & medical advice sought

INTRACTABLE EPILEPSY?

Re- evaluate the following possibilities Is it a seizure /non epileptic event Anticonvulsant dose not optimized Poor compliance to anticonvulsant Wrong classification of epilepsy syndrome →

wrong anticonvulsant Anticonvulsant aggravating seizures Lesional epilepsy, hence a potential epilepsy

surgery candidate Progressive epilepsy or neurodegenerative

disorder

REFERRAL TO PAEDIATRIC NEUROLOGIST

Poor seizure control despite monotherapy with 2 different anticonvulsants

Difficult to control seizures beginning in the 1st year of life

Seizures & developmental regression Structural lesion on neuroimaging

ADVICE FOR PATIENTS

Educate and counsel on epilepsy. Emphasize compliance if on anticonvulsant. Don’t stop the medication by themselves.this

may precipitate breakthrough seizures. In photosensitive seizures-watch tv in

brightly lit room.avoid sleep deprivation. Use a shower with bathroom door unlocked No cycling in traffic,climbing sports or

swimming alone. Know emergency treatment for seizure Inform teachers and school abt the condition.

PARTIAL SEIZURES

Simple partialComplex partialSec.generalised

FIRST LINE

carbamazepine valproate

SECOND LINE

lamotrigine topiramate levetiracetam phenytoinPhenobarbitameclonazepam

GENERALISED SEIZURES tonic clonicClonic

AbsenceAtpical absenceAtonic,clonicMyoclonic

Infantile spasms

valproate

valproate valproate

Valproate,clonazepam

ACTH,prednisolone

lamotrigine topiramate levetiracetam phenytoin Phenobarbitame Clonazepam

LamotrigineTopiramate,clonazepamTopiramate,phenobarbitone,piracetam,levetiracetam,lamotrigine.Nitrazepam,valproate

SIDE EFFECTS AND SERIOUS TOXICITIES OF ANTICONVULSANT CARBAMAZEPINE—

drowsiness,dizziness,ataxia,diplopia,rash (serious toxicity—agranulocytosis Steven Johnson syndrome)

CLONAZEPAM---- hypotonia,salivary and bronchiol

hypersecretion,paradoxical hyperactivity,aggresiveness

PHENYTOIN--- ataxia,diplopia,rash,gum hypertrophy,hirsutism (serious toxicity—megaloblastic anemia)

PHENOBARBITONE---- cognitive dysfunction,ataxia,rash,behavioural

disturbance serious toxicity—liver toxicity,steven johnson syndrome

VALPROATE---- epigastric pain,tremor,alopecia,weight gain,hair

loss,thrombocytopenia serious toxicity—hepatic toxicity(<2 yrs age) hepatitis,pancreatitis, encephalopathy

STATUS EPILECTUS

Any seizures lasting > 30 minutes OR

Intermittent seizures longer than 30 minutes from which the patient does not regain consciousness

CURRENT DEFINITION

IMPENDING STATUS EPILEPTICUS 0 to 5-10 mins

ESTABLISHED STATUS EPILEPTICUS >30 mins

REFRACTORY STATUS EPILEPTICUS >60 mins

•Highest incidence in very young children

• 70% of children with epilepsy experience at least one episode of SEMortality rate 8 to 32%

CAUSES OF SE

1)Prolonged febrile seizures

Lasting for >30mins Particularly in child younger than 3 years old Associated with severe damage to the

hippocampus in children(Hippocampus sclerosis)

Most common cause of SE May be the initial manifestation of

encephalitis, and epilepsy may be a long term complication of meningitis

CAUSES OF SE

2)Idiopathic status epilepticus

Includes epilepticus patients in whom SE followed sudden withdrawal of anticonvulsants(esp. benzodiazepines and barbiturates)

Given anticonvulsants on an irregular basis or who are noncompliant are more likely to develop SE

CAUSES OF SE(CONTINUED)

Acute head trauma Brain tumor Neurodegenerative disorders Hepatic or renal encephalopathy Storage diseases.

MECHANISM OF SE

Inreased cerebral metabolic rate

Increased in cerebral flow (half an hour) Inadequate oxygen tension and togetther

with other factors lead to

Neuronal injury

STATUS EPILEPTICUS

MANAGEMENT OF STATUS EPILEPTICUS

Securing airway ,breathing and circulation(with continuous monitoring of vital

signs ,ECG)

Determination and management of the underlying etiology(eg.hypoglycemia)

Laboratory studies(glucose,sodium,Ca) Blood and spinal culture,toxic screens test for inborn error of metabolism Antiepilectic drugs level EEG(ruling out pseudostatus epilepticus)

SUMMARY

affects 1 in 200 children Is classified as generalised or focal(partial) or

an epilepsy syndrome of childhood If suspected EEG is indicated Most but not all requires antiepileptic drug

therapy,which should be appropriate for the seizure,compromise as few drugs and with the least potential for unwanted effects as possible

Requires liaison with the school about the management of seizures and avoiding situations ehich could lead to injury.

REFERENCES

PAEDIATRIC PROTOCOL 2ND EDITION

ILLUSTRATED TEXTBOOK OF PAEDIATRICS 3RD EDITION BY TOM LISSAUER,GRAHAM

CLAYDEN

NELSON TEXTBOOK OF PEADITRICS 19TH EDITION

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