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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