Neurodegeneration is the umbrella term for the progressive loss of structure or function of neurons, including death of neurons. Many neurodegenerative diseases including Parkinson’s, Alzheimer’s, and Huntington’s occur as a result of neurodegenerative processes.
WHAT IS EPILEPSY?
Epilepsy is a chronic neurological condition
characterized by recurrent, unprovoked seizures,
occurrence of at least 2 unprovoked seizures 24
Epileptic seizure → clinical manifestation of
abnormal & excessive discharge of a set of neurons
in the brain
TONIC CLONIC SEIZURES
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,
This stage lasts for 10-20 seconds.
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
Patient stares briefly and stop talking or ceases to
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
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
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 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.
• Involve the motor
• May lead to clonic
movements → travel
• Asymmetrical tonic
seizures → bizarre
,hyperkinetic & easily
dismissed as non –
Temporal lobe seizure
• Most common
• Strange warning
feeling/ aura with
smell , taste
distortion of sound &
• Automatism → spread
to the premotor cortex
• Deja-vu & Jamais-vu
• Consiousness may be
impaired, length of
event is longer than a
• Distortion of vision
• Causes contralateral
• Distorted body image
SPECIAL EPILEPSY SYNDROME
-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.
Two unprovoked seizures >24 hr apart suggest the presence of an
Any aura?change in the behaviour?
Types of seizures
-tonic clonic(tensing,then shaking,LOC)
-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)
Infection during pregnancy?TORCH
Birth history - birth asphyxia , birth trauma
POST NATAL HISTORY
Central nervous system (CNS) infection e.g. meningitis,
Lead contact (lead fumes from burning batteries, pica)
Age at 1st seizure?describe seizure?
h/o febrile seizures
When and how diagnosed?any event preceding seizure?
1. Anticonvulsant medications
2. 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
2. Test done
3. Other investigations(eg.EEG to date)
1. How many?reasons?
Any identified medical problems associated with seizures?
1. Any history of trauma,meningitis?encephalitis?
2. How are this problem managed?
1. Of convulsion?inborn error metabolism?
Impact on child:
2. Athletic participation?
3. Self esteem
4. Does teacher know about the condition?
5. On family:financial burden
1. What to do in the event of a seizure?
The clinical diagnosis is more important then any
tool… (H/O, Eye witnesses, substantiated by video
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
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
Blood test and metabolic investigations(seizures related to
feed and fasting).
PRINCIPLES OF ANTICONVULSANT THERAPY
Treatment recommended if ≥ 2 episodes→ recurrence risk
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 &
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/
If seizures recur → last dose reduction is reversed &
medical advice sought
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 aggravating seizures
Lesional epilepsy, hence a potential epilepsy
Progressive epilepsy or neurodegenerative disorder
REFERRAL TO PAEDIATRIC NEUROLOGIST
Poor seizure control despite monotherapy with 2
Difficult to control seizures beginning in the 1st year
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
Know emergency treatment for seizure
Inform teachers and school abt the condition.
SIDE EFFECTS AND SERIOUS TOXICITIES
Steven Johnson syndrome)
hypotonia,salivary and bronchiol hypersecretion,paradoxical
(serious toxicity—megaloblastic anemia)
Any seizures lasting > 30 minutes OR
Intermittent seizures longer than 30 minutes from which the
patient does not regain consciousness
IMPENDING STATUS EPILEPTICUS
0 to 5-10 mins
ESTABLISHED STATUS EPILEPTICUS
REFRACTORY STATUS EPILEPTICUS
•Highest incidence in very young children
• 70% of children with epilepsy experience
at least one episode of SE
Mortality 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
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
Hepatic or renal encephalopathy
MECHANISM OF SE
Inreased cerebral metabolic rate
Increased in cerebral flow
(half an hour)
Inadequate oxygen tension and togetther with
other factors lead to
MANAGEMENT OF STATUS EPILEPTICUS
Securing airway ,breathing and circulation
(with continuous monitoring of vital signs ,ECG)
Determination and management of the underlying
Blood and spinal culture,toxic screens
test for inborn error of metabolism
Antiepilectic drugs level
EEG(ruling out pseudostatus epilepticus)
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.
ILLUSTRATED TEXTBOOK OF PAEDIATRICS
3RD EDITION BY TOM LISSAUER,GRAHAM
NELSON TEXTBOOK OF PEADITRICS