PEDIATRICS
BATCH 2021
DR. SONIA ROSARIO
SEIZURE AND EPILEPSY
 SEIZURE (previously known as “fits”) – most common neurologic
symptom encountered in the pediatric age group
- an abnormal excessive neuronal discharge arising from the
brain capable of causing alteration of function and/or
behaviour.
- It is a symptom, not a disease
 Could be provoked by
 fever
 metabolic disturbances
 hypoxia
 trauma
 infections
 unprovoked (spontaneous)
- can be convulsive/ non-convulsive
 CONVULSION a type of seizure with motor component
example: tonic clonic, clonic, tonic
 EPILEPSY – also known as seizure disorder
- characterized by two or more unprovoked seizures (recurrent
unprovoked seizures)
- epilepsy or seizure disorder is a diagnosis
- considered benign if it follows a favourable course ending in
almost complete remission (>95%) by adult life, absence of
risks of acquired brain damage and limited interference of the
patient’s everyday life.
many of benign seizure disorders belong to the pediatric
epilepsy syndromes
- considered refractory or recalcitrant if the seizures continue
despite optimal choice medications and/or Antiepileptic drug
(AED) used interfere with the patient’s daily functioning.
ETIOLOGY
- any injury to the brain sufficient enough can result in epilepsy
- many of the seizures are situational
example: presence of a trigger like metabolic disturbances like
hypoglycemia, trauma, infections, fever). These do not fall in the
category of epilepsy and requires serious and demand
treatment but don’t require continuous chronic antiepileptic
drug therapy
- epilepsy being a recurrent disorder, may impose danger to the
patient and to others (driving a car), thus requires chronic AED
management
- etiology of epilepsy is largely age dependent
- children <4 years of age are almost always due to symptomatic
causes like
a. the effects of a chronic encephalopathy that transpired
during the neonatal period (HIE)
b. past history of CNS infections
c. trauma
d. metabolic problems
- after 4 years of age, many epilepsies have no identifiable
cause(s) and by genetic
- common causes of seizures in adults like tumors and strokes,
seen rarely in children
GENERALIZED SEIZURE
more common in children
PARTIAL SEIZURE – with or without secondary generalization are more
common in adults
EPILEPSY SYNDROME is like any other syndrome, which is constellation
of signs and symptoms customarily occurring together at a greater
frequency by chance, with seizure as its major manifestations
PATHOPHYSIOLOGY
MAY BE GENERALIZED OR PARTIAL
 GENERALIZED – means that there is bilateral simultaneous firing of the
brain-leading to a convulsive (generalized tonic-clonic) or non-convulsive
(absence seizure)
- consciousness is impaired in generalized seizures
 PARTIAL – signifies that the discharging area is focal (localized) leading
to various manifestations, depending upon the location of the lesion.
- may be motor, sensory, autonomic psychic or mixture of these
- consciousness is usually preserved in partial seizures, although
it could be blunted
EPIDEMIOLOGY
- overall incidence of epilepsy in PH is not known
- epilepsy has a worldwide prevalence rate of about 0.5% to 0.7%
- majority of px (>75%) begin to have seizures before age 20
years
CLINICAL MANIFESTATIONS
- depend on whether they are generalized of partial
 GENERALIZED TONIC-CLONIC SEIZURE – most dramatic and most
frequently recognized type
January 27, 2020
BICOL CHRISTIAN COLLEGE OF MEDICINE
“GOD HEALS, WE SERVE”
- Typical episode would occur with or without warning, px
suddenly loses consciousness, the eyes turn upward, followed
by generalized stiffening and jerky movements of the
extremities
- Patient clenches the teeth and may become incontinent of both
urine and feces (relaxation of sphincters)
- The absence of incontinence during a seizure does not preclude
its occurrence – it simply means that the patient just emptied
the bladder before the event
DURING THE EPISODE – the many become apneic and cyanotic or at
the very least, have some labored breathing. Episodes generally lasts a
few minutes
- Rarely does the seizure lasts for longer than 5 minutes. Seizures
lasting longer than 5 minutes are generally classified as status
epilepticus, which represents a neurologic emergency
AFTER THE ICTUS – the px may go to deep sleep and wake up confused
and could recall any of the events.
 AURA – a warning before a seizure. Auras are actually the beginning of
seizure itself.
 NON-CONVULSIVE GENERALIZED SEIZURE is manifested by a starting
spell lasting for a few seconds and is usually associated with some
automatisms like:
 eyelid fluttering
 lip smacking
 chewing movements
 ABSENCE EPISODES are common in childhood absence epilepsy,
juvenile absence epilepsy and juvenile myoclonic epilepsy and are
regarded as generalized seizures
- Absence seizures are also seen in in partial seizures like
complex partial seizures
 MYOCLONIC JERKS are also manifestations of generalized seizure
problem and are characterized by bilateral synchronous brief
contraction of the head/neck and the upper limbs
 MYOCLONIC SEIZURES often occur in metabolic disorders but could
also be the major manifestations of certain epilepsy syndromes like the
juvenile myoclonic epilepsy
 ASTATIC OR ATONIC SEIZURES (drop attacks) – are also a form of
generalized seizure wherein the patient suddenly loses tone and falls.
More common in children with serious developmental problems like
mental retardation
 The signs and symptoms of partial seizures are largely dependent on
the location of the discharging lesion
- any part of the brain may be involved
 PARTIAL SEIZURES could be
a. simple (no impairment of consciousness)
b. complex (with impairment of consciousness)
- the patient may complain of sensory symptoms like tingling and
numbness on the side opposite the lesion in the parietal lobe
 FOCAL JERKING (unilateral and contralateral) may be experienced
when the lesion is in the frontal lobes
 OCCIPITAL LESIONS may manifest as visual aberrations or
hallucinations
 TEMPORAL LOBE involvement makes the symptoms more
complex, hence the term complex partial seizure (CPS) which was
originally called psychomotor seizures or temporal lobe seizures)
COMPLEX PARTIAL SEIZURES
CPS are associated with:
a. impairment of consciousness (confused state)
b. with some automatisms and absence episodes
- automatisms include:
 lip smacking
 chewing
 swallowing
 sucking
- the difference between CPS and that of classic absence
epilepsy is the longer duration of these in the former
 AURAS (warning signs) may precede the episode and consist of
complex illusory or hallucinatory experiences
- formed auditory hallucinations (voices) are more characteristic
of psychotic pxs
- visual hallucinations may also be present but these are more
common in toxic encephlopathies (use of hallucinogens)
 THE BEHAVIOR of patients with CPS may be erratic
- they could be aggressive and destructive
 some patients may run around (cursive seizures)
 laugh (gelastic seizures)
- others complain of olfactory hallucinations
 usually undesirable odors (uncinate fits) and
generally indicate a lesion in the uncus of temporal
lobe
 THE DURATION of CPS episodes vary from a few to several minutes
- partial seizures may remain partial however some can be
secondarily generalized
1
 PEDIATRICS
DR. SONIA ROSARIO
 CATAMENIAL EPILEPSY – adolescent girls may have increased seizure
frequency during menstruation
 THE RECOGNITION OF CPS IN INFANTS IS DIFFICULT
- symptoms and signs are often missed
- characterized by a TRIAD of:
1. motion arrest
2. blank stare
3. mild convulsive movements
- CPS in infancy is more common in neurologically impaired
children, though may rarely affect otherwise normal children
DIAGNOSIS
“NOT EVERYTHING THAT SHAKES IS EPILEPSY”
- this dictum should always be remembered when seeing a px
with alleged seizures
 DIAGNOSIS is mostly clinical
 based on history
 neurologic examination – however neuro examination especially
in a seemingly normal child in non-revealing
 GENERAL PHYSICAL EXAMINATION – should put emphasis on the
 head (size, shape, etc)
 skin (search for neurocutaneous stigmatas)
 eyes (cataracts, retinopathies)
 abdominal viscera (organomegaly)
 NEXT STEP after obtaining a detailed history and performing a decent
systemic and neurologic examination – and convinced that it is indeed
a seizure  CLASSIFY THE TYPE OF SEIZURE as to whether it is
a. partial (with or without secondary generalization)
b. generalized
 THE IMPORTANCE of classifying the type of seizure is because of the
relative specificity of the various AED’s to various seizure types
- this will limit the choices of drugs to prescribe
- another practical way of classifying seizures is according to
etiology
 idiopathic – genetic epilepsy
 symptomatic – px with identifiable etiology
(remnants of events that occurred during the
perinatal period and thereafter like postnatal
infections , trauma)
 cryptogenic – there is no obvious etiology but there
is evidence of neurologic problem (developmental
delay of unknown cause, mental retardation of
unknown etiology)
DIAGNOSTIC DILEMMAS AND DIFFERENTIAL DIAGNOSES
- one of the greatest problems encountered in the diagnosis and
management of seizures is seeing px who presents with a first
unprovoked seizure
- diagnosis of epilepsy on this px could not be justified
 definition requires 2 or more unprovoked seizures
 should be labeled “first unprovoked seizures”
 FIRST UNPROVOKED SEIZURE – defined as a single or cluster of
seizures occurring in a 24-hour period
- it is impt to exclude all possible triggers
 trauma
 hypoxia
 fever
 infections
 metabolic disturbances
 MAKE CERTAIN THAT THERE HAS NOT ANY NON-CONVULSIVE
SEIZURE(S) (absence, myoclonic jerks, etc) prior to the alleged first
unprovoked seizure.
- when these are present, then it is not a first unprovoked seizure
but rather it is a previously unrecognized/undiagnosed seizure
disorder
- seizure recurrence in the majority of patients following a first
unprovoked seizure recurrence is low, hence requires long term
observation
- putting the px on long term AED therapy is not warranted
January 27, 2020
BICOL CHRISTIAN COLLEGE OF MEDICINE
BATCH 2021
“GOD HEALS, WE SERVE”
 there are many disorders that mimic seizures hence it is
essential that the correct diagnosis is made otherwise
unnecessary AED therapy is prescribed
 ADVERSE EFFECTS OF AED’s are not only physical or systemic
but are impairment of cognition and behaviour as well
INVESTIGATIONS
 ELECTROENCEPHALOGRAPHY (EEG) – appears to be the most
impt of the various neurodiagnostic tools available
ADVANTAGES OF EEG
1. supports the diagnosis of epilepsy although a normal EEG
does not preclude diagnosis
- the only time that seizure can be confirmed by the EEG is
when the electrographic seizure discharge is recorded
2. it may identify a specific epilepsy syndrome. Some of the
syndromes are benign which may require a short term
treatment or none at all
- some however are not so benign and require tx for a long
time if not for life
3. it may predict recurrences
 REMEMBER: the EEG patterns during the walking period and during
sleep are different and certain sedatives could produce artifacts in
the EEG (can be mistaken for “spike and waves.”
 IF A SEDATIVE IS TO BE USED: it is highly recommended NOT TO
USE THE BENZODIAZIPINES as these may “wipe out” epileptic
discharges.
 Neuroimaging studies are not always indicated in newly diagnosed
seizure disorder especially in a child with normal
neurodevelopmental assessment.
- can be necessary if seizures become refractory or if there are
abnormal neurologic examination findings
 MRI – neuroimaging of choice in seizure
- when requesting for MRI, one must insist that the seizure
protocol be used otherwise the yield of the test is not that great
 LUMBAR PUNCTURE (LP) with CSF analysis is also not indicated for
most seizure px except when infection of the CNS is suspected
MANAGEMENT
 The general management of seizures, whether situational or
epileptic in nature is the same (termination of the episode with
the use of AED’s
 DIFFERENCE IN MANAGEMENT BETWEEN 2 GROUPS
1. The etiology of situational seizures should be treated
and/or corrected, otherwise the seizure perpetuates
 Seizures from hypoglycemia should be treated
with glucose ifusion, which usually follows after
an AED has been infused.
2. Chronic AED therapy in situational seizure is not indicated
 the AEDs are stopped once the etiology has been
corrected and the seizures have not recurred
 depending on the duration of the metabolic
defect and/or seizure these patients are at risk
for future seizures because the damage induced
by metabolic abnormality and/or seizure may be
permanent
 should epilepsy occur after situational seizures,
then chronic AED therapy may have to be started
in the same manner as managing epilepsy
 EPILEPSY is a chronic disorder
- Although short lasting seizures per se do not produce
permanent damage, prolonged and repeated seizures may be
deleterious and may produce long term sequelae or death
 DEATH IN EPILEPSY may be due to injuries or an end-effect of a
very prolonged seizure episode (status epilepticus)
 THE GOAL OF THERAPY is to
1 eliminate recurrences or at least reduce the frequency of
occurrence
2 to reduce undesirable side effect of AEDs
 THE CHOICE OF MEDICATIONS will greatly depend on the type
- the efficacy of the AEDs in specific seizure types and their
adverse effects should be given the highest priority in their
choice
- polytherapy produces a number of disadvantages such as drug
interactions, non-compliance, increased side effects and higher
cost
 THE MANAGEMENT of epilepsy should be continuous use of AEDs
- although AEDs do not cure the problem, they raise seizure
threshold.
- for better compliance, doses should be kept minimum and these
depend on the half-life of the AED
- when seizures had no recurrence for a minimum of 2 years, AED
withdrawal should be considered especially in otherwise normal
children
- withdrawal should be gradually tapered off for a period of at least
2 months
2
 PEDIATRICS
DR. SONIA ROSARIO
 RISK FACTOR for recurrence include:
 mental retardation, long duration of epilepsy prior to
control, abnormal EEG showing slow backgroundand/or
“epileptic”pattern at the time of discontinuation
ANTIEPILEPTIC DRUGS
 The “newer” drugs are relatively specific to certain seizure types,
stressing the need to classify the type of seizure.
 Most AEDs are ion-channel blockers, glutamate blockers, carbonic
anhydrase inhibitors and gamma amino butyric acid (GABA) enhancers
 When prescribing AEDs, drugs are given slowly to minimize the side
effects and increased gradually (every 3-4 days) until the desired
dose is reached.
 WHEN SEIZURES ARE NOT CONTROLLED, they are placed on the
first choice drug and then serum levels have to be determined.
Serum levels should be taken before the next dose.
- if measured randomly and especially when drug was just
administered, the levels will be high
- the dose may be inadvertently lowered, while the actual level is
low
 when 2 or more drugs are listed on the same category, the actual choice
of a single drug may have to be made individually based on:
a. side effect profile
b. age
c. gender
d. preference
 Antiepileptic drugs of similar MOA should not be given in
combination or as a 2nd choice drug when the patient is not
responding to the first.
Kindly check page 893 (table 37-6) on our book for the antiepileptic agents and their uses, doses,
half-lives and adverse effects
CONTROLLING SEIZURES IN CHILDREN WITH DEVELOPMENTAL
DISABILITIES
 Because of the underlying brain pathology in children with
developmental disabilities is often diffuse and bilateral, the patients are
at high risk of developing seizures.
 Certain epilepsy syndromes are associated with developmental
disabilities
Examples: West syndrome, Lennox-Gastaut syndrome, Landau-Kleffner
syndrome
 Seizures are often refractory to treatment
 The principles of treatment in this group of children is essentially the
same as when treating otherwise normal children with epilepsy
 Seizures in this group are often mixed type and in general difficult to
control
 These children often experience frequent status epilepticus
episodes, many of which are non-convulsive in nature and may
escape recognition.
 Changes in the behaviour of children should alert the physician to the
possibility of non-convulsive status epilepticus.
 the choice of drug is dependent on the seizure type
check table 33-7 page 894
 graduating from AEDs in epileptic children with developmental
disabilities is often not possible, so that many will remain on AED
for a lifetime.
 These patients are at risk for adverse effects that are not readily
recognized because most of them are unable to talk
KETOGENIC DIET
 once popular in the treatment of refractory seizures especially the
difficult-to-control types like atonic seizures
 being used again because there is still a number of children whose
seizures remain refractory to high dose AED or polypharmacy, as well
as children who show adverse reactions to AEDs
 the initiation of the diet requires an initial hospitalization of the child
to ensure that ketosis develops and that the diet is strictly followed
- as well as the parents to learn how to prepare the diet
 many children dislike the diet because it is unpalatable, and this
poses a danger to the child who may be thrown into a negative
nitrogen balance
SURGICAL MANAGEMENT OF EPILEPSY
 After a good clinical trial of antiepileptic medications and still the
patient does not respond, surgical treatment may be considerd
 Surgery may be suggested for patients who show toxicity to the drugs
and the seizures themselves have incapacitated their lives
- not all patients with intractable seizures are considered for
surgical management
 individuals with persistent focal seizures with a focal EEG and a focal
abnormality on the MRI are perhaps the best candidates for surgery
- they are likely to benefit from excision of the discharging focus
(lesionectomy)
 patients with severe focal seizures with or without secondary
generalization whose entire hemisphere is involved may benefit from
hemispherectomy such as:
 Sturge-Weber syndrome
 Rasmussen encephalitis
 Congenital hemiplegia
January 27, 2020
BICOL CHRISTIAN COLLEGE OF MEDICINE
BATCH 2021
“GOD HEALS, WE SERVE”
 cases with refractory atonic seizures that fail to respond to AEDs may
benefit from collosotomy (splitting of the corpus callosum)
- this prevents the spread of the discharge to the other
hemisphere
- not effective for pure focal seizures
VAGUS NERVE STIMULATION
- another surgical intervention for epilepsy
- uses a programmed stimulus from a chest-implanted generator
via coiled electrodes to the left cervical vagus nerve
 HOARSENESS is the main adverse effect
- significant seizure reduction has been reported with this
method of tx
PEDIATRIC EPILEPSY SYNDROMES
 EPILEPSY SYNDROME – is a cluster of signs and symptoms
customarily occurring together with seizures as a major component
- some are not true epilepsies, but these can be triggered by a
provoking factor
- yet the phenotypes are more or less constant and possess the
other criteria enumerated above to make them part of epilepsy
syndromes
 example: febrile seizure (FS) are not epilepsies
because there is a trigger (fever) yet the
characteristic picture is practically the same for all of
the patients
FEBRILE SEIZURES
 no longer exist as an independent condition
 a number of seizures phenotypes have been recognized related to
fever
 FS are the most common seizure phenomenon in children
- 2–5% of all children worldwide experience at least 1 episode of
FS
- convulsive event occurring in a febrile child between the ages of
1 month to years but without intracranial infection
- rare before 5 months and after 5 years of age. It excludes those
with prior non-febrile seizures
- has a genetic basis and is inherited as an autosomal dominant
disorder. It affects both sexes, but with a slight male
preponderance
 PEAK OCCURRENCE OF FS is 18 months of age
 FS are generally brief, generalized and non-recurrent in that particular
febrile illness
- may be prolonged (>15 minutes), focal or recurrent in the same
febrile illness
- in the former is called simple FS while the latter is termed
complex FS
 DIAGNOSIS OF FS IS USUALLY STRAIGHTFORWARD
- eliciting a good history will establish the diagnosis
- a common event mistaken for FS is febrile syncope
- fainting due to fever is not common
 FEBRILE SYNCOPE is characterized by
a. blank stare
b. unusual pallor
c. limpness in contrast to that of FS which is actual shaking,
upward rolling of the eyes and cyanosis
- syncope lasts momentarily while FS may last for a couple of
minutes
 FS ARE BENIGN, leaving no sequelae. The risk for death, neurologic
sequelae or injuries following FS is not different from that of general
population
 MENINGITIS – most important differential diagnosis of FS irrespective
of its etiology
- Since the s/sx of meningitis are meagre in a very young child, it
is highly recommended that a lumbar puncture (LP) with CSF
analysis be performed in all children under 12 months of age
 RECURRENCE RATE for FS is low. 1/3 of the children who has 1 FS
episode will experience a 2nd, while ½ of those with 2 FS episode will have
a third.
- more than 3 FS episodes are unusual and may indicate another
problem
 RISK FACTORS FOR RECURRENCE:
1. occurrence of the FS at young age <18 months
2. seizure at the outset of the febrile illness
3. family history of FS
4. presence of neurodevelopmental abnormality
5. attendance in a day care nursery (due to increased illness
exposure)
each risk factor represents a 6% risk for recurrence in FS
 MANAGEMENT OF FS lies on the tx of the cause of fever. Nothing is done
for the seizure
- the child who is actively convulsing should be given a rapid
acting AED like diazepam to terminate the seizure.
- under no circumstance should a child not in active convulsion
should be given parenteral AED
 the relationship between FS and epilepsy is not known. Although FS is the
most frequent antecedent for epilepsy, predicting which child will develop
epilepsy is virtually impossible.
3
 PEDIATRICS
DR. SONIA ROSARIO
- a child with a single FS has a 10-fold risk of epilepsy, which is
low according to statistics. Overall, 96-98% of the children with
FS will not develop epilepsy
- FS remits spontaneously usually by age 6 years
 because it is benign and rare to occur, prophylactic
AED therapy is not indicated
 if however, prophylactic AED is prescribed, there are only a
handful of AEDs that are effective in preventing recurrences of
FS
 these are phenobarbital and valproate given everyday
 intermittent rectal diazepam at the time of febrile
illness to a child who is highly susceptible to
recurrence
FEBRILE SEIZURE PLUS
 FEBRILE SEIZURE PLUS (FS+) is a term to denote the phenotype of
children who have FS extending beyond the age of 7 years
- these children suffer from febrile seizures, and generally at
younger age than the regular FS
- the average age at which FS occurs in this group of children is
12 months (in contrast to 18 months in the regular FS)
- the seizures are recurrent and over time, the children develop
non-febrile seizures and other types of seizures, although the
generalized tonic-clonic seizure is still most frequent
 FS+ is the 2nd most common disorder in the spectrum
- like the regular FS, FS+ is also a genetic disease
- it is autosomal dominant in transmission
- has been found due to sodium channelopathy
 THE EEGs of these children are initially normal, but become abnormal
particularly when they develop non-febrile seizures
- seizures are frequent and occur with or without fever
- despite multiple recurrences of the seizures, children with FS+
remain neurologically and developmentally normal
- seizures generally remit at age 11 years
- children with FS+ and with other types of seizures may
experience continuation of the seizures through adult life
SEVERE MYOCLONIC-ASTATIC EPILEPSY OF INFANCY (DRAVET SYNDROME)
 more common term for severe myoclonic-astatic epilepsy of infancy
is DRAVET SYNDROME
- worst of the spectrum of febrile seizures but rare
- like FS and FS+ patients begin to have seizures in association
with fever
- patients who develop DS are not just like those with the regular
FS and FS+
- no known etiology for this syndrome
 THE CLINICAL COURSE OF THIS SYNDROME IS DIVIDED INTO 3
STAGES:
 FIRST STAGE
- occurs in infancy
- there is early onset of FS at less than or equal to 6
months which are recurrent
- the seizure is usually generalized but could be focal and
short or prolonged
- FEBRILE STATUS EPILEPTICUS may be the presenting
symptom and may be recurrent and frequent
- other types of seizures as well as non-febrile seizures
emerge
- the neurodevelopmental status of these children during
this stage is normal
 SECOND STAGE
- occurs during toddler age extending through mid -
childhood
- myoclonic seizures generally begin to manifest at about
4 years of age or so and continue to be recurrent and
frequent bouts of status epilepticus are seen
- neurologic sequelae
 neurocognitive decline
 spastic hemiparesis or quadriparesis
(especially in prolonged status epilepticus)
 THIRD STAGE
- occurs from mid-childhood to adolescence
- during this stage, the seizure frequency becomes less
but neurocognitive decline and deficits are quite
noticeable and are permanent
 THE EEG of these children are initially normal, but become abnormal
in time
- MRI studies are also normal in the beginning but will show some
form of cerebral atrophy
 THE DIAGNOSIS of this syndrome is clinical, although
neurodiagnostic tests like the EEG and MRI are abnormal (these are
non-specific). The clinical course of this disease is more highly
suggestive
 TREATMENT for this syndrome is often non-rewarding.
- multiple AEDs may have to be combined but seizures often are
refractory
- of the newer AEDs, topiramate and levetiracetam may be
beneficial
- carbamazepine and lamotrigine may worsen the seizure
January 27, 2020
BICOL CHRISTIAN COLLEGE OF MEDICINE
BATCH 2021
“GOD HEALS, WE SERVE”
- aggressive control of prolonged seizures may minimize
neurocognitive decline and may be life saving
HEMICONVULSION-HEMIPLEGIA SYNDROME (HHS)
HHS is a rare epilepsy syndrome

- it is characterized by the occurrence of prolonged unilateral
seizure that may last for days in a febrile child
- occurs between the ages of 5 months and 4 years without any
known antecedent illness
- after the cessation of the seizure, the affected limb is flaccid
and involvement of the face is noted
- the hemiplegia becomes spastic over time
- the seizure may shift from 1 side to the other and wherever the
side manifest the last convulsion becomes the hemiplegic limb
 nothing can be done to the hemiplegia once it has set
in
 there might be a confusion as to whether patients with this syndrome
may have had a pre-existing unrecognized mild hemiparetic cerebral
palsy (CP)
- the flaccidity that follows the seizure and the involvement of the
face are strong evidences that the hemiplegia is acquired
- it could also be argued that the hemiplegia is post-ictal (TODD
PARESIS)
- post-ictal paresis is transient whereas that of HHS is permanent
 TREATMENT consists of aggressive control of the unilateral seizures
to prevent or at least minimize hemiplegia
- Benzodiazepines (diazepam, lorazepam, midazolam)  drug of
choice to terminate the seizure
- Focal epilepsy effecting the hemiplegic limbs is expected to
follow however may not appear until 1-3 years
- Tx consists of AEDs particularly effective against partial seizures
 PROGNOSIS of this syndrome is not good because the hemiplegia is
permanent and focal epilepsy is persistent
MYOCLONIC-ASTATIC EPILEPSY OF CHILDHOOD (DOOSE SYNDROME)
 cases of this syndrome begin with recurrent febrile seizures
 children with this condition are normal prior to the onset of seizures
 the peak of occurrence is between the ages of 2 and 4 years (range: 7
months to 6 years)
 several months after its onset:
- myoclonic and astatic (drop attacks) seizure appear which are
hallmark of the diagnosis
- MYOCLONIC JERKS are symmetrical followed by loss of tone
(astatic) and may cause patients to fall
- ASTATIC COMPONENT may just be a simple head nod or bending
of the knees
- Half of the patients with the Doose Syndrome have absence
seizures too. Astatic and absence seizures may occur many
times a day
 These children may suffer bouts of convulsive and nonconvulsive status
epilepticus. The EEGs are abnormal but are non-specific
 There is no known etiology of this syndrome. It is highly genetic and is
related to the febrile seizure spectrum
 TREATMENT consists of high-dose valproate, which may go over the
recommended maximum dose
- A combination of this drug with levetiracetam or ethosuximide
or clonazepam has been found to be beneficial in the patients
who suffer from multiple seizures
 PROGNOSIS of this syndrome is not well delineated, but half of those
with the true (without an etiology) Doose Syndrome attain seizure
freedom and are normal in their developmental status
- the other half will experience seizures even in adult life
- patients with known etiology do not attain seizure freedom and
are cognitively impaired
BENIGN CHILDHOOD EPILEPSY WITH CENTRO-TEMPORAL SPIKES (BENIGN
ROLANDIC EPILEPSY OF CHILDHOOD)
 The syndrome is officially termed benign childhood epilepsy with centro-
temporal spikes but it is better known as benign rolandic epilepsy of
childhood (BREC)
- this is a common epilepsy syndrome but it is frequently
unrecognized. It is called rolandic epilepsy because the spike
discharge falls along the rolandic fissure (centro-temporal)
 BREC is a genetic disorder and is autosomal dominantly transmitted
- both sexes are affected with a very slight male preponderance
- the seizures appear in an otherwise healthy child between the
ages of 3 and 13 years, with a peak occurrence at 8-9 years
- the seizures may occur at any time of the day, but mostly
nocturnal
- the child awakens about 2 to 3 hours or so after falling asleep
making gurgling sounds. Then the face twitches, and could
remain focal or could be secondarily generalized
 PURE FOCAL SEIZURE is more common in the older child, while
secondarily generalized seizure is more seen in younger ones
- the episode lasts for a few minutes. seizures occur rarely, but
may be more common in young children and in those with short
interval of seizures
4
 PEDIATRICS BICOL CHRISTIAN COLLEGE OF MEDICINE
BATCH 2021
DR. SONIA ROSARIO “GOD HEALS, WE SERVE”

- the child remains normal in all domains of development. The

diagnosis is mainly clinical aided by the EEG, which shows the
characteristic centrotemporal spikes on 1 side
- because of its rare occurrence and its benign nature, the option
for a no AED therapy course is advised
- AEDs are indicated when seizures are frequent, and this
syndrome responds fairly well to the more common AEDs,
especially those formulated for partial seizures
BENIGN OCCIPITAL EPILEPSY (PANAYIOTOPOULOS SYNDROME)
 Benign occipital epilepsy is characterized predominantly with visual
symptoms, consisting of hallucinations, illusions, or blindness at the
outset of the episode
- the age of onset for this syndrome can be as early as 15 months
but could be as late as 17 years. The peak of onset is 5 to 7
years
- there is a slight preponderance among females.
- the symptoms may occur at any time of the day, but more
frequently diurnal and are expressed by the visual symptoms.
Vomiting, jerky movements, and tonic deviation of the eyes
manifest nocturnal seizures
 This syndrome mimics the symptoms of vertebro-basilar migraine and
is often difficult to distinguish.
- the seizure frequency is variable from rare to frequent. The
EEG shows a characteristic high-voltage spike and wave
complexes located in the posterior head regions (occipital) and
postero-temporal but these appear only when the eyes are
closed. The seizures may remit early (2 years from onset) but
may extend through the age of 12 years
- the syndrome is benign and like rolandic epilepsy, there is an
option of no AED treatment
LENNOX-GASTAUT SYNDROME
 The Lennox-Gastaut syndrome (LGS) is not an uncommon pediatric
epilepsy syndrome
- it is nonspecific and is due to a variety of causes.
- brain injuries acquired during the perinatal or even antenatal
period are most common causes
- it may also be due to dysgenetic CNS as well as progressive
neurologic disorders
- it is the continuum of the West syndrome.
 The LGS is characterized by a triad of
1. mental retardation
2. epilepsy
3. a "petit mal" EEG discharge of 2-4 Hz spike and wave
 Mental retardation is an integral component of the syndrome.
Seizures are of variable types and occur quite frequently, but are
often missed. Convulsive and non-convulsive status epilepticus may
happen in this syndrome.
 Ambulatory Lennox-Gastaut patients may show behavioral problems
such as hyperkinesis and aggressiveness.
- the seizures are often refractory to treatment.
- valproate is the drug of choice, followed by either levetiracetam
or topiramate
- the ketogenic diet may also be tried.
 THE PROGNOSIS of this syndrome is poor because of the
refractoriness of the seizures and mental retardation
===================END OF FIRST PART====================


INFANTILE SPASMS (WEST SYNDROME)
this is an age-specific epilepsy syndrome occurring in infants between
“you’re already a little
the ages of 3 and 12 months, peaking between 4 and 7 months farther than you were
yesterday, keep going.”
 it is characterized by a triad of:
1. developmental delay (mental retardation),
2. spasms
3. characteristic EEG pattern called hypsarrhythmia (high
amplitude disorganized brain waves with frequent multifocal
spike and waves appearing mostly during sleep though these
are also seen during the waking period) TRANSCRIBED BY: DOC THEL
 THE TRIAD IS NOT ALWAYS PRESENT
 they last only momentarily and are often missed except if they occur
quite frequently. There may be a few episodes, but often occur in
hundreds per day and in clusters
- the child may moan or cry during a spasm. Because of the
brevity of the episode, it is also known as lightning jerks or
salaam seizures mimicking the Muslims gesture of greetings
- there is no specific etiology for this disorder
- majority of infants suffering from infantile spasms have an
underlying etiology. Known causes are the after effects of
stigmatizing neonatal-perinatal events (hypoxic ischemic
encephalopathy) infections, severe metabolic uncorrected
disorders, and dysgenetic CNS
- this syndrome is also frequently seen in a neurocutaneous
syndrome called tuberous sclerosis. Thus, when confronted with
an infant with infantile spasms, a thorough search for the
cutaneous manifestations of tuberous sclerosis should be
made it is also a characteristic seizure pattern in a rare
condition called Aicardi syndrome
- Aicardi syndrome is of unknown etiology practically limited only
to girls. In this syndrome, there is :
a. agenesis of the corpus callosum
b. vertebral anomalies
c. ocular abnormalities including microphthalmia,
chorioretinal defects, and coloboma of the optic nerve
 The demonstration of hypsarrhythmia in the EEG is highly diagnostic
of the condition.
 TREATMENT for this syndrome deviates from the usual management
of the other epileptic conditions in that adrenocorticotrophic hormone
(ACTH) is the drug of choice.
- ACTH, when given, should be in the gel form.
- it is administered intramuscularly, starting at a relatively high
dose of 150 u/m2/day for about 2 weeks, and tapered off on a
weekly schedule for 6-8 weeks.
- other AEDs found to be effective include vigabatrin especially in
patients with tuberous sclerosis, and high dose valproate.
- Prednisone has been used, but is not as effective as ACTH
 THE PROGNOSIS of this syndrome is not good because of its
associated developmental delay, leading to mental retardation.
- the cognitive impairment is not due to the seizures, but to the
underlying encephalopathy that has generated the spasms

January 27, 2020

5