Paroxysmal disorders:
Abrupt onset of a clinical episode that
and repetitive , tends to be stereotyped
lasts seconds or minutes (rarely hours ),
and ends abruptly.
Definition of seizure :
transient and abruptly disturbance of
cerebral function(impaired consciousness,
abnormal motor activity, sensory
disturbances or autonomic dysfunction)
caused by excessive or over synchronized
cerebral neuronal discharges.
Differential diagnosis of Paroxysmal
disorders:
• Seizures
• Migraine
• TIA
• Syncope
• Breath holding
spells
• Paroxysmal Vertigo
• hypoglycemia
•
•
•
•
•
Tics
Pseudo seizures
Night terrors
Narcolepsy
Paroxysmal
torticollis
• Paroxysmal
dystonia
Seizures ocurr in 10% of children.
Less than one third of seizures in
children are caused by epilepsy.
• Epilepsy is the occurrence of two or
more unprovoked seizures at an
interval > 24 hours
• Provokeing factors :
fever ,infection, syncope, hypoxia,
toxins, head trauma, stress,fatigue,
cardiac arrhythmias
Epileptic syndromes classification
•
•
•
•
Age of onset
Etiology
Seizure type
Cognitive
development
• Neurologic exam
,CT
• prognosis
•
•
•
•
•
•
Janz syndrom
West syndrom
Lennox-Gastaut syn.
BPEC
LKS
Benign neonatal
convulsion
Clinical Seizure classification
Partial
(Only a portion
of the brain)
- Simple
(Normal consciousness)
- Complex
(Impaired consciousness)
Generalized
(Both hemispheres are
involved)
Type of Seizure
Classification
• Partial (Focal Seizures) : 40-60 % of
epilepsy of childhood
• Simple partial
– Simple with motor signs
– Simple with sensory signs
– Autonomic: abdominal epilepsy
– Psychic :déjà vu,fear,…
• Complex partial : psychomotor seizures or
temporal lobe epilepsy or limbic
Classification
• Generalized Seizures
– Absence Seizures (Petit mal)
– Tonic/Clonic (Grand mal)
– Atonic Seizures (Drop attacks)
– Myoclonic Seizures
– Clonic
– tonic
Simple Partial seizures :
• The most common form : motor activity
• SPS arise from a anatomic specific focus.
Location and direction of spread of the
seizure focus determine clinical
symptomology.
• Spread of partial seizure to the whole brain
produced GTCS. (secondry generalization ).
• Only pshycic or autonomic symptoms can be
difficult to diagnosis.
SPS : 10 -20 sec
• Uncinate seizure :)‫احساس بوي نامطبوع (تمپورا ل مياني‬
• Gelastic seizure : spells of uncontrolled laugher
hypothalamic tumors
• Versive seizure :‫انحراف سر و چشم به يك سمت‬
• lip smacking : anterior temporal lobe
• Macropsia , Micropsia, vertigo: posterior temporal
lobe
• Autonomic :fever ,tachycardia ,shivering and
increased GI motility
• Limbic seizure :dream like state and bizarre
psychic abnormalities
CPS
• Aura:in one third (epigastric discomfort,
vague unpleasant feeling , fear)
• Automatisms: in 50-75 %
in infant :alimentary , in child:behavioral
• EEG: Spike or sharp in anterior temporal
• Neuroimaging: abnormal in 50-80%
tumor, AVM, MTS, cortical dysplasia,focal
atrophy, gliosis, infarction
• There may be a brief blank stare or a
sudden cessation or pause in activity.
Goals of epilepsy treatment
Indication for start of AEDs
 Control of seizures
 Minimal adverse events
 Good patient compliance
 AEDs suppress but do not cure seizures
 80% of patients have control of seizures
60% seizure free, 20% drastically reducing
with treatment
 Main aim is monotherapy
 Significant improvement in only 10 % of
patients with second drug
Choosing AEDs :
 Seizure type & Epileptic syndrome
 Pharmacokinetic profile
 Medical history , interactions/potential for future
problems
 Efficacy
 Expected adverse effects
 Cost
Start at low dosage , gradually increase until
seizure control or side effect threshold
 Never abruptly stop medications
Principles of antiepileptic drugs
treatment
Pharmacokinetics : What the body does to drug
( absorption , distribution, metabolism, elimination )
Pharmacodynamics : What the drug does to body
( study of biochemical and physiologic effects of drug)
 Toxicology
When a total daily dose is increased , sufficient
time (about 5 t 1/2) should be allowed for the
serum drug level to reach a new steady-state level
 Withdrawal may be considered after
seizure- free period of 2-3 or more years.
 Relapse rate after withdrawn AEDS : 20 -40 %
Treatment of generalized Seizures
GTCS:
First choice : valproate
Second : CBZ , PHT , TPM , LMC , Pb
CLZ , PRM , Zonisamide
Absence :
First choice :Valproate , ethoxysuximide , LMC
Second choice : TPM, Levetiracetam , Zoni
Myoclonic :
First : Valproate
Second : LMC , CLZ, Levetiracetam , Zoni
 Carbamazepine may exacerbate absence and
myoclonic seizures
Partial Onset Seizures
First-line drugs are carbamazepine and phenytoin.
PHT rarely used as first-line agent in children
because of toxicity.
 Carbamazepine : an acceptable first drug
 Gabapentine is another option
Adjunctive (add-on) therapy :
oxacarbamazepine , LMC, TPM, VPA , Pb, PRM ,
felbamate, levetiracetam , tiagabine, zonisamide
 WHO recommends Pb as choice treatment of partial &
TCG seizures in countries with restricted resources .
Treatment of partial seizure
• Carbamazepine : drowsiness, dizziness,
diplopia ,SIADH (hyponatremia ) ,aplastic
anemia , agranulocytosis
• Phenytoin :
gingival hyperplasia , hirsutism ,rickets ,
SLE, ataxia, coarse face, nystagmus,
psudolymphoma, Stevens – Johnson
syndrome
Benign focal epilepsy or rolandic epilepsy or
BPEC :
• Age of onset : 5-10 years
• 16% of all afebrile seizure in <15 years
• 50-75 % occur during sleep(20 % only one
seizure)
• Somatosensory aura around the face and
mouth and then focal motor (face and then
ipsilateral exterimity ) ,and finialy secondry
generalized.
• Good prognosis and normal CT,IQ,N.E
• EEG :spike in centrotemporal
• Treat: CBZ in frequent seizures for 2 yr
Landau-Kleffner syndrome or
Acquired Epileptic Aphasia :
• Onset age :early childhood (4-7 yr)
• Cortical auditory deficit and language
disability
• Seizure in 80% (partial and generalized)
• EEG :highly epileptiform during sleep
• CT or MRI :normal
• Prognosis :not good
• Treat :Nav, clobazam, steroid ,IVIG
Rasmussen encephalitis:
chronic , progressive focal inflammation of the
brain of unknown origin
• Usual age of onset :6-10 yr
• Nonspevific viral disease
focal persistent
motor activity (epilepsia partialis continiua )
hemiplegia and cognitive deterioriation.
• EEG:focal spike and slow wave activity
• Imaging :initially normal and then show atrophy in
the involved area
• Prognosis :progressive and lethal
• Hemispherectomy for seizure eradication and
prevention of cognitive deterioration but with
permanent hemiparesis
Absence seizures
•
•
•
•
•
•
•
•
In 6-20% of epileptic children
No aura, no postictal,no loss of tone
Age: 4-6 yr
NE & imaging :normal
A brief loss of enviroment awareness and starig or
eye fluttering or simple automatism such as head
bobbing &lip smacking
EEG: 3cps spike and wave
Seizures provoked by HV & flashing light
40-50% have generalized seizures(60%before
absence & 40% after the onset of absence.
Treatment : Ethosuximide ,valproate
clonazepam as alternative
•
•
•
•
•
•
Differentiating of absence from CPS
1.The automatism of CPS are more
complicated (repetitive swallowing, picking of
the hands or walking in nonpurposeful circles
2.postictal confusion in CPS
3. Absence provoked by hyperventilation
4.CPS last few minutes ,absence :few sec
5.absence :dozens per day but patients with
CPS rarely have more than one or two seizures
in day
EEG
Often referred as behavior problem,
Poor school marks; disruptive behavior
Side effects of drugs:
• Valproate :pancreatitis, drowsiness, tremor ,
alopecia, weight gain, fatal liver failure
(Reye-like syndrome ) in <2yr
• Ethosuximide : nausea ,lethargy, hiccups
,SLE, Stevens – Johnson syndrome ; blood
dyscrasia
• Clonazepam :ataxia, lethargy, blood
dyscrasia ,depression, salivation
Myoclonus:a lightning-like jerk of part
of the body
• Epileptic: EEG shows epileptiform discharges
during the jerk.
• Nonepileptic :may originate in the B.G ,BS, or
spinal cord.
•
Benign: sleep myoclonus
•
Serious pathology
• Underlying illness produce myoclonic epilepsy:
- developmental or static
- progressive
Juvenile myoclonic epilepsy or Janz
syndrom :
• Onset age : 12-16 yr (adolesenct )
• AD ( chromosome 6)
• Myoclonus in the morning with or without
generalized clonic and absence seizure .
• N.E is normal.
• EEG : 4-6 / SWD
• Treat : valproic acid for lifelong
Infantile spasms or west syndrome :
•
•
•
•
•
•
•
•
•
•
•
Age : 3-8 month , 86 % onset of seizures : < 1yr
Forms : mix , flexor , extensor
spasms occur in drowsiness state.
Repeated clusters occur each day.
EEG : hypsarrhythmia ( HVSW, spikes, polyspike and
disorganized background)
Poor prognosis
Classification : cryptogenic (40% )
symptomatic :TS is the most common
40 % of cryptogenic have a good intellectual.
Very Poor intellectual prognosis in symptomatic
Treat : ACTH , cortone ( irritability , swelling ,hypertension ,
glycosuria, severe infection ) benzodiazepines , valproate
Etiologies of infantile spasms
• Methabolic : PKU,
MSUD, biotinidase
deficiency ,NKH,
hypoglycemia , B6
dependency,
lipidosis,…
• CNS dysgenesia :
polymicrogyria,
lissencephaly, Down
syndrome,…
• Neurocutaneous :
tuberous scerosis,….
• Congenital infections :
toxo, CMV, syphilis
• Encephalopathies:
postasphyxia
posttraumatic
posthemorragic
postinfections
postimmunization
(pertusis )
Etiology of seizures
Perinatal conditions :
CNS malformation, hemorrhage, HIE, congenital
infection, trauma
 Metabolic conditions:
hypoglycemia,hypocalcemia,B6 deficiency
hypomagnesemia, hypo or hypernatremia , storage
disease, degenerative , Reye syndrome
 Poisoning :
lead , cocaine, cyanide , co, pesticide, strychnine
 drugs :
، ‫فنوتيازين ليتيوم‬،‫آنتي كولينرژيك‬، ‫آنتي دپرسانت‬، ‫آمينوفيلين‬
‫ ساليسيالت‬، ‫ آمفتامين‬، ‫ناركوتيك‬
‫ايزونيازيد‬،
Etiology of seizures
Neurocutaneous
syndromes :
Tuberous sclerosis , NF,…
Systemic disorders :
vasculitis ( CNS or systemic ) SLE
hypertensive encephalopathy
renal failure
hepatic encephalopathy
 Infection: ‫ آبسه مغزي‬، ‫ مننژيت‬،‫انسفاليت‬
 Other : trauma , tumor, idiopathic , familial
 Over-the-counter drugs, illicit substances herbal
preparations, can precipitate seizure
Laboratory evaluation of seizures
• CBC
• Glucose,
ca,mg,p
• Na,K,Bun,Cr
• Toxicology
• CSF
• EEG
• Imaging
• metabolic
Neuroimaging :
 Neonatal seizure
 Focal seizure
 focal EEG
 Focal neurologic finding
 Neurodevelopmental delay
 Dysmorphic face
Breath holding spells
• In 5 % of children, rare in <6mo and >5-8 yr,
80% <18mo all in <3yr,
• Cyanotic BHS :crying,prolonged expiratory
apnea,cyanosis,UWG,tonic-clonic movement
treat: reassurance, piracetam, ferrous
• Pallid BHS: painful stimuli,asystole, pallor,
bradycardia, opisthotonos ,seizure
treat : atropine , less benign than cyanotic
Fever and seizure:
• 1. CNS infection
• 2. Epilepsy triggered by fever
• 3. Febrile seizure
 F.C occur in 2- 4 % of children.
 AD (choromosome 19 & 8)
 50 % in 1-2 yr , 93% in < 3 yr
 URI, roseola, AOM are the most common causes of
F.C.
Recurrent F.C :
first FC in <1yr
50 %
first FC in >1yr
28 %
10 % of children have 3 or more recurrence
•
•
•
•
•
Febrile Seizures
Fever of over 38.5C (even 37.8)
Age range of 6 mo to 7 yr
No infection of the CNS or electrolyte abnormality
No previous non-febrile seizure or neonatal seizure
Simple:
– Generalized
– Less than 15 minutes
– One in 24h
• Complex:
– Focal
– Over 15 minutes
– More than one in 24h
– Focal neurologic sign in postictal state
Risk factors of recurrence of FC
• Positive family history of
FC
prophylaxis of recurrent
• FC in < 1 yr
FC:
• Seizure with
Oral diazepam at the
T< 40 degree
onset of each febrile
illness
• Seizure with
fever <1 hour
Prolong anticonvulsant
: pb or Nav
• Complex FC
Risk of epilepsy in FC
 Abnormal neurologic examination or
development
 Positive family history of epilepsy
 Complex FC
 FC in < 1 yr
 Recurrent FC
 Seizure with T< 40 degree
prophylaxis :
Prolong anticonvulsant : pb or Nav
‫‪Meningitis AND FC‬‬
‫ا نديكاسيون ا نجام ‪LP‬‬
‫‪ .1‬تشنج با تب در زير يك سال‬
‫‪ .2‬وجود لتارژي پرسيستانت‬
‫‪.3‬اولين تشنج تب كمپلكس‬
‫‪ .4‬در بچه ايي كه قبال آنتي‬
‫بيوتيك گرفته و امكان پيگيري‬
‫نباشد‪.‬‬
‫ريسك فاكتورهاي احتمال‬
‫مننژيت‬
‫‪ .1‬ويزيت در ‪ 48‬ساعت گذشته‬
‫‪ .2‬تشنج در بدوورود به‬
‫اورژانس‬
‫‪ .3‬تشنج فوكال‬
‫‪ .4‬معاينه عصبي غير طبيعي‬
Routine laboratory testing in epileptic patients
 Routine laboratory testing is not cost effective or
necessary with the exception of felbamate, which is
associated with a relatively high risk of aplastic anemia
and requires close laboratory monitoring.
 Vomiting (symptom of hepatotoxicity or pancreatitis),
prolonged unexplained fever, easy bruising, extreme
fatigue or lethargy, flu-like symptoms, worsening of
seizures, change in mental status, and abdominal pain
should lead to further investigations
 Many idiosyncratic reactions of AEDs (StevensJohnson syndrome, TEN, serum sickness , pancreatitis)
are not predicted by presymptomatic blood test
abnormalities
 Patients taking AEDs should be monitored for
emergence or worsening of suicidal ideation or
depression.
Adverse effects of new AEDs
AED
Serious Adverse Events
Nonserious Adverse
Events
Gabapentin
None
Weight gain,
peripheral edema,
behavioral changes*
Lamotrigine
Rash, including Stevens
Tics* and insomnia
Johnson and toxic epidermal
necrolysis (increased risk for
children, also more common
with concomitant valproate
use and reduced with slow
titration); hypersensitivity
reactions, including risk of
hepatic and renal failure, DIC,
and arthritis
AED
Serious Adverse Events
Nonserious
Adverse Events
Levetiracetam
None
Irritability
behavior change
Oxcarbazepine
Hyponatremia (more
common in elderly), rash
None
Tiagabine
Stupor or spike wave stupor
Weakness
Topiramate
Nephrolithiasis, open angle
glaucoma, hypohidrosis
(predominantly children)
Metabolic acidosis,
weight loss,
language
dysfunction
Zonisamide
Rash, renal calculi,
hypohidrosis (predominantly
children)
Irritability,
photosensitivity,
weight loss
Status
epilepticus
Definition :as a seizure that lasts for 30 minutes
or longer or is repeated frequently so as
consciousness not regained between seizures
 Seizure lasting more than five minutes has a
high risk of lasting  30 minutes and treatment
delay is associated with delayed treatment
response.
 In 1.3 to 16% of epileptic patients.
 Mortality: 2.7 - 20 percent
SE occurs in 1.3 to 16% of epileptic patients
 Mortality: 2.7 - 20 percent
 Incidence rates, causes, and prognosis vary
substantially by age
 Most in the first year of life
 Febrile SE is the most common etiology
 60 % of children are neurologically healthy
prior to the first episode of SE.

•
•
•
•
•
.
What the common complications of status
epilepticus ?
Reduction of CPP
Hyper/hypotension
Dysrhythmeia, CHF, Apnea, Aspiration
Non cardiogenic pulmonary edema,
Rhabdomyolysis,
Hypo/hyperglycemia
Status epilepticus classification




Clinical
Simple partial
Complex partial
Generalized convulsive
Generalized non-convulsive
Etiologic
• Symptomatic ( acute or remote)
• Idiopathic
• Reactive : Prolong FC
Subtypes of status epilepticus
• Symptomatic :
- acute brain injury : ( 25 % of children )
CNS infections , electrolyte disorder, acute
anoxia
congenital malformation or previous brain
injury ( in 10 % of children )
-other : hypoglycemia , hypocalcemia,
poisoning,Reye ,lead, frontal tumor,…
• Prolonged FC: most common cause in <3yr
• Idiopathic : sudden cessation of AED
Pathophysiology :
excessive
excitatory neurotransmitters :
glutamate , aspartate , acetylcholine
Ineffectiveness of inhibition : GABA
Neuronal loss with every episode,
particularly if prolonged
Complication:










Hypoxemia and acidosis
Hyperglycemia & hypoglycemia
Hyper tension & hypotension
IICP and cerebral herniation
Hyperpyrexia
Hyperkalemia
Myoglobinuria
Non cardiogenic pulmonary edema
Leukocytosis(60%)
CSF pleocytosis(13%)
•
•
•
•
Risk Factors
Partial seizures with clustering
Focal background EEG abnormalities
Secondary generalized Partial seizures
Generalized abnormalities on
neuroimaging
• Younger age of epilepsy (1 year or less) at
onset
• Symptomatic etiology of epilepsy
• Occurrence of SE as the first seizure
Management
Assess respiratory & circulatory status &
maintenance of an adequate airway and o2
therapy , cardiac & BP monitoring, pulse
oximetry
 A rapid history & neurologic exam: to
determine classification & etiology
 Establishment of IV line and obtain blood
samples : CBC, glucose , Ca ,P , Mg , Cr, ABG,
LFT, Na, K .
Toxic and metabolic screens ,AED level, blood
culture,…in some patients
 Correction of metabolic abnormalities:
hypoglycemia

Management
 Neuroimaging: after stabilization of patient ,
especially in a patient with focal neurologic signs
 EEG for background activity evaluation : as
soon as possible after the seizure stops, ideally in
1-2 hour
 If the patient has not regained a relatively
normal mental state within a few hours after SE
has stopped, an EEG should be performed to
evaluate the possibility of subclinical seizures.
Drugs:
• Oral or IM medication (Pb, Pht, VPA ,
OXCBZ , Levetiracetam) : in partial seizures
or brief generalized motor seizures have
stopped before the child arrives in the
emergency department, which are short in
duration, or the child is conscious despite
multiple seizures.
• If IV access is delayed or impossible, many
AEDs can be given by alternative routes :
interosseous , IM, rectal , intranasal
Missed medication
 Paradoxical effects
 Nonprescription medications : over-the-counter
drugs , illicit substances, herbal preparations
 Some AEDs commonly used to treat SE may
worsen seizures caused by illicit drugs.
In SE is induced by cocaine, other local anesthetics,
theophyliine , lindane treatment with
benzodiazepines is recommended ( not phenytoin )

Benzodiazepines:
Drugs:
Diazepam : 0.3mg/kg, 2mg/min, Max :10mg
 duration of anticonvulsant effect <20 minutes
 first choice in outside of emergency department
 is not considered first-line therapy for SE(less efficacy and
more respiratory depression)
 Lorazepam :
 Effective duration of action : 4-6 hours
 IV-rectal , 0.5 - 2 mg/min , Max: 4mg
<12 yr : 0.1 mg/kg >12yr : 0.05 mg/kg
 Midazolam :
 IV, IM, rectal , nasal ,oral
 0.15-0.2mg/kg
Antiepileptic Drugs
Phenytoin: IV,15-30 mg/kg each time 10mg/kg , 1mg/kg/min
or
Fosphenytoin (IV-IM,15-20 mg/kg , 3mg/kg/min) with
monitoring of HR and BP and avoiding of venous
extravasations
Side effects :
hypotension ,arrhythmias, local pain and injury, venous
thrombosis and purple glove syndrome (edema,
discoloration ,and pain in distal extremity to the site of
infusion)
Phenobarbital : IV-IM-SC, 15-20mg/kg , 2mg/kg/min
Side effects : sedation, respiratory depression
IV
Lidocaine: (1 - 2 mg/kg ,once or twice) is
one of the second-line drugs in SE treatment
with favorable properties of prompt
responses, less alteration of consciousness and
respiratory depression

Valproic acid : 20-40 mg/kg IV(diluted 1:1
with normal saline or DW 5% ) over 5 to 10
minutes can be used as a second or third-line
treatment.
Refractory status epilepticus
is
defined as ongoing seizures despite the use of
two first-line drugs, usually a benzodiazepine
plus either phenytoin or phenobarbital, or
which continues for > 60-90 minutes in spite of
adequate treatment
 Admit in P-ICU
 treatment of cerebral edema
Drugs in refractory SE
• Pentobarbital : 5-15 mg/kg stat , and 0.5-5 mg/kg/h in
stable hemodynamic
Side effects: hypotension , pulmonary edema, ileus,
delayed neurologic recovery
• Midazolam:0/15-0/2mg/kg stat, 1-5 µg/ kg /min
• Propofol:1-3mg/kg stat, 2-10mg/kg/h , should not be
used in children on the ketogenic diet
Adverse effects:
bradycardia , apnea, hypotension ,
hypertriglyceridemia , acidosis , rhabdomyolysis
• IV valproic acid : 15-20mg/kg in 1-5 min, 5mg/kg/h
• lidocaine drip
• Paraldehyde
• General anesthesia
• Topiramate
Neonatal seizures
• 1.subtle :the most common forms
apnea, eye deviation ,tongue thrusting, eye
blinking,staring,bicycling,fluctuation of vital
sign, sialorrhea,pedaling movements
• 2.Focal or generalized tonic :sustained
posturing of limbs or trunk
• 3.focal or multifocal clonic: repetitive,rhythmic
contraction of muscles of limbs,face, or trunk
• 4. Focal or generalized myoclonic:
arrhythmic contraction of muscles of limbs,
face, or trunk
Etiology of neonatal seizures
• HIE :the most common
cause in full term,
occur 12- 24 hours
after birth asphyxia
• Hypoglycemia
• Hypocalcemia
• Hypomagnesemia
• Hypo or hypernatremia
• Congenital brain
malformation
• IVH :between 1 and 3
days of age in preterm
• Vit B6 dependency
• Injection of local
anesthetic agents into
fetal scalp (transient
bradycardia,fixed
mydriasis) in lab. room
• Sepsis:after 5 days
• Drug withdrawal
• IEM :lethargy,acidosis,
FH of infant death
• SAH: sudden onset on
days1-3,short duration
,do not recur
Benign idiopathic neonatal seizure
or fifth day fits
•
•
•
•
•
•
In 5 % of fullterm neonate
Seizure in 4- 6 days
Multifocal clonic seizure
Duration of seizures : 24 hours
Prognosis : good
Mental development : normal
Benign familial neonatal seizure
• AD , choromosom 20
• Generalized clonic seizures occurring
toward the end of first week of life (2-3 )
• 10- 20 times in a day
• Outlook : favorable
• Seizures stop in 6 months of age.
• Response to treat is variable.
Differentiate of seizure from
jitteriness or tremulousness:
• Jit are sensory dependent, elicited by
stimuli,interrupted by holding the limb
• Jit is fine &rapid, seizure is coarse,fast and
slow clonic activity
• Abnormal eye movement in seizure
• Autonomic abnormality ( increased in BP or
PR ) in seizure
• Jit ocurr in crying or examining
jit
: IDM, after asphyxia,infants with narcotic
withdrawal
Treatment of neonatal seizure
• Treat of specific cause:
Hypoglycemia Hypocalcemia,
Hypomagnesemia, hypo or hypernatremia and
B6 deficiency
• In the absence of an indentifiable cause ,pb 20
– 40 mg/Kg and then dilantin 10 -20 mg/Kg od
diazepam 0.1- 0.2 mg/Kg followed by one of the
two longer- acting drugs
• Prognosis : dependent to underlying cause
Imitators of epilepsy :
Nonepileptic paroxysmal disorders
Paroxysmal disorders
 Abrupt onset of a clinical episode that
tends to be stereotyped and repetitive ,
lasts seconds or minutes (rarely hours) ,
and ends abruptly
 25 % of patients referred to epilepsy
clinic don't have epilepsy & recognition
is important:
 to avoid unnecessary treatment
 to institute the correct treatment when required
‫سواالت کمک کننده‬
‫‪‬آيا هميشه اين حمالت بدنبال شرايط خاصي نظير گريه کردن ‪،‬‬
‫سرفه ‪ ،‬ورزش ‪ ،‬غذاخوردن ‪ ،‬خواب و ‪ ...‬صورت می گيرد؟‬
‫‪‬آيا هوشياری کودک در زمان بروز اين حمالت مختل می شود و‬
‫يا اينکه طفل آن را کامال به ياد می آورد ؟‬
‫‪‬آيا رنگ کودک در حين اين حمالت ‪،‬تغيير می کند؟‬
‫‪‬آيا چشمها در جهت خاصی حرکت می کنند ؟‬
‫‪‬کدام قسمت از بدن بيش از ساير قسمت ها درگير می شود؟‬
‫‪‬آيا کودک‪ ،‬می تواند از بروز اين حاالت جلوگيری کند ؟‬
‫‪‬آيا والدين می توانند اين حمالت را نزد پزشک‪ ،‬شبيه سازی کنند ؟‬
‫‪‬آيا سابقه فاميلی مثبت از اين حمالت در خانواده وجود دارد؟‬
‫‪ ‬مشاهده فيلم گرفته شده زمان بروز حاالت‬
Nonepileptic paroxysmal events
Neonate :
Infants :
 Apnea
Syncope (BHS ,…)
 Benign myoclonus of infancy
 Shuddering attacks
 Sandifer syndrome
 Paroxysmal torticollis of infancy
 Extrapyamidal drug reaction
 Benign paroxysmal vertigo
 Spasmus nutans
 Opsoclonus-myoclonus
 Masturbation
 Jitteriness
 Sleep myoclonus
 Hyperkplexia
Nonepileptic paroxysmal events
Children
Adolescents and young adults
 BHS
 Vasovagal syncope
 Migraine
 Vasovagal syncope
 Migraine
 Daydreaming
 Tic disorders
 Sleep disorders
 Paroxysmal dyskinesia
 Tic disorders
 Hemifacial spasm
 Stiff person syndrome
 Pseudoseizures
 Episodic rage
 Staring spells
 Sleep disorders
 BPV
 Masturbation
 Movement disorder
In neonate
 Apneic seizure : if is accompanied by abnormal eye
movement, mouth movement , in BP or HR
 Jitteriness :
 elicited by stimuli, interrupted by holding the limb
 Jit is fine &rapid, seizure is coarse, fast and slow clonic
activity
 Abnormal eye movement in seizure
 Autonomic abnormality in seizure
 Jit occur in crying or examining
Severe jitteriness :
IDM, HIE, narcotic withdrawal, hypocalcemia,
hypoglycemia
Benign neonatal sleep myoclonus
 Bilateral, symmetric myoclonic jerks of the arms
and/or legs, during non-REM sleep in the first few
weeks of life and resolve by 2-3months
Absence of autonomic disturbances
EEG , neurologic exam & development : normal
 Sleep myoclonus will cease when the baby is
aroused
 Neonates with severe cerebral dysfunction can also
have myoclonic jerks, but these typically occur
with a stimulus, or upon wakening or falling asleep
Syncope
An abrupt loss of consciousness due to sudden  in
cerebral perfusion ( brief tonic contraction in muscles
of face , trunk, extremities in 50 % or UWG )
Causes : vasovagal (painful procedures, blood drawing
warm setting),cardiac arrhythmia, HV & panic attack
, Reflex syncope (micturition or cough, carotid sinus
hypersensitivity)
 Predisposing situation , accompanying pallor, nausa
, perspiration , prodromal lightheadedness and visual
changes, lack of postictal state , short duration
 EEG : transient slowing in attack , CPK in seizure
 Tilt table testing can provoke vasovagal syncope
Breath-holding spells
 In 5 % of children, rare in <6mo and >5-8 yr
80% <18mo , all in <3yr
 Iron deficiency is more prevalent in BHS
 A positive FH is present in 20-35%
 An autosomal dominant trait in some families
 The two clinical types of BHS:
 Cyanotic
 Pallid
 Family members and individual children can
demonstrate both types, usually one predominates
Cyanotic BHS
Crying, prolonged expiratory apnea, cyanosis, UWG,
followed by limpness and loss of consciousness
 Decorticate or decerebrate posture in prolong BHS
 A few children have generalized tonic or clonic
seizures , prolonged postictal unconsciousness
15 -25 % have multiple episodes daily ,most children
have one to six spells per week
 Treat : reassurance
 Ferrous sulphate :5-6 mg/kg/ day
 Piracetam, a GABA-derivative
Pallid BHS
It is less common than the cyanotic variety
 The event is caused by cardiac bradycardia
 Occur after a minor fall or blow to the head or
upper body, then child stops breathing and
becomes pale, diaphoretic, and limp
 May followed by opisthotonos, incontinence &
low amplitude clonus , confusion or sleepy for
several minutes afterward.
 Treat : in severe attacks with prolonged, severe
bradycardia or asystole, atropine and cardiac
pacing

Benign myoclonus of infancy
Known as benign nonepileptic infantile spasms
 Clusters of spasms at mealtime at 3-8 months
 Clusters increase in intensity and severity over
weeks or months and then remit spontaneously at
two to three years of age
 EEG, neurologic exam , development : normal
 An anticonvulsant is not indicated
 Without subsequent epilepsy

Masturbation
 In girls ( 2 mo – 3 yr ) , in boredom or stress
 Episodes of tonic posturing with copulatory
movements without manual stimulation of genitalia
 Suddenly flushed & perspires , may grunt & breathe
irregularly without loss of consciousness
 Persists for a few minutes ( rarely hours )
 Search for evidence of sexual abuse or abnormality
of perineum
 Subside by 3 yr of age
 No specific therapy is required
Sandifer syndrome
Intermittent paroxysmal spells of generalized
stiffening & opisthotonic posturing with feedings
(within 30 minutes following a meal) in infants
due to gastroesophageal reflux
 These spells may be associated with apnea,
staring, and minimal jerking of the extremities
 Can be seen in normal children or children with
hypotonia and tracheomalacia
 Treatment of GER reduces the frequency and
severity of attacks.

Benign paroxysmal torticollis in infancy
Periodically, head tilts to one side, with the face
rotated toward the opposite side with pallor ,
agitation & vomiting , begin and end suddenly,
with a duration between a few hours and a few
days. The child is alert and responsive during an
attack.
 Episodes usually first occur in the first 3 months of
life and resolving by the age of 3 years
 EEG, neurologic exam , development : normal,
 A FH of migraines is common , may develop
migraine later in life
Benign paroxysmal vertigo (BPV)
 In toddlers ,sudden attacks of ataxia, ± nystagmus,
diaphoresis, nausea, and vomiting , without altered
consciousness, the child appears frightened and pale
 Episodes : <1minute,clusters: daily for several days
then remitting for several weeks & recurring again
 Usually resolves by 3-5yr, may develop migraine
several years later
 BPV remains a diagnosis of exclusion.
 Treat in clusters of attacks :
Diphenhydramine 5mg/kg/day PO, IM ,IV, rectal
Shuddering attacks
Brief episodes a rapid tremor of the head, shoulder,
and trunk as "shiver" from a chill in 4-6 mo
 Episodes last a few seconds, can occur multiple
times a day (100 times ) and occur with feeding ,or
when child is excited or distressed
 They never occur during sleep and virtually never
when being held and cuddled
 EEG ,Neurologic exam , development: normal
 FH of benign essential tremor exists
 The spells spontaneously resolve by the second
decade without treatment
Nonepileptic staring spells or pseudoabsences
 In MR, ADHD, autism & in normal children
 Spells in boredom or inactive (watching TV or
sitting in a class)
 Staring interrupted by tactile or vocal stimulation
(but usually not hand-waving )
 HV in young children :blow out imaginary candles
 In HV , Video-EEG show generalized HVSW in the
healthy child, but no epileptiform activity.
An inexperienced electroencephalographer may
confuse this striking slow activity, "build-up" with
the paroxysmal activity of an absence seizure.
Dystonia
 An abnormal posture due to sustained
contraction of both the agonist and antagonist
muscle groups
 A dystonic posture may be generalized or focal:
examples include opisthotonic posturing,
torticollis, oculogyric crisis , facial spasm
 A common etiology of dystonia in infants is an
acute reaction to drugs such as :
metoclopramide ,phenothiazines , haloperidol
 In infants : generalized
 In children: face and trunk