Paroxysmal disorders: Abrupt onset of a clinical episode that tends to be stereotyped and repetitive , 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:

• Seizure disorders• Migraine and variants :

Paroxysmal torticollis , Paroxysmal vertigo (benign)• Syncope and vasovagal events : Breach-holding spells• Transient ischemic attack• Metabolic disorders : Hypoglycemia• Sleep disorders :Narcolepsy, Cataplexy , Night terrors• Paroxysmal dystonia or choreoathetosis• Shudder attacks• Pseudoseizures

Seizures occur 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

• Provoking 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 syndrome• West syndrome• Lennox-Gastaut syndrome• BPEC• LKS• Benign neonatal

convulsion

Clinical Seizure classification

Partial (Only a portionof the brain)

- Simple(Normal consciousness)- Complex

(Impaired consciousness)

Generalized(Both hemispheres areinvolved)

Type of Seizure

When partial seizures spread to involve the whole brain and produce a generalized tonic-clonic seizure, they show secondary generalization or Jacksonian seizures .

Partial or 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

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 symptom logy.

• Spread of partial seizure to the whole brain produced GTCS(secondary generalization )

• Only psychic or autonomic symptoms can be difficult to diagnosis.

SPS : 10 -20 sec• Uncinate seizure : ( تمپورا نامطبوع احساسبوي

) مياني ل• Gelastic seizure : spells of uncontrolled laugher

hypothalamic tumors• Versive seizure : يك به چشم و انحرافسرسمت

• 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.

Treatment of generalized Seizures GTCS: First choice : valproate Second : CBZ , PHT , TPM , LMC , Pb CLZ , PRM , Zonisamide Absence :First choice :Valproate , ethoxysuximide , LMCSecond choice : TPM, Levetiracetam , ZoniMyoclonic : 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 finial secondary generalized.

• Good prognosis and normal CT,IQ,N.E• EEG :spike in centrotemporal• Treat: CBZ in frequent seizures for 2 yr

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 environment awareness and staring 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 CPS3. Absence provoked by hyperventilation4. CPS last few minutes ,absence :few sec5. absence :dozens per day but patients with CPS

rarely have more than one or two seizures in day

6. 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

1. Epileptic: EEG shows epileptiform discharges during the jerk

2. Nonepileptic : may originate in the B.G ,BS, or spinal cord - Benign: sleep myoclonus

- Serious pathology

The underlying illness producing myoclonic

epilepsy may be developmental and static or progressive

and associated with neurologic deterioration (neuronal

Ceroid lipofuscinosis).

Juvenile myoclonic epilepsy or Janz syndrome :

• Onset age : 12-16 yr (adolescent )• AD ( chromosome 6)

• Myoclonus in the morning (predominantly within 90 minutes of awakening )with or without generalized clonic and absence seizure

• N.E : 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 , prednisolone ( irritability ,

swelling ,hypertension , glycosuria, severe infection ) benzodiazepines , valproate , vigabatrin (sabrile )

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 seizuresPerinatal 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 :

،آنتي دپرسانت ،آنتي آمينوفيلين ، آمفتامين ، ناركوتيك ، ليتيوم كولينرژيك،فنوتيازين،ايزونيازيد ساليسيالت

Etiology of seizuresNeurocutaneous syndromes :

Tuberous sclerosis , NF,… Systemic disorders : vasculitis ( CNS or systemic ) SLE

hypertensive encephalopathy

renal failure hepatic encephalopathy

Infection: مغزي آبسه ، 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 seizureFocal seizure focal EEGFocal neurologic finding Neurodevelopmental delayDysmorphic 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 (chromosome 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 for recurrence of FCMajor :

1. Age <1 yr

2. Duration of fever <24 hr

3. Fever 38-39

Minor:

1. Family history of FC

2.Family history of epilepsy

3.Complex febrile seizure

4. Day care

5. Male gender

6. Lower serum sodium

Having no risk factors carries a recurrence risk of about 12%;

1 risk factor, 25-50%

2 risk factors, 50-59%

3 or more, 73-100%

prophylaxis of recurrent FC:Oral diazepam at the

onset of each febrile illness

Prolong anticonvulsant pb or Nav

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

Risk factorRisk for subsequent epilepsy

Simple febrile seizure1%

Neurodevelopmental abnormalities33%

Focal complex FC29%

Family history of epilepsy18%

Fever <1 hr before febrile seizure11%

Complex febrile seizure, any type6%

Recurrent febrile seizures4%

Risk factors for occurrence of subsequent epilepsy

Meningitis AND FC

مننژيت احتمال فاكتورهاي ريسكدر. 1 گذشته 48ويزيت ساعتاورژانس. 2 به بدوورود در تشنجفوكال. 3 تشنجطبيعي. 4 غير عصبي معاينه

انجام LPانديكاسيونسال. 1 يك زير در تب با تشنجپرسيستانت. 2 لتارژي وجودكمپلكس. 3 تب تشنج اولينامكان. 4 و گرفته بيوتيك آنتي قبال كه ايي بچه در

نباشد .پيگيري

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 )Stevens- Johnson 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.

Status epilepticus: Generalized tonic-clonic activity lasting longer than

20 minutes, or repeated seizures without restoration of consciousness for more than 30 minutes, is defined as status epilepticus and may lead to irreversible brain injury.

Complications of SE:• Reduction of CPP• Hyper/hypotension• Dysrhythmia, CHF, Apnea, Aspiration• Non cardiogenic pulmonary edema, Rhabdomyolysis,• Hypo/hyperglycemia

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

Management of status epilepticus• Open the airway / administer oxygen, if

necessary intubate

• Check the circulation

• Obtain vital signs

• Insert NG tube & IV line

• Laboratory evaluation : CBC , glucose, Ca, Mg, P, BUN, Cr, lactate other per case

• IV Anticonvulsant medications

Drugs in SE1.Benzodiazepine: max rate of administration :1 mg/min

Diazepam :

IV: 0.2-0.4 mg/Kg (up to 10mg) ,Rectal : 0.3 -0.5 mg/Kg

Midazolam 0.1-0.2 mg/kg

Lorazepam 0.05 – 0.1 mg/Kg IV

2. Phenytoin :10- 20 mg/Kg (up to 1- 1.5 g) IV at a rate 1mg/Kg/m in with ECG & heart rate monitoring

3. Phenobarbital :10 -20 mg/Kg IV at a rate of 1mg/Kg/min

4. Valproic acid : 20 mg/kg

Then: Continuous drip of diazepam, midazolam , pb and general anesthesia

Neonatal seizures1.Subtle :the most common form

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• Hypomagnesaemia• Hypo or hypernatremia• Congenital brain

malformation• IVH :between 1 and 3 days

of age in preterm

• 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 full term neonate

• Seizure in 4- 6 days

• Multifocal clonic seizure

• Duration of seizures : 24 hours

• Prognosis : good

• Mental development : normal

Benign familial neonatal seizure• AD , chromosome 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 occur in crying or examining Jit : IDM, after asphyxia, infants with narcotic withdrawal

Treatment of neonatal seizure• Treat of specific cause:

Hypoglycemia , Hypocalcemia , Hypomagnesaemia,hypo or hypernatremia , B6 deficiency

• In the absence of an identifiable cause , pb 20 – 40 mg/Kg and then dilantin 10 -20 mg/Kg or diazepam 0.1- 0.2 mg/Kg followed by one of the two longer- acting drugs

• Prognosis : dependent to underlying cause