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SEIZURES IN CHILDREN Rashmi Kumar Prof & Head, Pediatrics King George Medical University Lucknow
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Prevalence Definition Conditions that mimic seizures Pathophysiology Etiology Age wise etiology Classification Assessment Febrile seizures Management
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SEIZURES One of the most common life threatening events in childhood, more than adults Paroxysmal electrical activity in brain --> motor/sensory/autonomic disturbance with /without alteration of consciousness Convulsion – seizure with motor activity 5% Epilepsy – recurrent (2 or more) unprovoked seizures beyond newborn period 0.5%
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Seizures: DDx Tremors –distal, rhythmic, equal amplitude, no loss of consciousness Jitteriness Breath holding spells –always after crying, sequence of events important Syncope – after prolonged standing/emotional upset, gradual loss of consciousness, slow pulse, pallor, sweating, improves in supine/head down position Pseudoseizures – older girl, never hurts herself, bizarre movements, normal s Prolactin Detailed sequence of events necessary – HISTORY, HISTORY, HISTORY
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Seizures: Pathophysiology: Sustained partial depolarisation in a group of neurons -->excitability --> sudden depolarisation in response to stimuli -->conduction to surrounding cells, distant synaptically connected cells & subcortical neurons -->dissemination -->loss of consciousness
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SEIZURES - ETIOLOGY 1st fit/ recurrent fits I Symptomatic Infectious/ post infectious (including granulomas) Anoxic/post anoxic Vascular Trauma/post traumatic Tumour Congenital - porencephaly, lissencephaly, agenesis of corpus callosum, neurocutaneous syndromes Degenerative Metabolic - hypocalcemia/hypomagnesemia hypo/hypernatremia hypoglycemia pyridoxine deficiency Inborn errors Drugs/Toxins -aminophylline,antihistamines,steroids,phenothiazines, hexachlorophene, strychnine, camphor, INH, tetanus, lead, shigella/salmonella Acute cerebral edema - Hypertension Febrile II Idiopathic
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Newborn 1-6 mths 6m-3 yrs >3 yrs Birth asphyxia/trauma birth asphyxia Febrile idiopathic IVH cranial malformations CNS infections Hypocal/hypoglyc inborn errors IU infections IU infections Degenerative Meningitis metabolic Tetanus tumour Inborn errors other Kernicterus Polycythemia Narcotic withdrawal
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CLASSIFICATION OF EPILEPTIC SEIZURES: ILAE 1981 I Partial 54% Simple - motor/sensory/autonomic 7.7% Complex 35.5% Partial with secondary generalization 56.4% II Generalised 40.4% Tonic clonic 69% Absence 3% Myoclonic 20.5% Tonic 4.1% Atonic 3.1% III Unclassifiable 6% (hospital based study in Mumbai) However, same patient can have more than 1 type Many patients show a distinct evolution of disease
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CLASSIFICATION OF EPILEPTIC SYNDROMES : ILAE 1989 I Localisation related Symptomatic Cryptogenic Idiopathic II Generalised Idiopathic Cryptogenic West syndrome Lennox Gastaut syndrome epilepsy with myoclonic astatic seizures epilepsy with myoclonic absences Symptomatic Non specific specific III Epilepsies undetermined whether focal or generalised IV Special syndromes CLASSIFICATION OF EPILEPSY STILL EVOLVING
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EPILEPSY - SPECIAL TYPES: GTCS: v common Aura  tonic spasm loss of consciousness  fall  clonic movements Rolling of eyeballs/Frothing at mouth/Distortion of face Incontinence/ Jerky breathing Post ictal sleep
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Absence epilepsy 2-4% of childhood idiopathic epilepsy Girls 3-7 yrs, normal IQ Transient loss of consciousness for few secs No loss of tone Ppted by hyperventilation - Treatment – Ethosuximide, valproate May develop GTCS EEG - 3/sec spike & wave activity
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EPILEPSY - SPECIAL TYPES: Infantile spasms: Onset in 1st year Sudden flexion/extension in series esp on awakening Upto 100 times /day 60% secondary, 30% cryptogenic Treatment - ACTH/steroids/ vigabatrin Associated with mental regression EEG - hypsarrhythmic May develop GTCS Lennox Gastaut: 1-8 yrs, tonic/atonic/absence type EEG - diffuse 2 Hz spike-waves Very difficult to control
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EPILEPSY - SPECIAL TYPES: Psychomotor (Temporal lobe) seizures: Complex partial seizures with origin in temporal lobe. Purposeful but inappropriate acts 'automatisms' Associated with behavioral problems Difficult to diagnose or treat. Benign epilepsy with centrotemporal spikes: Partial, idiopathic, orofacial/hemifacial, 3-13 yrs, often during sleep. Easy to control Myoclonic: heterogenous, multiple causes Juvenile myoclonic: myoclonic jerks esp after awakening EEG - 4-6 Hz polyspike, photosensitivity, GTCS may occur Good response to Valproate
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FEBRILE SEIZURES: 2-4% of children 3m - 5 yr age Assn with fever due to extracranial infection Generalised, Short lasting, only one sz per illness No mental/neurological/EEG abnormality Typical vs Atypical (complex) Focal Prolonged >1 seizure during illness 1/3 have at least 1 recurrence 1/6 have multiple recurrences Risk of epilepsy: Fh/o epilepsy Atypical Abnormal neurologic/mental status
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Febrile Seizures: Management Exclude CNS infection Control fever Look for & treat cause of fever Rectal diazepam Explain to parents, reassure If multiple - intermittent oral diazepam   by 80% If high risk for epilepsy  long term phenobarb/valproate.
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Seizures: ASSESSMENT History: 1st seizure/ recurrent seizures Fever Precipitating factors – diarrhea/ vomiting/ drug/ toxin/ metabolic Headache/vomiting/visual loss Duration Age at onset No of attacks Frequency /, change in seizure type, last seizure when? Exact description Aura partial/generalised onset Loss of consciousness Tonic/clonic phase Associated events - bed wetting/fall/tongue bite Duration Post ictal Precipitating factors Diurnal Family history Antecedant events - trauma/CNS infection/asphyxia Personality change/intellectual deterioration Failure to thrive Developmental milestones Treatment
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Seizures: ASSESSMENT Examination: BP Head circumference Skin lesions Facial features Organomegaly Fundus Meningeal signs Neurological deficit Development
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Seizures: Investigations If features of CNS infection - CSF examination Glucose, Ca, Mg - low yield Skull Xray - calcification/  ICT - low yield EEG: Always diagnostic during a seizure Interictal record : normal in 40-50% of epileptics (spikes/sharp waves & spikes –slow wave complexes)  yield with sleep, sleep deprivation, hyperventilation, photic stimulation 2-10% normal population may have epileptic changes EEG indicated in all cases of epilepsy for: -confirmation of diagnosis & syndrome -type of seizures - absence vs temporal lobe, primary generalised vs secondarily generalised -presence of underlying lesion/ idiopathic vs symptomatic -follow up -before withdrawal of AEDs -localisation of focus before surgery Video EEG
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Seizures: Imaging - CT/MRI Has revolutionised the management of epilepsy Indications: focal features on exam, EEG Features of  ICT Intractable However, now indicated in every case with unknown cause Not necessary in febrile/absence/BETS/ JME etc. Western studies - 30% abnormal (30-50% of focal) -only 3% treatable Indian studies: Very high prevalence of granuloma like lesions –recent onset partial seizures in child/young adult 40% abn even after 1st seizure  indicated in every case
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MCQ The following are features of benign (typical) febrile seizures except: They are short lasting They are always generalised They only occur within 4 hours of fever onset They do not recur in the same febrile illness
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The typical EEG pattern in absence epilepsy is: Intermittent spike and slow waves Hypsarrythmia Burst suppression 3 per second spike and waves
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The following is true about absence epilepsy It occurs more commonly in boys There is loss of tone It is precipitated by hyperventilation Imaging is usually abnormal
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Definition of epilepsy includes: At least 3 seizures EEG is abnormal Imaging is abnormal Beyond neonatal period
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The following is true about breath holding spells: It is usually preceded by crying Child is always blue There is no loss of consciousness EEG may show spikes
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The following is true about infantile spasms except: They occur in clusters They may appear like ‘startling’ They usually occur during sleep They are also called ‘salaam attacks’
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West syndrome usually has the following features except: Infantile spasms Onset in newborn period Hypsarrythmia on EEG Psychomotor retardation or regression
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Imaging in seizures is not indicated in: Generalised tonic clonic seizures Absence seizures Temporal lobe seizures Infantile spasms
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Prevention of febrile seizures can be achieved by: Intermittent phenobarb Long term phenytoin Intermittent diazepam Long term carbamazepine
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Emergency dose of IV diazepam for seizure control is: 1 mg/kg 0.5 mg/kg 0.1 mg/kg 0.3 mg/kg
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Seizures - Management I Management of acute attack: Calm down Head down lateral position Prevent hurt If does'nt stop convulsing in 3-5 min, Inj Diazepam 0.3 mg/kg slow iv bolus Maybe repeated after 20 min Effect lasts 0.5-3 hrs SE- hypotension, respiratory depression, secretions or Rectal diazepam 0.5 mg/kg dose/ nasal midzolam 0.2 mg/kg/dose
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Domiciliary Mx Rectal Diazepam 0.5 mg/kg Intranasal midzolam 0.2 mg/kg
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Seizures: Status epilepticus: Prolonged seizure for >20 min or repeated seizures without regaining consciousness Persistent seizure activity  hypoxia, hypoglycemia, hyperthermia, cerebral edema & vasomotor instability Life threatening Risk of permanent brain damage  Medical emergency
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Mx of Status epilepticus ICU, monitoring IV dextrose drip Oxygen IV Inj Diazepam 0.3 mg/kg or Lorazepam 0.1 mg/kg (longer action) or Midzolam (lesser respiratory depression) Inj phenytoin 15-20 mg/kg iv at a rate of <1mk/kg/min Inj Phenobarbitone 20 mg/kg iv at a rate of 1 mg/kg/min or IV Valproate 20 mg/kg as infusion in 50 ml NS over 30 min Ventilatory support + diazepam/midzolam infusion `` Thiopental infusion
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LONG TERM MANAGEMENT OF EPILEPSY: I General advice: As normal a life style as possible No swimming/cycling on road/driving Inform teacher First aid Seizure dairy Regularity
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LONG TERM MANAGEMENT OF EPILEPSY: Drugs: When to start? If 2 or more seizures within a 12 month period Monotherapy: Start at lower limit & build up gradually till toxicity/control If no effect at maximum dose, taper off while introducing 2nd drug 4 first line drugs - Carbamazepine, phenytoin, valproate and phenobarbitone No drug completely safe 70% can be controlled
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First line AEDs Carbamazepine: Ind: Partial, tonic clonic Dose: 10-30 mg/kg/d in 2-3 doses13-18 hrs, Adv: Relatively safe, improves cognitive fn. SE: Diplopia,drowsiness, giddiness initially.Hepatitis, skin rash, BM depression, drug interactions, dystonia, can aggravate minor motor seizures
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First line AEDs Sodium valproate: Ind: Broad spectrum Dose: 20-30 mg/kg/d (upto 80) in 2-3 doses Half Life; 7-10 hrs SE: Nausea, vomiting, wt gain, hair loss, hepatic failure, tremors, platelets, s ammonia, s carnitine, no correlation between drug levels & toxicity, levels of other AEDs
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First line AEDs Phenobarbitone Ind: Tonic-clonic, partial, febrile Dose: 3-6 mg/kg/d as single doses level:10-15 g/ml20-80 hrs Adv: Cheap, once daily dose SE: Drowsiness, hyperkinesia, cognitive impairment ??, rash, rickets
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First line AEDs Diphenylhydantoin: Ind: Tonic-clonic, atonic, partia Dose: l4-8 mg/kg/d in 2 doses level: 10-20 g/ml Half Life: Upto 20 hrs SE: Hirsutism, gum hyperplasia, rickets, ataxia, lymphoma like syndrome, Sle like illness, megaloblastic anemia, rash, low margin of safety
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Ethosuximide: Ind: Absence seizures Dose: 20-25 mg/kg/d in 2 doses Half Life: 4-30 hrs SE: Photophobia, WBC, nephrosis, blood dyscrasia ACTH: Ind: West syndrome Dose: 20-40 u/d for 4-6 wks SE: hypercortisolism
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Nitrazepam Ind: Myoclonus, atypical absence Dose: 0.5 mg/kg/d in 2 doses SE: Sleepiness, salivation,hypotonia, ataxia, tolerance Clonazepam Dose: 0.05-0.25 mg/kg/d in 3 doses\ Drug level monitoring EEGs When to stop ? 2-3 yrs seizure free
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Newer AEDs Clobazam Ind: Partial, generalised & myoclonus (add on drug) Dose: 0.5 mg/kg/d single dose SE: Drowsiness, tolerance,  secretions Gabapentin Ind: Secondarily generalised, complex partial SE: liver enzymes, impaired swallowing & aspiration, somnolence, fatigue, dizziness, wt gain Lamotrigine Ind: Generalised, absence, JME, LG syndrome SE: Synergy with valproate, skin rash, SJ syndrome
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Newer AEDs/ Other modalities Topiramate: Ind: Partial, generalised, drop attacks, LG syndrome SE: ?cognitive impairment Vigabatrine: Ind: Partial, infantile spasms Dose: 40-80 mg/kg/d SE: Drowsiness, agitation, confusion Oxcarbazepine: Derivative of carbamazepine Ketogenic diet Surgery