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I. Neonatal Seizures

I. Neonatal Seizures. II. Conditions That Mimic Seizures. Seizure. transient and reversible alteration of behavior caused by a paroxysmal, abnormal and excessive neuronal discharge attack of cerebral origin sudden and transitory abnormal phenomena motor, sensory, autonomic, or psychic

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I. Neonatal Seizures

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  1. I. Neonatal Seizures II. Conditions That Mimic Seizures

  2. Seizure transientandreversiblealteration of behavior caused by a paroxysmal, abnormal and excessive neuronal discharge attack ofcerebralorigin sudden and transitory abnormal phenomenamotor, sensory, autonomic, or psychic transient dysfunction of part or all of the brain

  3. Epilepsy A paroxysmalbrain disorder of various etiologies characterized byrecurrent seizuresdue toexcessive electrical discharge of cerebral neuronsassociated with a variety of clinical and laboratory manifestations two or more seizuresnot directly provokedby intracranial infection, drug withdrawal, acute metabolic changes or fever

  4. Neonatal Seizures • Tonic Seizures—focal or generalized, may mimic decorticate or decerebrate posturing, primarily seen in preterms with intracranial hemorrhage & generally have poor prognosis • Subtle seizures • Consist of chewing motion, excessive salivation and alteration in respiratory rate including apnea, blinking, nystagmus, bicycling and pedaling movements, changes in color

  5. Clonic- focal (repetitive movements localized to a single limb) or multifocal (random migration of movements from limb to limb), consciousness may be preserved, primarily seen in term infants • Myoclonic- sudden flexor movements (lightning-like jerks), may be focal, multifocal or generalized, may occuring singly or in clusters, if due to early myoclonic encephalopathy it carries a poor prognosis. Brief focal or generalized jerks of the extremities or body that tend to involve distal muscle groups

  6. Why are seizure patterns in neonates more fragmentary than in older children? • The cellular organization of the mature and immature brain is different. The neonatal brain has incomplete glial proliferation, w/ continuing migration of neurons, establishing complex axonal & dendritic contacts and myelin deposition. The electrical discharges therefore spread incompletely and may remain localized to one hemisphere. The electrical discharges are slow to diffuse and bilateral synchronous discharges are rare.

  7. Neonatal SeizuresEEG Classification • Clinical seizure with consistent EEG event • Clinical seizure occurs in relationship to seizure activity • Includes focal clonic, focal tonic and myoclonic • Responds to antiepileptic drugs • Clinical seizure with inconsistent EEG event • Clinical seizures with no EEG abnormality • Seen in all generalized tonic and subtle seizures • Seen in patients who are comatose, HIE

  8. Neonatal SeizuresEEG Classification • Electrical seizures with absent clinical seizures • Electrical seizures associated with markedly abnormal background EEG • Seen in comatose patients

  9. Epileptic vs Non-epileptic Neonatal Phenomena

  10. Neonatal SeizuresEtiologic diagnosis • Hypoxic –ischemic encephalopathy • Metabolic • Infections • Trauma • Structural abnormalities • Hemorrhagic and embolic strokes • Maternal disturbances

  11. Causes of neonatal seizuresAges 1 – 4 days • HIE • Drug withdrawal • Dug toxicity • Lidocaine, penicillin • Intraventricular hemorrhage • Acute metabolic disorder • Hypocalcemia • Hypoglycemia • Inborn errors of metabolism

  12. Infection Metabolic disorders Hypocalcemia Diet Hypoglycemia Inherited disorder of metabolism such as galactosemia,fructosemia Hyperinsulinemic hypoglycemia Becwith syndrome Anterior pituitary hypoplasia Drug withdrawal Benign neonatal convulsion Kernicterus, hyperbilirubenemia Causes of neonatal seizuresAges 4 – 14 days

  13. Infection Head injury Subdural henatoma Inherited disorder of metabolism Aminoacidurias Urea cycle defects Organic acidurias Neonatal ALD Malformations of cortical development Lissencephaly Focal cortical dysplasia Tuberous sclerosis Sturge weber syndrome Causes of neonatal seizuresAges 2 – 8 weeks

  14. Neonatal SeizuresEtiologic diagnosis • Blood • Glucose, calcium, magnesium, electrolytes, BUN • In hypomagnesemia  MgSO4 0.2 ml/kg • Lumbar puncture • Indicated in all neonates with seizures unless related to a metabolic disorder • Inborn errors of metabolism • Inherited as autosomal recessive or X-linked recessive

  15. Neonatal SeizuresEtiologic diagnosis • Inborn errors of metabolism • Serum ammonia  urea cycle abnormalities • Acidosis + anion gap + hyperammonemia urine organic acids should be determined • Unintentional injection of local anesthetic • Supportive measures • Promotion of urine output with IV fluids

  16. Neonatal Seizures(Epileptic Syndromes) • Benign familial neonatal seizures • Begins on the 2nd – 3rd day of life • Seizure frequency : 10 – 20 /day • Patients are normal between seizures • Seizure stops in 1 – 6 months

  17. Neonatal Seizures Fifth-day fits – • 5th day of life • normal appearing neonates with mulifocal seizures • Present for less than 24 hours • Good prognosis

  18. Neonatal SeizuresEtiologic diagnosis • Pyridoxine dependency • resistant to conventional AED’s • Inherited as autosomal recessive • Tx: Pyridoxine 100 – 200 mg IV • May not have a dramatic effect with IV pyridoxine thus maintain on oral pyridoxine 10 -20 mg/day x 6 weeks • Lifelong supplementation : 10 mg/day

  19. Neonatal SeizuresEtiologic diagnosis • Drug withdrawal seizures • Barbiturates, benzodiazepenes, heroin and methadone • Jittery, irritable, lethargic, may show myoclonus or frank seizures • Serum or urine analysis may identify the responsible agent

  20. Why should the infant with epileptic seizures be treated with AED Potential adverse effects of seizure on: • Ventilatory function • Circulation • Cerebral Metabolism • Brain Development disturbance in cerebral blood flow energy metabolism homeostasis of excitotoxic amino acids neurogenesis and synaptic reorganization

  21. Volpe, Neurology of the Newborn, 5th ed. 2008

  22. Clinical Scenario 1 F.M. a 36-37 month old baby boy is noted to have blinking of the eyelids with sucking movements of the mouth at 30 hours of life. The extremities are jittery when tactile stimuli is applied. Maternal history is unremarkable, NSD, G1P1 (1-0-0-1) no hypertension, no infection. Birth weight is 2.5kg. Apgar 8 and 10 at 1 and 5min. The blinking of the eyes and jittery movements of the extremities recur within the next hour.

  23. Clinical Scenario 1 What is your impression? What work-ups will you request? Hgt, CBC, Serum Calcium, Electrolytes What will be your management? Na Luminal 20mg/g IV at 1mg/Kg/min infusion, maintain at 3.5 mg/g/day.

  24. Management of Neonatal Seizures • Na Luminal 20 mg/kg/day IV bolus • Rate of infusion—1 mg/kg/min • Example: Wt is 3 kg, 3 x 20 = 60 mg • Give 60 mg for 20 mins. IV push • Maintenance dose of Na luminal—5mg/kg/day • Example: 3 x 5 = 15 mg • Give 7.5 mg IV q12 hrs

  25. Conditions that Mimic Seizures • Night terrors • Common in boys • 5 – 7 years of age • Sudden onset between midnight and 2:00 am during stage 3 or 4 of sleep or slow-wave sleep • Child screams and appears frightened, dilated pupils, tachycardia and hyperventilation • Child thrash violently can not be consoled , unaware of parents or surroundings

  26. Conditions that Mimic Seizures • Night terrors • 1/3 will have somnambulism • Emotional disorder should be explored in patient with prolonged and persistent night terrors • Short course diazepam maybe considered while the family dynamics is investigated

  27. Conditions that Mimic Seizures • Breath holding spells • Cyanotic spells • Provoked by upsetting or scolding an infant • Brief shrill cry followed by forced expiration and apnea • Rapid onset of generalized cyanosis or loss of consciousness may be associated with repeated generalized tonic jerks, opisthotonos, bradycardia • EEG: normal • Rare before 6 months, peak about 2 years & abate by 5 years old • TX: parent counseling

  28. Conditions that Mimic Seizures • Breath holding spells • Pallid spells • Initiated by painful experience • Child stops breathing  loss of consciousness  pale and hypotonic  tonic seizures • Bradycardia with asystole for 2 seconds may be recorded • EEG: normal • TX supportive but may give atrophine sulfate at 0.01 mg/kg/24 hr in divided doses with a maximum dose of 0.4 mg

  29. Conditions that Mimic Seizures • Syncope • Simple syncope • Decreased blood flow  loss of consciousness  ischemia influences the higher cortical centers to release inhibiting influence on reticular formation within the brainstem  brief tonic contractions of muscles • Results from vasovagal stimulation precipitated by pain, fear, excitement , prolonged standing particularly in a warm environment • Age : 10 -12 years old, females

  30. Conditions that Mimic Seizures • Syncope • Simple syncope • Tilt test – effective in producing symptoms including hypotension • Tx: oral B adrenergic blocking agents

  31. Conditions that Mimic Seizures • Syncope • Cough syncope • Most common in asthmatic children • Occurs shortly after sleep and coughing paroxysm awakens the child • Patients face become plethoric, perspires, agitated, frightened • Loss of consciousness with generalized muscle flaccidity, vertical upward gaze and clonic muscle contraction lasting for several minutes • Urinary incontinence is frequent

  32. Conditions that Mimic Seizures • Syncope • Cough syncope • Cough causes an increased intrapleural pressure  decreased venous return to the right side of the heart  decreased right ventricular output  reduction of left ventricular filling  rapidly diminished cerebral blood flow  cerebral hypoxia  loss of consciousness • Tx: Prevention of bronchoconstriction

  33. Conditions that Mimic Seizures • Syncope • Prolonged QT syndrome • Sudden loss of consciousness during exercise or emotional and stressful experience • Onset late childhood or adolescence • With cardiac arrhythmias such as ventricular fibrillation • ECG: abnormal lengthening of the QT interval (corrected QT of 0.46 or more) • May be associated with acquired heart disease (myocarditis, mitral valve prolapse, electrolyte abnormalities, drug induced) or congenital forms

  34. Conditions that Mimic Seizures • Syncope • Prolonged QT syndrome • Autosomal recessive trait (Jervell and Lange-Nielsen syndrome) associated with deafness • Autosomal dominant (Romano-Ward syndrome)  mutations in cardiac potassium channel gene linked to chromosome 11p15.5 LQT1 • LQT2 results from mutation to second potassium channel gene linked to chromosome 7q35-36

  35. Conditions that Mimic Seizures • Syncope • Prolonged QT syndrome • LQT3 result in mutation in cardiac sodium channel linked to 3p21-24 • LQT4 linked to chromosome 4q25-27 • Testing include supervised exercise test or Holter monitoring • Tx: B adrenergic antagonist drugs Permanent implantable cardiac pacing or left thoracic sympathectomy may be considered if drug is not effective Parents shoudls be taught CPR

  36. Conditions that Mimic Seizures • Paroxysmal Kinesigenic Choeoathetosis • Sudden onset of unilateral or occasional bilateral choreoathetosis or dystonic posturing of a leg, arm and facial grimacing and dysarthia • Precipitated by sudden movements, excitement or stress • Rare last for more than 1 minute • Onset between 8 – 14 years • Attacks may be daily or intermittent

  37. Conditions that Mimic Seizures • Paroxysmal Kinesigenic Choeoathetosis • NE, MRI and EEG – normal • Autosomal recessive inheritance is suggested • Tx: Phenytoin

  38. Conditions that Mimic Seizures • Shuddering attacks • Onset at 4 – 6 months of age • Sudden flexion of the head and trunk and shuddering or shivering movements • May be a precursor to benign essential tremors

  39. Conditions that Mimic Seizures • Benign Paroxysmal torticollis of infancy • Recurrent attacks of head tilt with pallor, agitation and vomiting • Onset : 2 – 8 months • Spontaneous remission at 2 – 3 years of age • Abnormalities in vestibular function • Some patients develop migraine in childhood

  40. Conditions that Mimic Seizures • Hereditary Chin trembling • Repeated episodes of rapid 3/sec chin trembling movements • Precipitated by stress, anger, frustration • Autosomal dominant • NE and EEG - normal • Narcolepsy and cataplexy • Narcolepsy begins before adolescence • Attacks of irrepressible daytime sleep with transient loss of muscle tone (cataplexy)

  41. Conditions that Mimic Seizures • Narcolepsy and cataplexy • EEG shows recurrent sleep attacks consist of REM sleep • Patients are easily aroused • Tx for narcolepsy Modafinil acetamide 200 mg/day

  42. Conditions that Mimic Seizures • Cataplexy • sudden loss of muscle tone and fall to the floor precipitated by laughter, stress or frightening experience • Tx: scheduled naps, amphetamines, methyphenidate, tricyclic antidepressant and counselling regarding occupational safety

  43. Conditions that Mimic Seizures • Rage attacks or episodic dyscontrol syndrome • Sudden and recurrent episodes of violent behavior with minimal provocation • Seem to be psychotic at the time of the attack • EEG: normal

  44. Conditions that Mimic Seizures • Pseudoseizures • Occurs typically between 10 – 18 years old • Lack of cyanosis, normal reaction of pupils to light, no loss of sphincter tone • Normal plantar response • Absence of tongue biting • Can be persuade to have an attack by the physician • EEG-excessive muscle artifact

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