HEADACHE Epidemiology uncommon before the age of 4 years < 10-12 years ( male:female ratio 1:1)

1. HEADACHE Epidemiology • uncommon before the age of 4 years • < 10-12 years(male:female ratio 1:1) • after 10-12 years, (male:female ratio 1:1.5)

2. Classification based on DDx • Primary= Benign (exam normal, no papilledema, no fever/meningismus, normal neuroimaging / LP) • Migraine • Tension-type • Cluster-type • Secondary (neuroimaging / exam / CSF abnormal) most headaches in children are • MIGRAINE or • TENSION

3. Migraine • Genetic predisposition (esp. “classic” with aura) • Pain bilateral or unilateral, usually frontotemporal • Pulsating quality (“throbbing”, “heartbeat”) • Must have autonomic symptoms: • Nausea/vomiting or phonophobia • May be preceded by transient aura (< 1 hr, 15-30 min) • Visual aura most common • Without aura = “common” migraine (70-85 % children)

4. Pathophysiology of Migraine • Vascular theory-NOT supported by cerebral blood flow studies) • Initial vasoconstriction  results in ischemia and transient neurologic symptoms (aura) • Reactive vasodilatation  throbbing head pain • Neuro-vascular theory • Release of pro-inflammatory vasoactive peptides into the dural spac

5. Uncommon forms of migraine •Familial – linked to a calcium channel defect, dominantly inherited • Sporadic hemiplegic migraine • Basilar-type migraine – vomiting with nystagmusand/or cerebellar signs • • Periodic syndromes –and include: • – Cyclical vomiting – • – Abdominal migraine – an idiopathic recurrent • – Benign paroxysmal vertigo of childhood – a

6. Tension • Pain typically posterior > anterior, or band-like • Squeezing quality (“tight”, “vice-like”) • Neck muscles sore • Common trigger: STRESS ! • NO autonomic symptoms • NO nausea/vomiting or photo/phonophobia • NO aura

7. Treatment for primary recurrent headache • MIGRAINE • Avoid / minimizetriggers Optimize hydration • Good sleep hygiene / avoid sleep deprivation • Avoid hunger • Avoid food triggers (aged cheeses, chocolate, caffeine/ soda, processed deli meats, red wine) TENSION • minimize stress (TENSION) • Biofeedback / relaxation • Acupuncture • Self-hypnosis

8. Rescue treatmentsof MIGRANE • • Analgesia – paracetamol and non-steroidal • anti-inflammatory drugs (NSAIDs) • • Anti-emetics – • • Serotonin (5-HT1) agonists, e.g. sumatriptan. A • nasal preparation of this is licensedfor use in • children over 12 years of age.

9. Prophylactic agents • Where headaches are frequent and intrusive: • • Pizotifen (5-HT antagonist) – can cause weight gain and sleepiness • • Beta-blockers – propranolol; contraindicated in asthma. • • Sodium channel blockers – valproate or • topiramate.

10. Secondary headache • Vision problems • Chronic sinusitis

11. Rethink the diagnosis of benign headache when: • headache is always in the same location • headache fails to respond to multiple medical therapies • focal neurologic findings appear • VI nerve palsy, diplopia, new onset strabismus • hemiparesis • progressively increasing frequency / severity of headache • headache awakens from sleep

12. NEUROIMAGING for headache (prior to LP) if: • abnormal neurologic exam • altered mental status • papilledema, VI nerve palsy, diplopia, new onset strabismus • focal findings (hemiparesis) • nuchal rigidity, fever • change in headache frequency, intensity, type

13. SEIZURES AND EPILEPSY IN CHILDREN

14. Epidemiology of Seizures and Epilepsy in Children • 4-6 % incidence of a singleseizure • 1% incidence of epilepsy(> 2 unprovoked seizures) • 70-80 % achieve remission (“outgrow” seizures) • HISTORY is the most important tool in differentiating a seizure from a non-seizure look-alike • EEG is an adjunctive test to clinical history • after 1st unprovoked seizure: • If EEG normal, 40% recurrence risk • If EEG abnormal, 80% recurrence risk • 50% of 2nd unprovoked seizures occur within 6 months of 1st seizure

15. Epilepsy • Definition: Two or more unprovoked seizures that occur at interval greater than 24 hours (i.e recurrent seizures)

16. Causes • 1. Idiopathic (primary) in 80% of cases • - Genetic basis exist for many epileptic syndromes • 2. Organic (secondary) in 20% of cases • - Congenital cerebral malformation. • - Degenerative brain diseases. • - Post-traumatic. • - Post-hemorrhagic. • - Post-infection. • - Post-toxic. • -Post-anoxic.

20. Absence (Petit Mal) Epilepsy • - Sudden onset of staring, interrupting speech or activity • Occurs multiple times per day • Short duration (seconds) • - Occurs in school aged children ~ 4-12 years, otherwise normal

22. Childhood • Frequency 8% • Genetic predisposition- strong 20% • Female preponderance 75% • EEG: bilateral, synchronous, symmetrical 3 / sec spike wave, normal background. • Seizures: Very frequent simple absences. • Rx: VPA or ESM with control in 70 - 80%. • Evolution: Remission- 95%. • Rare persistence of absences only- 6%

23. Absence (Petit Mal) Epilepsy EEG findings characteristic: - bilateral generalized 3 Hz spike-and-wave discharges - provoked by hyperventilationandphotic stimulation

24. EPILEPSYSYNDROMES

26. Infantile Spasms (West Syndrome) – a severe epilepsy Severely abnormal EEG pattern: disorganized, discontinuous, high amplitude, multifocal spikes calledHYPSARRHYTHMIA • Clinical spasms (1-2 secs) • a subtle momentary flexion or • extension of the body • occur in clusters when drowsy • (waking or falling asleep) Treatment: ACTH

30. Infantile spasms • may be mistaken for colic, reflux, hiccups, or a startle ! • called symptomatic if etiology identified: • brain insult at birth (ex. hypoxia-ischemia, meningitis) • brain malformation • neurocutaneous disorder (Tuberous Sclerosis) • metabolic disorder • called cryptogenic if NO identifiable cause • prognosis best (10% good outcome) if idiopathic • normal development at onset of infantile spasms • extensive etiology testing negative • prognosis poor for: • seizure control (infantile spasms and future seizures) • future neurocognitive and developmental abilities

31. Lennox-Gastaut Syndrome –a severe epilepsy • Often evolves frominfantile spasms • Neurodevelopmentally impaired children • Syndrome defined by a TRIAD of: • 1. mixed seizure types: atonic, atypical absence, myoclonic, tonic-clonic, partial • 2. developmental delay • 3. abnormal EEG pattern: slow (< 2.5 Hz) spike wave discharges • Symptomatic or cryptogenic etiologies (like IS) • Prognosis poor

32. Juvenile Myoclonic Epilepsy (JME) EEG: bilateral generalized 4-6 Hz spike-wave or polyspike-wave activity Seizure types: - myoclonic in AM - “grand mal” - absence

37. Benign Rolandic Epilepsy Aka Benign Focal Epilepsy of Childhood with Centrotemporal Spikes EEG has characteristic pattern: bilateral independent centrotemporal spikes

38. Benign Rolandic Epilepsy • Treatment recommended only if: • Seizures frequent (which is unusual) • Socially stigmatizing if occur in wakefulness • Anxiety provoking for parents if occur in sleep • Effective treatments: • Avoidance of sleep deprivation • Medications: carbamazepine, oxcarbazepine • Time(outgrown by adolescence)

39. Other Epilepsy Syndromes • Landau-Kleffner Syndrome • an acquired EPILEPTIC APHASIA in a PREVIOUSLY NORMAL child, usually 3-7 years old • Gradual or sudden inability to understand or use spoken language (“word deafness”) • Must have EEG abnormalities in sleep (sleep activated) • Additional behavioral and psychomotor disorders (hyperactivity, aggressiveness, depression, autistic features) • May have additional overt clinical seizures (80 %) in sleep

40. Other Epilepsy Syndromes • Rett Syndrome • Occurs only in girls (X-linked lethal mutation) • Initial normal development  dev regression / autistic (loss of motor / language / social skills) • Acquired microcephaly (deceleration of head growth) • Hand wringing / alternating hand movements • Apnea / hyperpnea / breathholding • Seizures

41. Partial (Focal) Epilepsy • onset of seizure begins in one area of one cerebral hemisphere (apparent clinically or via the EEG) • “simple”: no impairment of consciousness • “complex”: impairment of consciousness (staring) • “secondarily generalized”: a simple or complex partial seizure that ends in a generalized convulsion

42. Anatomic Onset of Focal Epilepsies • Most frequently involved brain regions: • Temporal Lobe (80 %) > Frontal Lobe >> Parietal or Occipital • MRI or CT: • Normal or Abnormal • Neurologic exam: • Normal or Abnormal Mesiotemporal sclerosis

43. Spells that mimic seizures • Apnea / ALTE • GER • Sleep disorders (nocturnal myoclonus, night terrors, narcolepsy/cataplexy) • Migraine variants (esp. aura) • Benign breathholding spells • No neuro consult / lab / EEG / CT, Fe for cyanotic type • Syncope • Movement Disorders (tics, tremor, dystonia) • ADD • Behavioral Stereotypies (PDD) • Pseudoseizures (psychogenic seizures) • Strange posturing, back arching, writhing • Alternating L and R limb shaking during same seizure • Psychosocial stressor

45. Treatment of epileptic seizures • often not until after the second unprovoked seizure • choice of AED based on maximum efficacy for that particular seizure type and minimal side effects • 70% become seizure free on monotherapy • an additional 15% become seizure free on polypharmacy • 15% remain intractable • Discontinue AED after 2 years seizure free EXCEPT for JME • Alternate treatments: • Ketogenic diet (high fat diet) • Vagal nerve stimulator – FDA approved for partial seizures in 12 years+ • Epilepsy surgery

46. Classic side effects of AEDs • valproic acid (Depakote): hepatotoxicity, weight gain, acute pancreatitis • lamotrigine (Lamictal): Stevens-Johnson syndrome • phenytoin (Dilantin): gingival hypertrophy, acute ataxia, osteoporosis • phenobarbital: adverse behavior / hyperactivity • carbamazepine (Tegretol): agranulocytosis, aplastic anemia • oxcarbazepine (Trileptal): hyponatremia • ethosuximide (Zarontin): lupus-like reaction • topiramate (Topamax): weight loss, acidosis, renal stones • felbamate (Felbatol): aplastic anemia • gabapentin(Neurontin): behavioral changes

47. Status Epilepticus • Def: any type of seizure lasting > 30 minutes or repeated seizures without recovery between seizures over 30+ minutes • seizures > 1 hour are associated with neuronal injury due to glutamate excitotoxicity • Evaluation and acute treatment for seizure > 5 minutes: • ABC’s (RR, HR, BP) • check temp, glucose, electrolytes, CBC, renal and hepatic function, AED levels • Benzodiazepine  phenytoin  phenobarbital

48. Neonatal Seizures (not epilepsy) • Benign Neonatal Familial Convulsions • Onset 2nd or 3rd day of life • No perinatal complications • Autosomal dominant condition (+FHx) • chromosomes 20 and 8 • affected gene product: alpha-subunit of Ach Receptor • Mixed seizure types • apneic, clonic, tonic, autonomic, oculofacial • Typically easy to control seizures which resolve in 1st year of life • Neuroimaging and EEG normal

49. Neonatal Seizures (not epilepsy) • Symptomatic (secondary) neonatal seizures • Multiple Causes • Hypoxia-Ischemia • Infection (meningitis, sepsis) • Hemorrhage (IVH, subarachnoid, intraparenchymal) • Infarction (thrombotic, hemorrhagic) • Metabolic derangement (sodium, calcium, glucose) • Inborn errors of metabolism • CNS malformation • Treatments: IV phenobarbital, IV phenytoin

50. Febrile Seizures • 2-4 % of children age ~ 6 months – 6 years • Provoked by a sudden spike in temp usually with URI, Acute OM, AGE (genetic predisposition) • “Simple” • Generalized convulsion(whole body shaking) • Brief(< 15 minutes) • Only onein the course of an illness • Future risk of epilepsy (1%) SAME AS other children • “Complex” • focal seizure (one side of body shaking, staring) • prolonged (> 15 minutes) • multiple in 24 hours • Complex febrile seizures hint at an increased risk of future epilepsy