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Pediatric Neurology

Pediatric Neurology. Dr. Kristen Johnson Dr. Adam Oster April 14, 2011. In 60 minutes…. What we will try to cover. What we will not. Headache Stroke Intracranial mass lesions Lower motor neuron weakness. Seizures Common seizure mimics Hydrocephalus and VP shunts

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Pediatric Neurology

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  1. Pediatric Neurology Dr. Kristen Johnson Dr. Adam Oster April 14, 2011

  2. In 60 minutes…. What we will try to cover. What we will not. Headache Stroke Intracranial mass lesions Lower motor neuron weakness • Seizures • Common seizure mimics • Hydrocephalus and VP shunts • A few other interesting cases….

  3. 18 month old girl arrives at the PLC Runny nose and cough Seizure this afternoon lasting 1 minute Temp 39C, VSS Normal neuro exam

  4. Diagnosis?

  5. What are the criteria to diagnose a febrile seizure? ✓6-60 months of age ✓No evidence of intracranial infection ✓Neurologically normal child ✓No history of afebrile seizures Simple febrile Complex febrile Prolonged Focal Occur more than once in a 24 hour period • < 15 minutes in duration • Generalized • Occur once in a 24 hr period

  6. What investigations would you like to order?

  7. CBCD Lytes Ca Mg PO4 glucose NO ROLE American Academy of Pediatrics – Clinical Practice Guideline 2011

  8. . A lumbar puncture should be performed if: • A. signs and symptoms of meningitis • in an infant between 6-12mo who is considered deficient in • Hib or S. pneumo immunizations (option) • C. in a child who has been pretreated with antibiotics (option) American Academy of Pediatrics – Clinical Practice Guideline 2011

  9. The parents now have a list of questions for you….

  10. What are the chances that this will happen again? • Children < 12mo at the time of their first febrile seizure • 50% probability of recurrence • Children > 12mo at the time of their first febrile seizure • 30% probability of recurrence • Those who have a second febrile seizure have 50% chance of at least 1 additional recurrence

  11. What are the chances my child will have epilepsy? • Baseline risk of epilepsy in the population • 1% • Risk of epilepsy in those with simple febrile seizures • 2% • Risk increases slightly if: • Multiple febrile seizures • < 12mo old at time of first febrile seizure • Complex febrile seizure • Family history of epilepsy

  12. Is there anything I can do to prevent this in the future? • NO

  13. American Academy of Pediatrics – Clinical Practice Guideline 2008 • 1. Neither continuous or intermittent anticonvulsant therapy is recommended. • Adverse effects outweigh benefits • 2. Antipyretics do not reduce the recurrence risk and are therefore not recommended.

  14. Same child except…. Afebrile Vomiting and diarrhea for 2d Had 4 brief seizures in a row Does this change your thoughts?

  15. 4 year old boy presents to ACH with his first episode of seizure • Well prior to event • GTC seizure shortly after waking; • lasted 3 minutes; • post-ictal for 20 minutes; • back to baseline now • No history of fever or viral sx • No history of head trauma • or toxic ingestion • Developmentally normal • Normal neurological exam

  16. 1st unprovoked seizure… What investigations would you like to order?

  17. 1. Lab tests should be ordered based on individual clinical circumstances • i.e. Vomiting, diarrhea, dehydration • Higher incidence of abnormalities (hypoNa+) in < 6mo • 2. Consider toxicology screening if clinical suspicion • 3. LP is of limited value in absence of clinical signs and symptoms of meningitis/encephalitis • Consider in < 6mo age group American Academy of Neurology – Practice Parameter 2000

  18. 4. EEG should be part of standard workup • Helps determine seizure type, epilepsy syndromes, risk of recurrence • Recurrence rate of 54% with abnormal EEG vs. 25% with normal EEG • 5. If neuroimaging is obtained, MRI is preferred modality • Emergent neuroimaging • postictal focal deficit (Todd’s paresis) or slow return to baseline • Nonurgent MRI should be considered in • Children < 1yo • Those with focal seizures • Those with EEG not in keeping with a benign epilepsy of childhood • Those with developmental delays American Academy of Neurology – Practice Parameter 2000

  19. You are called STAT to the trauma room • EMS has just arrived with a 2 yo girl who is having a generalized tonic clonic seizure • Seizure began 20 minutes ago • EMS unable to gain IV access • Given diastat 5mg PR x 1 dose

  20. The nurses cannot get an IV… what are your options? • Rectal diazepam 0.5mg/kg • Buccalmidazolam 0.5mg/kg • Intranasal midazolam 0.2mg/kg • Intramuscular midazolam 0.2mg/kg • Intranasal lorazepam 0.1mg/kg • Sublingual lorazepam 0.1mg/kg

  21. Is one first line agent better than the others? • Buccalmidazolam is better than rectal diazepam • 56% versus 27% • IV lorazepam is better than IV diazepam • At least as effective (70% versus 65%) • Fewer side effects • Bottom line: 1. when IV access is unavailable buccalmidazolam is the treatment of choice; 2. IV lorazepam should replace IV diazepam in initial management Appleton et al. Cochrane 2008

  22. What next? • Second line agents • Phenytoin 20mg/kg (1mg/kg/min) • Fosphenytoin (3mg PE/kg/min) • Third line agents • Phenobarbital (20mg/kg) • Valproic acid (20-30mg/kg) • Keppra/Levetiracetam • Infusions • Midazolam (1-30mcg/kg/min) • Thiopental • Pentobarbital (5mg/kg then 1-3mg/kg/hr) • Ketamine • Propofol • Intubation • Propofol (1-3mg/kg) • Thiopental (3-5mg/kg)

  23. Would your management change if this was a neonate? • 1st line agent • Phenobarbital • Pyridoxine trial

  24. Convulsive Status Epilepticus

  25. Convulsive Status Epilepticus • Incidence 38/100 000/year in age 1-19 • Higher incidence < 1yo – 135/100 000/year • 9-27% of children with epilepsy will have an episode of status epilepticus • 12% present with status on 1st episode of seizure • Outcomes mainly dependent on cause • Mortality 4%

  26. The importance of treating early… Status aborted in 100% who received 3rd AED within 60 minutes of 1st versus Only 22% who received 3rd AED > 60 minutes from the 1st

  27. Etiology of Convulsive Status Epilepticus

  28. Diagnostic yield of investigations

  29. Now on to random cases… Spot diagnoses

  30. 2 mo boy with jerky movements • Mom notices that he flinches and tenses up periodically • Occurs during awake periods • Becoming more and more frequent • She has brought a video in for you to see

  31. See video http://www.youtube.com/watch?v=AQ3ZbWPSx1g

  32. Infantile spasms Cryptogenic (1/3) Symptomatic (2/3) Known etiology Tuberous Sclerosis most common CNS malformations IEMs Congenital infections Developmental delay • Normal development • No known etiology • Normal exam • Normal EEG • Normal MRI

  33. 18 month old boy presenting with ? seizure • He was crying and all of the sudden he turned blue, fell over and had a few twitches • He was awake 1 minute later and now seems fine • Otherwise healthy boy • Mom recorded the episode for you

  34. See video http://www.youtube.com/watch?v=2bKVHSe6hVQ

  35. Breath-holding spells6 months – 4 years old associated with Fe deficiency Cyanotic Pallid Less common Preceded by minor trauma Child turns pale and collapses Apnea and limpness followed by rapid recovery • Most common • Child crying +++, holds breath, rapidly cyanotic, becomes rigid • Transient LOC with brief twitching of extremities • Return to baseline quickly

  36. 4 month old with recurrent episodes of stiffening and arching back Sandifer’s Syndrome

  37. 3 wk old girl with jerky movements during sleep • Video clip http://www.youtube.com/watch?v=7z2FXVtxgaI

  38. 5 month old girl with jerky movements • Come in clusters • Often around mealtime • Otherwise developing normally • Normal examination • Watch video • http://www.youtube.com/watch?v=KI7JTnQodGE

  39. 2 mo boy presenting with irritability and vomiting

  40. Infants = 50mL CSF Adults = 150mL CSF

  41. Hydrocephalus • Blockage • Aqueductalstenosis • Congenital • Intrauterine infection • Tumor • Congenital malformation • Myelomeningocele • Chiari malformations • Dandy-Walker malformations • Impaired absorption • Hemorrhage • Ex-prem with IVH • Meningitis • Oversecretion • Choroid plexus tumors

  42. Ventriculoperitoneal shunt

  43. Same infant returns 6 weeks later with vomiting • Low grade fever for the past 2 days • Has been more irritable than usual • Hr 140, rr28, BP 85/50, T 38.2 C • Fontanel feels slightly full • Full EOM, PERL, no facial asymmetry • Mild increased tone in extremities

  44. VP shunt complications Infection Obstruction Most common within first 6 months; over half within first 2 years Investigations Shunt series Fast MRI CT • Usually within first 2 months • Staph epi and Staph aureus most common • Late infections are usually gram negative organisms • Treat with vancomycin and cefotaxime

  45. Shunt series

  46. 6 year old girl • Previously healthy • Woke this am with a facial droop • Remainder of neurologic • examination is normal

  47. What is your differential diagnosis? • Bell’s palsy • Lyme disease • HIV infection • Otitis media • Cholesteatoma

  48. How are you going to treat her? Lacri-lube eye ointment ? Corticosteroids ? Antiviral Therapy

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