Block 11 Board Review Part 2 of 4

1. Block 11 Board ReviewPart 2 of 4 Neurology/Heme-Onc 18April2014 Chauncey D. Tarrant, M.D. Chief of Residents 13-14

2. 3% of Initial Certifying Exam!!!

3. Pediatrics In Review Articles • Seizures • Malformations

4. PIR Quiz

5. 1. A 2-year-old has febrile seizures. Which of the following factors will increase her risk of subsequently developing epilepsy? A. An initial febrile seizure with a relatively low temperature. B. Brief duration between onset of the fever and seizure. C. Complex febrile seizure. D. Family history of febrile seizures in a first-degree relative. E. Younger age at onset of febrile seizures.

6. 1. A 2-year-old has febrile seizures. Which of the following factors will increase her risk of subsequently developing epilepsy? A. An initial febrile seizure with a relatively low temperature. B. Brief duration between onset of the fever and seizure. C. Complex febrile seizure. D. Family history of febrile seizures in a first-degree relative. E. Younger age at onset of febrile seizures.

7. 2. Treatment with antiepileptic drugs is recommended after 2 or more recurrent afebrile seizures. A common question from parents in response to this treatment parameter is, “What percentage of children who experience a single unprovoked seizure will not have another?” A. 10%. B. 20%. C. 40%. D. 60%. E. 80%.

8. 2. Treatment with antiepileptic drugs is recommended after 2 or more recurrent afebrile seizures. A common question from parents in response to this treatment parameter is, “What percentage of children who experience a single unprovoked seizure will not have another?” A. 10%. B. 20%. C. 40%. D. 60%. E. 80%.

9. 3. An 8-year-old child with a long history of recurrent generalized tonic-clonic seizures develops generalized convulsive status epilepticus. Which of the following is the first treatment of choice on encountering trained medical personnel? A. Diazepam. B. Fosphenytoin. C. Lorazepam. D. Pentobarbital. E. Phenobarbital.

10. 3. An 8-year-old child with a long history of recurrent generalized tonic-clonic seizures develops generalized convulsive status epilepticus. Which of the following is the first treatment of choice on encountering trained medical personnel? A. Diazepam. B. Fosphenytoin. C. Lorazepam. D. Pentobarbital. E. Phenobarbital.

11. 4. A 7-year-old boy develops spells that consist of a unilateral contraction of the left side of his face without impairment of consciousness or speech arrest. Which of the following is the most likely diagnosis? A. Absence seizures. B. Benign rolandic epilepsy. C. Juvenile myoclonic epilepsy. D. Psychomotor seizures. E. Simple partial seizures.

12. 4. A 7-year-old boy develops spells that consist of a unilateral contraction of the left side of his face without impairment of consciousness or speech arrest. Which of the following is the most likely diagnosis? A. Absence seizures. B. Benign rolandic epilepsy. C. Juvenile myoclonic epilepsy. D. Psychomotor seizures. E. Simple partial seizures.

13. 5. Generalized tonic-clonic seizures in adolescents are typical of which epileptic region of the brain? A. Centrofrontal lobe region. B. Corpus callosum region. C. Frontal lobe region. D. Parietal lobe region. E. Temporal lobe region.

14. 5. Generalized tonic-clonic seizures in adolescents are typical of which epileptic region of the brain? A. Centrofrontal lobe region. B. Corpus callosum region. C. Frontal lobe region. D. Parietal lobe region. E. Temporal lobe region.

15. 1. You are seeing a 12-year-old girl who has myelomeningocele in your office for complaints of radiating back pain over the past 3 months. She has not changed her bowel or bladder management program but is now having new urinary leakage between catheterizations. The additional symptom that most supports your presumptive diagnosis is: A. Declining school performance. B. Deteriorating gait. C. Difficulty swallowing. D. Early morning headache. E. Worsening handwriting.

16. 1. You are seeing a 12-year-old girl who has myelomeningocele in your office for complaints of radiating back pain over the past 3 months. She has not changed her bowel or bladder management program but is now having new urinary leakage between catheterizations. The additional symptom that most supports your presumptive diagnosis is: A. Declining school performance. B. Deteriorating gait. C. Difficulty swallowing. D. Early morning headache. E. Worsening handwriting.

17. 2. An emergency department physician calls you about her evaluation of a 14-month-old child who has myelomeningocele and a ventriculoperitoneal shunt. Results of computed tomography scan and shunt series are unchanged from previous studies, but the child is not “normal,” according to the parents. Which of the following is most concerning for a shunt malfunction? A. Ankle clonus. B. Limited upward gaze. C. Patulous anus. D. Pes cavus. E. Swelling on back.

18. 2. An emergency department physician calls you about her evaluation of a 14-month-old child who has myelomeningocele and a ventriculoperitoneal shunt. Results of computed tomography scan and shunt series are unchanged from previous studies, but the child is not “normal,” according to the parents. Which of the following is most concerning for a shunt malfunction? A. Ankle clonus. B. Limited upward gaze. C. Patulous anus. D. Pes cavus. E. Swelling on back.

19. 3. During a neonatal consultation for an infant born with a neural tube defect (neurologic level L3), the family asks if their child will walk. The most accurate statement regarding this child’s future walking ability is that she will walk: A. Independently. B. With ankle bracing. C. With high leg bracing. D. With hip bracing. E. With low leg bracing and forearm crutches.

20. 3. During a neonatal consultation for an infant born with a neural tube defect (neurologic level L3), the family asks if their child will walk. The most accurate statement regarding this child’s future walking ability is that she will walk: A. Independently. B. With ankle bracing. C. With high leg bracing. D. With hip bracing. E. With low leg bracing and forearm crutches.

21. 4. You are seeing an 8-month-old boy who was born with myelomeningocele for a routine follow-up evaluation in the clinic. His parents have been reading on the Internet about the Chiari II malformation and ask if their son has this problem. The clinical finding most commonly associated with Chiari II malformation is: A. Bulging fontanel. B. Leg pain. C. Scoliosis. D. Stridor. E. Urinary incontinence.

22. 4. You are seeing an 8-month-old boy who was born with myelomeningocele for a routine follow-up evaluation in the clinic. His parents have been reading on the Internet about the Chiari II malformation and ask if their son has this problem. The clinical finding most commonly associated with Chiari II malformation is: A. Bulging fontanel. B. Leg pain. C. Scoliosis. D. Stridor. E. Urinary incontinence.

23. Seizures

24. What are some metabolic causes of seizures?

25. What are some metabolic causes of seizures? • glucose, sodium, phosphate, magnesium, and calcium disturbances

26. Which drugs precipitate or exacerbate seizures?

27. Which drugs precipitate or exacerbate seizures? • Neuroleptics • Stimulants • Buspirone* • Diphenhydramine • Antibiotics (beta lactams) • Pink Grapefruit Juice (not a drug)

28. What are the most common causes of acute seizures?

29. What are the most common causes of acute seizures? • fevers • Infections • head injury • Neonates: hypoxia-ischemia

30. What are some examples of nonepileptic events?

31. What are some examples of nonepileptic events? • Breath Holding Spells • Tics • Self Stimulation • Syncope • Gastroesophageal reflux • Psychogenic seizures • Sleep Disturbances (sleepwalking/sleep apnea)

32. What are the factors associated with increased risk of seizure disorder?

33. What are the factors associated with increased risk of seizure disorder? • Family history of epilepsy • Previous history of seizure • Modest risk with complex febrile seizures • History of meningoencephalitis • History of penetrating traumatic brain injury • Presence of diseases that lead to electrolyte disturbances • Presence of acidosis associated with hypoxia • History of toxic Ingestion

34. What are the etiologic and therapeutic implications of partial versus generalized seizures?

35. What are the etiologic and therapeutic implications of partial versus generalized seizures?

36. How do you manage a child with a first seizure?

37. How do you manage a child with a first seizure? • Thorough history and physical • Consider Neurology Consult/EEG • Thorough family history • Seizure diary • Videotape events (if possible) • History of epilepsy? • History pf conditions that cause electrolyte disturbances? • Predisposing factors (sleep deprivation, fevers, illness, or infection)

38. How do you manage a child with recurrent seizures?

39. How do you manage a child with recurrent seizures? • MRI • EEG • Consider treatment with AEDs • Others: surgery (brain surgery vs. VNS), ketogenic diet (low glycemic index diet)

40. How would you manage a patient with psychogenic seizures?

41. How would you manage a patient with psychogenic seizures? • Discuss with parents after negative video EEG • Consultation with child psych • Treat underlying psychological cause (anxiety, depression, conversion, somatization etc.)

42. Common Side Effects and Toxicities in AEDs… (this article) • Lamictal • Trileptal • Clonazepam • Ethosuximide • Valproic Acid

43. Common Side Effects and Toxicities in AEDs… • Lamictal • Rash (SJS) if titration too fast • Can exacerbate known neurobehavioral symptoms • Trileptal • Decrease WBC counts and decreased Na • Clonazepam • BZD withdrawal seizures, sedation • Ethosuximide • GI upset • Valproic Acid • Thrombocytopenia, transaminitis, increased pancreatic enzymes leading to pancreatitis, weight gain, PCOS, balding

44. From PREP

45. Febrile Seizures • Natural History • Risk Factors Associated with later Epilepsy • Diagnostic Criteria • Evaluation • Management

46. Febrile Seizures • Natural History: 6mo-5yrs • Risk Factors Associated with later Epilepsy: family hx, complex, devo/neuro abnormalities • Diagnostic Criteria: age ≥6mo, associated with febrile illness, no previous sz history • Evaluation: None if simple; consider MRI/EEG if complex • Management: Reassurance (rule of 1/3s)

47. Infantile Spasms aka.._______ Syndrome • Characteristic Clinical Features? • Treatments? • Prognosis?

48. Infantile Spasms aka West Syndrome • Characteristic Clinical Features? • http://www.youtube.com/watch?v=aVoJtslvqOU&feature=player_detailpage • infants ages 3 to 9 months • spasm like seizures that involve flexion, extension, mixed flexion-extension of the arms, legs, and trunk • Treatments? ACTH/ Vigabatrin (Tuberous Sclerosis) • Prognosis? Poor

49. FYI • Drug selection is based on seizure type • Treat if patient has 2 or more recurrent seizures • Discontinue AED therapy after 2yrs seizure free, and wean off • Check AM trough levels if there is a concern for efficacy (fast metabolizers) or compliance • Trileptal and Tegretol levels can be elevated by macrolides • Children with epilepsy have a higher occurrence of anxiety, depression, ADHD

50. FYI • Benign Rolandic Epilepsy • centrotemporal spikes • Most common type of partial epilepsy in childhood, • usually between 5-10yrs • Involve unilateral facial sensory-motor and oropharyngogutteralsymptoms, hypersalivation, and speech arrest • Usually stop by age 16yrs • Meds after 3 or more