1. Block 11 Board Review Part 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. glucose, sodium, phosphate, magnesium, and calcium disturbances

26. Which drugs precipitate or exacerbate seizures?

27. Neuroleptics Stimulants Buspirone* Diphenhydramine Antibiotics (beta lactams) Pink Grapefruit Juice (not a drug)

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

29. fevers Infections head injury Neonates: hypoxia-ischemia

30. What are some examples of nonepileptic events?

31. 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. 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. PartialGeneralized (GTC) Focal findings with no altered consciousness (simple) Altered consciousness (complex) Multifocal Prodrome  seizure  postictal state Trileptal, KeppraLamictal, Valproic Acid, Topamax, Keppra, Zonegran

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

37. 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. 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. 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&feat ure=player_detailpage http://www.youtube.com/watch?v=aVoJtslvqOU&feat ure=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 oropharyngogutteral symptoms, hypersalivation, and speech arrest – Usually stop by age 16yrs – Meds after 3 or more

51. FYI Juvenile Myoclonic Epilepsy – Age 5-15yrs – (1) myoclonic jerks on awakening – (2) GTC seizures in 90% of patients, and – (3) development of absence seizures in one-third of all patients.

52. Malformations

53. What are common orthopedic problems associated with Spina Bifida?

55. What is the long term management for neurogenic bladder/bowel?

56. BladderBowel Self CatheterizationTimed Toileting Renal UltrasoundIncreased fiber in diet UrodynamicsLaxatives VesicostomyEnemas (antegrade colonic enema)*

57. What are the clinical and radiological features and prognosis of spina bifida occulta?

58. What are the clinical features and prognosis of spina bifida occulta? Sometimes called “hidden spina bifida” May have no clinical manifestations May only see a small gap in the spine, no opening Good prognosis

59. What is the Differential for a Child with Spina Bifida with Neurologic deterioration?

60. Increased ICP Shunt Malfunction Chiari II Malformation Tethered Cord

61. What are some signs and symptoms of hydrocephalus/VP shunt malfunction?

63. PREP

64. During the hottest week of the summer, a 6-month- old previously healthy infant presents to the emergency department via ambulance in tonic- clonic status epilepticus. After two doses (each 0.05 mg/kg) of intravenous lorazepam, the seizure continues. The bedside glucose measurement is normal, as is a noncontrast head computed tomography scan. Of the following, the MOST likely cause of the child’s prolonged seizure is

65. A. hyperthyroidism B. hypocalcemia C. hypomagnesemia D.hyponatremia E. pyridoxine deficiency

66. A. hyperthyroidism B. hypocalcemia C. hypomagnesemia D. hyponatremia E. pyridoxine deficiency

67. A 4-year-old boy has had two fairly similar, brief episodes within the past month consisting of abrupt arrest of ongoing behavior, glassy-eyed staring, and lip smacking, followed by confusion and sleepiness for 1 hour. He has had no fevers or other signs of illness at the time of either event. Findings on his medical and developmental histories are otherwise normal. Physical and neurologic examination results are normal. Brain magnetic resonance imaging yields normal results, and electroencephalography shows no abnormalities. Of the following, the MOST appropriate treatment for this boy is

68. A. carbamazepine B. ethosuximide C. felbamate D. phenobarbital E. phenytoin

69. A. carbamazepine B. ethosuximide C. felbamate D. phenobarbital E. phenytoin

70. A 12-year-old girl who has idiopathic partial epilepsy that has been well controlled with carbamazepine for 1 year develops an ear infection. Because she is allergic to penicillin, an urgent care physician prescribes a course of azithromycin. Three days later, she presents to the emergency department with vomiting and dizziness. On physical examination, she has end-gaze nystagmus in both horizontal directions and a broad-based gait. Of the following, the MOST likely explanation for this girl’s clinical findings is

71. A. carbamazepine toxicity B. confusional migraine C. otitic hydrocephalus D. postictal presentation E. unilateral labyrinthitis

72. A. carbamazepine toxicity B. confusional migraine C. otitic hydrocephalus D. postictal presentation E. unilateral labyrinthitis

73. A 2-year-old girl presents after a prolonged focal-onset seizure. She was playing when she suddenly started crying and her right arm started jerking. Her whole body then jerked for 10 to 15 minutes, during which time she could not respond to her mother. The jerking was subsiding when the paramedics arrived, and the child slept en route to the emergency department. The girl was born at 26 weeks’ gestation and has developmental delay. She sits but does not yet walk. Of the following, the STRONGEST predictor of seizure recurrence in this child is

74. A. exposure to brother’s video games B. family history of febrile seizures C. minor trauma the day before the visit D. preexisting neurologic impairment E. stress associated with visiting the biological father

75. A. exposure to brother’s video games B. family history of febrile seizures C. minor trauma the day before the visit D. preexisting neurologic impairment E. stress associated with visiting the biological father

76. A 9-year-old boy had 2 generalized tonic-clonic seizures 1 week apart at 7 years of age. Evaluation at that time included a normal routine electroencephalogram (EEG), sleep-deprived EEG, 3-day video EEG, and brain magnetic resonance imaging (MRI). Administration of carbamazepine was initiated and the boy has not had further seizures. He has done well in school, receiving average grades. His neurologist recently obtained an EEG, which was again normal, and is recommending that the boy discontinue anticonvulsant therapy. The boy’s mother is concerned that if medication is discontinued, her son might have another seizure. Of the following, the MOST accurate statement about discontinuing anticonvulsants is

77. A. a follow-up MRI should be obtained before stopping medications B. it is best to discontinue medications abruptly C. medications should be continued until after puberty D. the normal EEG is an indication that seizures will not recur E. the standard for tapering medications is 2 years of being seizure free

78. A. a follow-up MRI should be obtained before stopping medications B. it is best to discontinue medications abruptly C. medications should be continued until after puberty D. the normal EEG is an indication that seizures will not recur E. the standard for tapering medications is 2 years of being seizure free

79. Two months ago, an otherwise healthy 15-year-old girl presented to the emergency department (ED) with a generalized tonic-clonic seizure she had that morning. The seizure was described as an abrupt stiffening of the body that was followed by generalized fast rhythmic jerking; her eyes were open, but she was unaware of her surroundings. The episode lasted 1 to 2 minutes and was followed by confusion and sleep. A head computed tomography scan performed in the ED was normal, and she was discharged on no seizure medication. Her mother calls your office because she has just had another morning seizure after a sleepover with her friends. Of the following, the MOST appropriate course of action is to recommend

80. A. electroencephalography to determine if the child has epilepsy B. neurologic consultation for seizure medication C. psychological evaluation for psychogenic nonepileptic seizure (pseudoseizure) D. repeat head computed tomography scan E. vitamin D concentration to assess for risk of fracture

81. A. electroencephalography to determine if the child has epilepsy B. neurologic consultation for seizure medication C. psychological evaluation for psychogenic nonepileptic seizure (pseudoseizure) D. repeat head computed tomography scan E. vitamin D concentration to assess for risk of fracture

82. A 15-year-old boy who has idiopathic partial epilepsy presents to the emergency department after a prolonged seizure. His maintenance medication is oxcarbazepine. Of the following, the MOST appropriate next step in managing this patient is to

83. A. change his medication B. obtain a serum oxcarbazepine concentration C. obtain emergent magnetic resonance imaging of the brain D. obtain serum calcium, magnesium, and phosphorus concentrations E. schedule outpatient electroencephalography

84. A. change his medication B. obtain a serum oxcarbazepine concentration C. obtain emergent magnetic resonance imaging of the brain D. obtain serum calcium, magnesium, and phosphorus concentrations E. schedule outpatient electroencephalography

85. An 8-year-old girl has a seizure at school. She was sitting at her desk when suddenly her whole body stiffened and she fell to the floor and had jerking movements of her limbs. The event lasted about 2 minutes and then stopped. The girl was sleepy for about 30 minutes and then returned to normal. This has never happened before, she has been healthy all her life, and there is no one in the family who has seizures. You order an electroencephalogram and receive a report that describes “right and left centrotemporal spikes, consistent with benign rolandic epilepsy.” You refer her to a pediatric neurologist and her appointment is next week. Of the following, the MOST appropriate advice to provide the girl’s parents at this time is

86. A. brain damage is common even after a single seizure B. children who have epilepsy should not participate in contact sports C. children who have epilepsy should not take baths alone D. risk of sudden death is high in children who have epilepsy E. their daughter will likely need lifelong seizure medications

87. A. brain damage is common even after a single seizure B. children who have epilepsy should not participate in contact sports C. children who have epilepsy should not take baths alone D. risk of sudden death is high in children who have epilepsy E. their daughter will likely need lifelong seizure medications

88. You note an upcoming health supervision visit appointment for a 10- year-old child who has myelomeningocele complex. You are seeing him for the first time after your partner, who previously cared for him, retired. In preparation for the visit, you review the child’s medical history and some background on this complex condition. The boy had an open spinal dysraphism repaired at birth. He also had herniation of the cerebellar vermis (a Chiari II malformation) that required surgical decompression and hydrocephalus that necessitated a ventriculoperitoneal shunt. The boy has been stable for 3 years, but according to your reading, he is at risk for acute deterioration due to shunt malfunction or new problems in the brainstem or upper or lower spinal cord. Of the following, the finding that is MOST indicative of potential neurological deterioration in this child is

89. A. double vision B. facial weakness C. gait dysfunction D. impulsive behavior E.unilateral headache

90. A. double vision B. facial weakness C. gait dysfunction D. impulsive behavior E.unilateral headache

91. A female newborn is noted to have a sacral subcutaneous lump with a fatty consistency and a slight lateral deviation of the superior aspect of the intergluteal fold. Her physical examination findings were otherwise normal, and there was no patellar hyperreflexia. Magnetic resonance imaging of the thoracic and lumbar spine was obtained and showed a lipomeningomyelocele. It is unclear if the cord is tethered. The infant was referred to a multidisciplinary spina bifida clinic, where renal ultrasonography was normal and urodynamic assessment showed no evidence of detrusor hyperreflexia or bladder-sphincter dyssynergia. Of the following, the symptom or sign that you are MOST likely to look for during health supervision visits is

92. A. chronic progressive diarrhea B. development of an ataxic gait C. development of a sacral hemangioma D. loss of patellar reflexes E. urinary retention and leakage

93. A. chronic progressive diarrhea B. development of an ataxic gait C. development of a sacral hemangioma D. loss of patellar reflexes E. urinary retention and leakage

94. Quick Associations

95. Infantile Spasms are associated with which neurocutaneous disorder?

96. Tuberous Sclerosis

97. Kids with Spina Bifida are commonly allergic to…

98. Latex