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$1 Million

50:50. Welcome to Who Wants to be a Millionaire. 15. $1 Million. 14. $500,000. 13. $250,000. 12. $125,000. 11. $64,000. 10. $32,000. 9. $16,000. 8. $8,000. 7. $4,000. 6. $2,000. 5. $1,000. 4. $500. 3. $300. 2. $200. 1. $100. Neurology Board Review November 2008.

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$1 Million

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  1. 50:50 Welcome toWho Wants to be a Millionaire 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  2. Neurology Board Review November 2008 Brought to you by Dr. Latour

  3. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  4. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 What finding is suggestive of HA due to increased ICP? 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: Relieved with NSAIDs B: Worse in AM C: + Family history D: Band like pressure

  5. Increased ICP • Headache • Worse in morning, with sneezing, coughing or straining • Relieved with vomiting • Progressive • Frontal, temporal or occipital

  6. Symptoms of ICP • Infant • Bulging or full fontanelle • Separation of sutures • Macrocephaly • Irritability, poor feeding • Vomiting • FTT • “Sunsetting” eyes • High pitched cry • Papilledema (rarely) • Children • Headache • Vomiting • Visual disturbances • Papilledema • Double vision • “Sunsetting” eyes • Personality changes • Poor school performance

  7. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  8. 15 $1 Million 14 $500,000 Which of the following is a cause of pseudotumor cerebri? 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: Albuterol B: Lasix C: Minocycyline D: Azithromycin

  9. Pseudotumor cerebri • AKA Idiopathic Intracranial Hypertension • Diagnosis of exclusion • Lumbar puncture with elevated opening pressure (> 20 cmH20) • Symptoms: headache, double vision, progressive papilledema • Associated with: • Steroids Vitamin A (Isotretinoin) • Antibiotics Lithium • Tetracycline, Minocycline • Obesity Thyroxine • OCPs SLE • MRI study of choice (r/o mass, hydrocephalus) • Treatment: Acetazolamide • Last resort: Optic N. fenestration

  10. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  11. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 An infant with this lesion may experience what type of seizure? 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: Infantile spasms B: Absence C: Benign Rolandic D: Myoclonic

  12. Tuberous Sclerosis • Autosomal dominant • Presence of 2 or more: • Ash leaf spots (seen w/ Woods light) • *** may be present at birth • Periventricular / Cortical tubers • Adenoma sebaceum (present ages 2-10 yrs) • Shagreen patch • Sub / periungual fibroma (after adolescence) • Cardiac rhabdomyoma • Retinal nodular hamartomas • Renal angiomyolipoma • Definite association w/ infantile spasms

  13. Infantile Spasms • Sudden flexion of head / trunk with flexion / adduction of extremities • Peak age 4 – 6 mos • West Syndrome triad: • Infantile spasms • Hypsarrhythmia on EEG • Mental retardation • Spasms occur in clusters • >est upon awakening or falling asleep • Treatment: ACTH

  14. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  15. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 What type of hemorrhage is depicted here? 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: Intraventricular B: Subdural C: Subarachnoid D: Epidural

  16. Head Bleeds • Epidural hematoma • Tear of epidural veins, middle meningeal A. (temporal bone fracture) • Convex lens • Lucid interval • Subdural hematoma • Shearing of bridging veins • “Shaken Baby Syndrome” • Subarachnoid hematoma • Concave shaped • Acute onset, “Worse HA of life”

  17. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  18. 15 $1 Million 14 $500,000 10 yo female with weakness and bilateral ptosis that worsens as the day progresses? What test will help you make the diagnosis? 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: CK B: Tensilon test C: Lumbar puncture D: Muscle biopsy

  19. Myasthenia Gravis • Autoimmune • Ab against Ach receptors in NMJ • >er in females • Weakness worse with activity, improves with rest • + Ocular involvement (ptosis) • Associated with thymoma • Confirmed w/ Tensilon test • Edrophonium (short acting acetylcholinesterase inhibitor) • Transient improvement • Tx • Pyridostigmine (long acting Achnesterase inhibitor) • Curative—Thymectomy

  20. Other types of MG • Congenital • Autosomal recessive • Defect of NMJ • No circulating Ab • Neonatal • Transient • Transplacental transfer of ACh Ab • Weak cry, hypotonia, difficulty feeding, facial weakness, respiratory compromise • Resolves 6-12 wks

  21. Congratulations! Congratulations! Congratulations! You’ve Reached the $1,000 Milestone!

  22. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  23. 15 $1 Million 14 $500,000 13 $250,000 5 yo male with difficulty walking, running into objects. 3 wks prior dx with viral URI. What is the most likely cause of his ataxia? 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 B: Acute Cerebellar Ataxia A: Ataxia Telangiectasia C: Just clumsy D: Toxic Ingestion

  24. Acute Cerebellar Ataxia • Peak onset 2 – 5 years • Preceded by acute febrile illness (wks) • #1 Varicella • Other causes: Rubeola, Mumps, Rubella, EBV, Flu, Mycoplasma, Coxsackievirus • Resolves 6-8 weeks w/o specific therapy • Steroids not indicated

  25. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  26. 15 $1 Million 14 $500,000 13 $250,000 What is the normal head circumference at birth for a full term male? 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: 60 cm B: 40 cm C: 25 cm D: 35 cm

  27. Head Circumference • 50th %tile for HC at birth is 35 cm • Normal growth • 1 cm/month for first 6 mos • 0.5 cm/month from 6 mos – 12 mos • Macrocephaly • 2 std deviations above mean for age/ gender/ GA • May be normal – measure parents HC • Ultrasound • Microcephaly • 2 std deviations below mean age / gender/ GA • CT or MRI

  28. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  29. 15 $1 Million 14 $500,000 13 What disorder can be associated with Tourette Syndrome? $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: ADHD B: OCD C: Both A and B D: None of the above

  30. Tourette Syndrome • Motor and vocal tics present > 1 yr • Peak age of onset 3 – 8 yrs • > er in males • Tics may be suppressed (school), increase with stress • Associated with ADHD and OCD • Tics unmasked with use of stimulant meds • ***Meds do not cause tics, Do not stop meds!!!!

  31. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  32. 15 $1 Million 14 $500,000 What type of inheritance pattern is seen in the disorder depicted in this photo? 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: X linked recessive B: Autosomal dominant C: Autosomal recessive D: Multi-factorial

  33. Duchenne Muscular Dystrophy • X linked recessive • Absence of dystropin • Poor head control may be first sign • Present 2 – 6 yrs: clumsy, “waddling” gait, toe walking • + Gowers sign • Pseudohypertrophy of calves • Fat deposition, collagen proliferation • Associated cardiomyopathy and respiratory compromise • Elevated CK • Confirm: muscle biopsy or gene testing

  34. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  35. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 This mass is associated with? 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: Excessive urination B: Hyperkalemia C: cold intolerance D: All of the above

  36. Craniopharyngioma • Most common supratentorial brain tumor • Arises from Rathke pouch • Symptoms • Visual field defects • Hypothyroidism: cold intolerance, wt. gain, constipation, fatigue • Adrenal failure: hypoglycemia, hyperkalemia, arrhythmias, lethargy, N/V • DI: excessive urination, dilute urine • Growth retardation, delayed puberty • Calcification in sella turcica

  37. Congratulations! Congratulations! Congratulations! You’ve Reached the $32,000 Milestone!

  38. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  39. 15 $1 Million 14 $500,000 13 $250,000 7 yo female with abnormal facial movements and an inability to speak, worse at night when sleeping? Dx? 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: Absence B: Benign Rolandic Sz C: Myoclonic Seizures D: Training to be a mime

  40. Benign Rolandic Seizures • AKA Benign Epilepsy of Childhood • Most common form of epilepsy • Ages 3 – 13 yrs • “Can’t talk” during episode • Abnormal facial movements • Occur when sleeping • + FH • Self-limited, tend to “outgrow”

  41. Epilepsy syndromes of childhood

  42. Buzz words • Treated with ACTH • Infantile Spasms • 3 / second spike and wave • Absence • Treat with ethosuximide • Absence • Associated with tuberous sclerosis • Infantile Spasms • Slow wave EEG • Lennox-Gastaut Syndrome • Life long AED requirement • Juvenile Myoclonic

  43. And yet, more… • “Spaced out” • Absence • Hysparrhythmia • Infantile Spasms • “Sudden loss of muscle tone” • Juvenile Myoclonic • Induced by hyperventilation • Absence • Early AM incoordination / jerking • Juvenile Myoclonic • Loss of consciousness and bladder control • Generalized Tonic-Clonic

  44. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  45. 15 $1 Million What is the chance that a parent with this will transmit to his children?? 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: 25% B: 1% C: 0% D: 50%

  46. NF1 • AKA von Recklinghausen Disease • Autosomal dominant • First degree relative • Café au lait macules ( 6 or more) • > 5 mm prepub, • > 15 mm postpub • Neurofibromas • Increase with puberty and pregnancy • Lisch nodules: dx with slit lamp exam • Can develop pheochromocytoma and renal artery stenosis • Monitor BP closely

  47. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 1 $100

  48. 15 $1 Million 14 $500,000 13 $250,000 12 $125,000 Where is the defect in Werdnig Hoffmann Disease? 11 $64,000 10 $32,000 9 $16,000 8 $8,000 7 $4,000 6 $2,000 5 $1,000 4 $500 3 $300 2 $200 50:50 1 $100 A: Neuromuscular jxn B: White matter of brain C: Anterior horn cells D: Peripheral nerves

  49. Werdnig Hoffmann Disease • AKA Spinal Muscle Atrophy (SMA) type 1 • Autosomal recessive • Degeneration of anterior horn cells • Affects only motor function • Spares sensory • Buzz: “hypotonia”, “poor suck”, “ tongue fasciculations”, “symmetric muscle weakness” • Dx: muscle biopsy or gene testing • Treatment supportive only • Die by age of 2 (respiratory compromise)

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