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Case Conference

April 18, 2012. Case Conference. Patient…. 16 year old Male presenting with right sided weakness. History of Present Illness. 7 days prior to admission. Gradual onset of right sided weakness (clumsy, could not write well) Complains of poor sensation over the right face.

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Case Conference

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  1. April 18, 2012 Case Conference

  2. Patient… • 16 year old Male presenting with right sided weakness

  3. History of Present Illness 7 days prior to admission • Gradual onset of right sided weakness (clumsy, could not write well) • Complains of poor sensation over the right face. • Brought to an emergency room; Dx: Nerve impingement  Reassurance • No relief of symptoms • Was transferred to another correctional facility • Symptoms got worse • Can’t walk straight • Loss some feeling over the right leg. Day of admission

  4. History

  5. Physical Examination

  6. Neurologic Examination 2+ 4/5 2+ 5/5 3+ 4/5 2+ 5/5

  7. Differential Diagnosis • Neurologic • Ischemic vs Hemorrhagic Stroke • Peripheral nerve disease • Demyelinating Disease

  8. And so the story goes… • Concerned about ischemic stroke • Labs were ordered • IVF started • MRI obtained

  9. Laboratory Tests CBC Chemistries

  10. Laboratory Tests CHEMISTRIES OTHER TESTS

  11. Cerebrospinal Fluid Studies • CSF Cell count – normal • CSF Proteins – 23 • CSF Glucose – 79 • CSF Oligoclonal bands - Negative

  12. MRI (1/25/2012)

  13. MRI (1/25/2012)

  14. MRI (1/25/2012)

  15. Vincent Patrick Uy, MD PGY-1 The Child with A Demyelinating Disease

  16. Introduction • No single diagnostic test is specific to particular diagnosis • Must rely on History and PE, clinical features and imaging to arrive at a plausible diagnosis • Uncommon in the pediatric age group • Think of RARE disorders!

  17. The Working Diagnosis • Acute Disseminated Encephalomyelitis • Multiple Sclerosis • Optic Neuropathy • Transverse Myelitis • NeuromyelitisOptica

  18. Pathophysiology

  19. Leukodystrophies • Adrenoleukodystrophies • Cytoskeletal disorders (Alexander’s disease) • Myelin Basic Protein disorder • Organic acid diseases (Canavan disease) • Disorders of energy metabolism (MELAS) • Krabbe disease

  20. Malignancies • CNS lymphomas • High-grade glioma • Large area of tumefactive demyelination • CSF studies show atypical cells

  21. Collagen Vascular Diseases • Systemic Lupus Erythomatosus • Behcet’s disease • Neurosarcoidosis • Look for other symptoms • Anti-dsDNA, anti-phospholipids, elevated ACE enzymes

  22. Optic Neuritis • May be a clinically isolated syndrome or part of another condition • Monocular in most adults; bilateral in most children 12-15 years old. • Symptoms can progress from several hours to days • Risk factor for developing frank Multiple Sclerosis

  23. Optic Neuritis • Headache • Painful eye movements • Partial or complete vision loss • Relative afferent pupillary defect • Papillitis (75% in the acute phase)

  24. Optic Neuritis

  25. Transverse Myelitis • Spinal cord disease without evidence of compression • Inflammation of the spinal cord • May present as a clinically isolated disorder or part of other conditions. • Most cases are idiopathic • Subset of patients develop TM after vaccination

  26. Transverse Myelitis • Weakness, paresthesias, urinary and bladder problems • Hyperacute presentation • MRI shows contrast enhancing lesions on the cord

  27. Transverse Myelitis • Idi0pathic Transverse Myelitis • Spontaneous but partial recovery in 1-3 months • 40% will have a persistent disability

  28. NeuromyelitisOptica • Devic’s Disease • Combination of: • Optic neuritis • Transverse Myelitis • Seropositivity to NMO IgG antibodies • >3 contiguous spinal levels involved • Presentation can be primarily progressive with a fulminant course on presentation

  29. Acute Disseminated Encephalomyelitis • Acute or subacute onset of multifocal neurologic deficits with encephalopathy • Often follows a viral infection or vaccination • Very uncommon illness • Autoimmune disorder of the CNS, triggered by environmental factors, in genetically susceptible individuals

  30. Acute Disseminated Encephalomyelitis • Patients with ADEM, no matter what form, should have complete recovery to baseline.

  31. Acute Disseminated Encephalomyelitis • Clinical Presentation • Febrile illness occurs in about 50-75% of children a month before neurologic symptoms appear. • Headache and meningeal signs • Encephalopathy (changes in mental status) • Acute hemiparesis, cerebellar ataxia, optic neuritis and spinal cord dysfunction are also common

  32. Acute Disseminated Encephalomyelitis

  33. Acute Disseminated Encephalomyelitis

  34. Acute Disseminated Encephalomyelitis

  35. Acute Disseminated Encephalomyelitis • Treatment • Fever, meningisimus, headaches, change in mental state, etc. Treat for suspected meningitis/encephalitis • Imaging may be obtained • High dose IV Glucocorticoids treatment for 3-5 days + steroid taper for 4-6 weeks • Methylprednisolone (10-30 mg/kg/d) • Dexamethasone (1 mg/kg/d)

  36. Pediatric Multiple Sclerosis • Chronic disease characterized by repeated episodes of demyelination separated by space and time • Pediatric multiple sclerosis  age of onset before 16 years old • 2.5/100,000 children

  37. Pediatric Multiple Sclerosis • Genetic Susceptibility • Higher risk in monozygotic twins • Certain HLA subtypes are associated with increased risk • Environmental factors • Epstein-Barr Virus infection • Vitamin D deficiency

  38. Pediatric Multiple Sclerosis

  39. Pediatric Multiple Sclerosis

  40. Pediatric Multiple Sclerosis • Diagnostic Criteria for Diagnosis of MS • Dissemination in Space • Dissemination in Time

  41. Pediatric Multiple Sclerosis • Dissemination in Space • T2 lesions on MRI in at least two of the four MS-typical regions of the brain: • Periventricular • Juxtacortical • Infratentorial • Spinal Cord • Development of a further clinical attack implicating a different CNS site

  42. Pediatric Multiple Sclerosis • Dissemination in Time • Simultaneous presence of asymptomatic gadolinium-enhancing and non enhancing lesions at anytime or a new T2 lesion on a follow-up MRI (irrespective of timing) • Development of a second clinical attack

  43. Pediatric Multiple Sclerosis • Clinically Isolated Syndrome • INCREASED RISK for MS • Age 10 years or older • Optic nerve lesions • MRI pattern very typical for MS • DECREASED RISK for MS • Spinal cord lesions • Acute Mental status change (ADEM)

  44. Pediatric Multiple Sclerosis • Treatment strategy (Acute setting) • IV pulse methylprednisolone 20-30 mg/kg/d x 5 days • Steroid taper 1 mg/kg/day for 4-6 weeks if without complete resolution • May re-treat with IV pulse methylprednisolone if symptoms recur during steroid taper

  45. Pediatric Multiple Sclerosis • Treatment strategy (Chronic Therapy) • Glatiramer acetate • Interferons

  46. Pediatric Multiple Sclerosis Monitoring patients after and ACUTE ATTACK Completion of steroid taper Neurologic exam at 1/3/6 months thereafter Clinically stable Symptomatic Send for MRI Annual Physical Examination

  47. Patient Update… • Received 5 day high dose methylprednisolone therapy via IV • Completed steroid taper with prednisone PO • On follow-up 4/3/2012  Complete resolution of symptoms with return to baseline • Dismissed as a case of Acute Demyelination – Clinically Isolated Syndrome

  48. Let’s demyelinate YOUR neurons! A 9 year old girl with anxiety disorder was diagnosed with acute demyelination because of sudden onset of left sided weakness and ataxia. Typical MS lesions which included the cerebellum and spinal cord were discovered after MRI. She comes to the clinic for the first time after completing 6 weeks of steroid taper. On further questioning, mom has missed several doses of her prednisone at home. So far, she never experienced symptoms again. Mom is concerned that the catastrophe might happen again. Which of the following conditions increases her risk to develop frank multiple sclerosis? • Non compliance to steroid taper • Her young age • Typical lesions were seen on MRI • Concomitant anxiety disorder • She doesn’t have risk factors that increase her risk to develop MS.

  49. Now let’s give YOU ADEM! A 17 year old boy was brought to the ER for change in mental status. Ultimately, he was diagnosed with ADEM. He recovered after 5 days in the ICU and is now back to baseline. Which of the following environmental triggers could have increased his risk for developing the condition. • Receiving an unrecalled vaccine from the PMD a month ago during annual visit • Getting daily morning sun exposure for 15-20 minutes • Blurring of vision without eye pain • Experiencing an exudative sore throat which was negative on rapid strep test • Spider bite 2 weeks ago

  50. Thank You!

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