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meningitis

meningitis

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meningitis

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  1. ACUTE TRANSVERSE MYELITIS Elinore Kaufman MS III

  2. Patient JK: History ? JK is a 46 yo RH woman with a history of diabetes, hypertension and disc disease who presented with a 4d history of neck pain and progressive weakness. Course of illness: ?5d PTA: slight sore throat ?4d PTA: awoke with sore, stiff neck, relieved with ibuprofen. ?3d PTA: difficulty holding pen to sign name ?2d PTA: difficulty walking; dragging right foot.

  3. Patient JK: Physical Exam Vital signs: T: 98.7 P: 86 R: 18 BP: 136/70 SaO2: 98% RA General remarkable only for 2cm papular rash on L upper chest. Neurological examination: Mental Status: intact Cranial Nerves II-XII: intact Motor: Normal bulk and tone in upper and lower extremities. No tremor or asterixis. Strength: ? ? ? ? ? ? ? Delt Bic Tric WE WF FE FF IP Qu HS TA Gas EHL L 5 5 5 5 5 5 5 5 5 4+ 5 5 5 R 4+ 4 4+ 4+ 5 4 4+ 4+ 5 4+ 4+ 5 4 Reflexes: Left: intact. Right: inverted biceps. Babinski: mute Sensation: Light touch intact throughout. Pinprick diminished in R C5 distribution and L leg. Coordination: No evidence of resting or intention tremor or dysmetria, allowing for weakness. Gait: Using walker with good initiation and right steppage gait ? ? ? ?

  4. Now we will examine imaging techniques for the spinal cord and spinal anatomy.

  5. Imaging Myelopathy ? Plain films ? Stability after trauma ? Osteophytic narrowing of spinal canal ? CT: better assessment in trauma ? Spine MRI: MRI: study of choice for cord and soft tissues, as well as for surgical planning ? Gadolinium contrast, especially if AVM is in the differential ? High sensitivity ? CT myelogram if MRI is unavailable or contraindicated ? Invasive ? Identifies only large masses ? Does not distinguish between cystic and solid lesions ? Radionucleide bone scan can be useful in cases of tumor and infection ? Brain MRI ? Associated demyelinating lesions (Seidenwurm 2008)

  6. Companion Patient 1: Normal Cervical Spinal MRI Cerebellum Brain Stem Disc Spinal Cord Nerve Root CSF Spinous process Vertebral Body Sagittal and axial views of cervical spinal cord on T2-weighted MRI (PACS BIDMC ).

  7. Now we will return to our patient JK to discuss her imaging findings and diagnosis.

  8. Patient JK: Sagittal MRI of Cervical Spine Sagittal T2-weighted image showing disc bulge and hyperintense lesion within spinal cord. (PACS BIDMC) Sagittal T1-weighted image after IV gadolinium administration showing ring enhancing lesion. (PACS BIDMC)

  9. Patient JK: Enhancing lesion on axial T1-weighted Image with Contrast NORMAL Enhancing lesion post-contrast (PACS BIDMC) seen on axial T1 image,

  10. Work-up of Acute Myelopathy Patient JK Adapted from Jacob 2008

  11. Differential Diagnosis of Acute Myelopathy Inflammatory Noninflammatory ? Demyelinating disease ? Multiple sclerosis (MS) ? Neuromyelitis optica (NMO) ? Acute disseminated encephalomyelitis (ADEM) ? Idiopathic acute transverse myelitis (IATM) ? Compression ? Osteophyte ? Disc ? Metastasis ? Trauma ? Infection ? Viruses: coxsackie, mumps, varicella, CMV ? Tuberculosis ? Mycoplasma ? Tumor ? Paraneoplastic syndromes ? Inflammatory disoders ? Systemic lupus erythematosus ? Neurosarcoidosis Jacob 2008, Seidenwurm 2009

  12. Imaging Findings in ATM Condition MRI Spinal Cord MS Oval‐shaped lesion(s) < 2 spinal cord  segments, usually peripheral location.   Hyperintense on T2 with  homogeneous or ring enhancement. MRI Brain White matter lesions:  periventricular, juxtacortical,  infratentorial lesions.  Dawson’s Fingers. NMO lesion >3 segments;  cord swelling and  gadolinium enhancement acutely. Optic neuritis.  Cerebral lesions in 60%. ADEM lesion >3 segments;  cord swelling and  gadolinium enhancement acutely. Lesion of variable length and shape,  >50% of cross‐sectional area on axial  scan.  Hyperintense on T2‐weighted   images, variable hypointensity on T1,  patchy enhancement and swelling.  Large, confluent white matter  lesions. No brain lesions. IATM Jacob 2008, O’Riordan 1996, Scotti 2001, Tartaglino 1996

  13. Idiopathic Acute Transverse Myelitis ? Incidence: 1-4 per million per year ? Diagnosis ? Myelopathic symptoms ? 50% lose all leg movements ? Nearly 100% have bladder dysfunction ? 80-94% of patients sensory dysfunction ? MRI evidence of noncompressive myelopathy ? Inflammation: CSF pleocytosis, elevated CSF IgG index, or gadolinium enhancement ? if no evidence but strong suspicion, repeat MRI/LP in 2-7 d ? Exclude: cord radiation, thromboembolic disease, AVF, connective tissue disease, infection, MS/ADEM, malignancy ? Pathology: edema, necrosis, demyelination, white>gray matter changes (Misra 1996) ? Prognosis ? 1/3 recover without sequelae ? 1/3 have moderate, permanent disability ? 1/3 have severe disability (Transverse Myelitis Consortium Working Group 2002)

  14. Radiologic Findings in IATM ? Of patients with ATM and normal brain MRI, none with normal CSF progressed to MS. ? 53% of those with CSF oligoclonal bands or elevated IgG index developed MS. (Perumal 2008) ? Spinal cord atrophy may be evident on follow-up MRI. (Shen) ? Fractional anisotropy ? Shows water diffusion in the extracellular space along the axon fibers ? Higher sensitivity than T2 for white matter damage: can identify lesions in normal-appearing cord ? Decreased values may demonstrate increased extracellular space due to demyelination, and may predict greater wallerian degeneration and worse prognosis. (Renoux 2006, Lee 2008)

  15. Now we will examine other causes of acute transverse myelitis.

  16. Companion Patient 2: Transverse Myelitis in MS Axial T2-weighted images from MS patient (Jacob 2008) ? MRI Spine ? Lesions <2 segments ? No cord swelling ? Peripheral lesions (Jacob 2008) ? Differential: 1-3% of lupus patients develop ATM with similar findings to MS. (Scotti 2001) Sagittal T2-weighted image from MS patient (Scotti 2001)

  17. Risk of Developing MS after ATM ? MR Brain ? >= 2 lesions: 88% develop MS. (Jacob 2008) ? No brain lesions: 10-33% develop MS. ? Mean time to MS: 16m, no new cases >24m. ? Next evidence of MS is more likely to be clinical than MRI based (serial MRI does not speed diagnosis ? African American ATM patients are more likely to develop MS than Caucasian patients: (35% vs. 26%). ? African Americans develop MS more quickly, in 9.6 months vs. 20.3 for Caucasions. ? African Americans experience greater disability from this disease despite earlier use of disease-modifying therapy. (Perumal 2008).

  18. Companion Patient 3: Dural arteriovenous fistula on MRI Longitudinal hyperintense lesion on T2; contrast enhancement of dilated blood vessels: AVF (Jacob 2008) ? AVMs and dural arteriovenous fistulae can either bleed or cause increased venous pressure and progressive myelopathy; rarely acute onset. ? Anterior spinal artery occlusion: atherosclerosis or complication of aortic surgery: peak within 4h (Jacob 2008) ? Vasculitis can cause cord swelling followed by cord atrophy. (Scotti 2001)

  19. Companion Patients 4 & 5: Spinal Cord Tumor on MRI Sagittal T2 image of lesion initially thought to be tumor, ultimately determined to have an inflammatory cause. (Brinar 2006) Sagittal T2 image of lesion initially thought to be inflammatory, ultimately determined to be a tumor. (Jacob 2006)

  20. Companion Patient RB: History and Physical Exam ? RB is a 32yo LH woman who presented with severe headache and worsening neurological symptoms: Course of illness: ? 3 wks of severe R-sided retro-orbital headache ? 2 wk of numbness in both legs up to the navel ? 1 wk of R eye blurry vision ? 3d of urinary retention ? 1d of bowel incontinence ? Physical exam confirmed severe vision loss, decreased pinprick sensation to the T8 level, and upper motor neuron weakness in the legs.

  21. Companion Patient RB: Spinal lesions and leptomeningeal enhancement on MRI Post-contrast T1-weighted image showing three enhancing lesions and linear leptomeningeal enhancement. (PACS BIDMC)

  22. Companion Patient RB: Clinical Course ? She was treated with acyclovir (possible CNS HSV), ciprofloxacin (E. Coli UTI) and methylprednisolone. ? Her vision improved, and she was discharged ? 1 wk later, she returned with fever and severe L-sided retro-orbital headache. Her other symptoms were stable, but her vision was now extremely blurry in her left eye. ? She was readmitted and received plasmapheresis with slight improvement, but her symptoms worsened again after discharge.

  23. Companion Patient RB: Diagnostic Work-Up ? Brain MRI showed only the optic neuritis. ? CSF showed pleocytosis and elevated protein but no oligoclonal bands. ? Extensive CSF and serum studies showed no evidence of CNS infection, collagen vascular disease or malignancy. ? Preliminary diagnosis: Neuromyelitis Optica ? Atypical features ? Spinal cord lesion length <3 segments ? Presence of leptomeningeal enhancement more typically associated with lymphoma, sarcoidosis or Lyme disease ? Absence of NMO antibody in serum and CSF. ? Chest CT showed no evidence of sarcoidosis or lymphoma ? PET scan was performed to rule out lymphoma and showed no lymphadenopathy, bone lesions, or other abnormal uptake. ? Multiple sclerosis

  24. Summary ? Acute myelopathy is most often compressive but otherwise may be infectious, inflammatory or idiopathic ? MRI is the imaging modality of choice for the spinal cord, although CT myelography may be used if needed. ? Imaging locates the lesion and can provide clues to the diagnosis but often leaves a wide differential. ? MS: other CNS lesions ? NMO: lesion >3 segments ? Idiopathic ATM: diagnosis of exclusion

  25. Acknowledgements Many thanks to: ? Dr. Daniel Cohen, Dr. Jed Barasch, Dr. Jennifer Avallone and Dr. Courtney McIlduff of the BIDMC Neurology service ? Maria Levantakis ? Dr. Gillian Leiberman

  26. References R, Kinkel cases of myelitis: comparison between patients with and without multiple sclerosis. Eur J Neurol 1998;5:35–48. Brinar M, Rados M, Habek M, PoserCM.Enlargement neoplasm? Clin Neurol Neurosurg 2006;108(3):284–9. Brinar VV, Habek M, Zadro I, Barun B, Ozretić diagnosis of transverse myelopathies.Clin 27. Davis GA, Klug GL. Acute-onset nontraumatic embolism or acute myelitis? Childs Nerv Fux CA, Pfister S, Nohl F, Zimmerli S. Cytomegalovirus-associated acute transverse myelitis in immunocompetent adults. Clin Irani DN, Kerr DA. 14-3-3 protein in the cerebrospinal fluid of patients with acute transverse myelitis. Lancet 2000;355:901. Jacob A, Das K, Boggild M, Buxton N. Inflammation or neoplasm? Another side to the story. Clin Neurol Neurosurg. 2006 Dec;108(8):811-2. Jacob A, Weinshenker BG. An approach to the diagnosis of acute transverse myelitis. Semin Neurol. 2008 Feb;28(1):105-20. Kerr DA, Ayetey H. Immunopathogenesis of acute transverse myelitis.. Curr Neurol. 2002 Jun;15(3):339-47. Lee JW, Park KS, Kim JH, Choi JY, Hong SH, Park SH, Kang HS. Diffusion Tensor Imaging in Idiopathic Acute Transverse Myelitis Bakshi PR, Mechtler LL, et al. Magnetic resonance imaging findings in 22 of the spinal cord: Inflammation or D, Vranjes Neurol D. Current concepts in the Neurosurg. 2008 Nov;110(9):919- paraplegia in childhood: fibrocartilaginous Syst 2000;16:551–554. Microbiol Infect. 2003 Dec;9(12):1187-90. Opin AJR:191, August 2008 W53-57

  27. References, continued. Misra UK, Kalita transverse myelitis. J Neurol O’Riordan J, Kumar S. A clinical, MRI and neurophysiological Sci. 1996 Jun;138(1-2):150-6. study of acute JI, Gallagher HL, Thompson AJ, et al. Clinical, CSF, and MRI findings in Devic’s neuromyelitis optica. J Neurol Neurosurg Perumal J, Zabad R, Caon C, MacKenzie Khan O. Acute transverse myelitis with normal brain MRI : long-term risk of MS. J Neurol. 2008 Jan;255(1):89-93. Renoux J, Facon D, Fillard P, Huynh I, Lasjaunias imaging and fiber tracking in inflammatory diseases of the spinal cord. Am J Neuroradiol 2006; 27:1947–1951 Scott TF, Kassab SL, Singh S. Acute partial transverse myelitis magnetic resonance imaging: transition rate to clinically definite multiple sclerosis. Mult Scler. 2005 Aug;11(4):373-7. Scotti G, Gerevini S. Diagnosis and differential diagnosis of acute transverse myelopathy: the role of neuroradiological Neurol Sci 2001;22(suppl 2):S69–73 Seidenwurm DJ, Wippold FJ, Brunberg JA et al. American College of Radiology Appropriateness Criteria: Myelopathy. Updated 2008. Accessed September 17, 2009 at http://www.acr.org/SecondaryMainMenuCategories/quality_safety/app_criteria/pdf/Ex pertPanelonNeurologicImaging/MyelopathyDoc8.aspx. Shen WC, Lee SK, Ho YJ, Lee KR, Mak SC, Chi CS. MRI of sequela myelitis. Pediatr Radiol. 1992;22(5):382-3. Tartaglino LM, Croul SE, Flanders AE, Sweeney JD, Schwartzman RJ, Liem Idiopathic acute transverse myelitis: MR imaging findings. Amer Dec;201(3):661-9. Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002 Aug 27;59(4):499-505. Psychiatry 1996;60:382–387. A, Bao F, Latif Z, Zak I, Lisak M, Tselis R, P, Ducreux D. MR diffusion tensor with normal cerebral investigations and review of the literature. of transverse M. A Radiology. 1996

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