1 / 33

A Case Presentation & Discussion

Bobbye Thompson, MD University of Texas Medical Branch, Galveston Division of Neurosurgery. A Case Presentation & Discussion. History. 51yo F w/several months progressive BLE weakness; pain greater in LLE Bilateral numbness just above breasts & inferiorly

salena
Download Presentation

A Case Presentation & Discussion

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. Bobbye Thompson, MD University of Texas Medical Branch, Galveston Division of Neurosurgery A Case Presentation & Discussion

  2. History • 51yo F w/several months progressive BLE weakness; pain greater in LLE • Bilateral numbness just above breasts & inferiorly • Muscle aches cramps spasms falls • Urinary incontinence & saddle anesthesia • Constipation 2wks (manual disimpaction) • No history of spinal operation, injury, or trauma • No F/C/N/V

  3. Physical Exam • Sensory deficit inferior to T1 bilaterally • Decreased rectal tone • Motor: LLE 2/5, RLE 5/5 • Increased tone/spasticity • Hyperreflexia • Babinski: Bilateral upgoing toes • Gait: Able to stand with asisstance; drags LLE, circumduction

  4. Sag T1 FS MRI Post Sag T2 MRI

  5. Labs/CSF • Gram stain & culture: rare monocytes, no organisms isolated • CSF glucose: 69 • CSF protein: 31 • Oligoclonal bands: negative

  6. Differential Diagnosis • Tumor • Astrocytoma • Ependymoma • Demyelinating lesion: • Multiple sclerosis • Neuromyelitis optica • Transverse myelitis • ADEM

  7. Impression • Progressive LE weakness • Imaging consistent with Intramedullary tumor at T1 • Oligoclonal bands negative • Probable tumor, likely glioma • Proceed with C6-T2 laminectomy with biopsy and/or resection • Midline myelotomy firm, tan tissue • Frozen section: gliosis vs. low-grade glioma • Inflammatory infiltrate

  8. Surgical Pathology

  9. CD68

  10. CD45

  11. Diagnosis • Inflammatory demyelinating lesion • Multiple Sclerosis • No further surgical intervention • Neurology for management of MS • Follow up in clinic 2 months postop • Motor LLE improved • MRI Brain & C-spine

  12. MRI 2 mos Post Op

  13. Multiple Sclerosis (MS) Neuromyelitis Optica (NMO) Transverse Myelitis Acute Disseminated Encephalomyelitis (ADEM) Demyelinating Diseases Mimicking Tumors

  14. Multiple Sclerosis • Relapsing-remitting MS (RRMS): • Most common • 85% of MS initially diagnosed • Partial or total recovery between attacks • Secondary-progressive MS (SPMS): • RRMS course, but becomes gradually progressive • Attacks & partial recoveries may continue to occur • Over 60% initially RRMS progress to SPMS in 10 yrs

  15. Multiple Sclerosis • Primary-progressive MS (PPMS): • Progressive from onset • Symptoms generally do not remit • Progressive disability w/o acute attacks • 15% of MS initially diagnosed • Primary-relapsing MS (PRMS): • Same as PPMS, but with acute attacks

  16. Multiple Sclerosis • Clinical: Episodic, relapsing-remitting neurologic symptoms in a young adult (typically) • Neurological symptoms disseminated in time & space • Common presentations: monocular visual disturbances (optic neuritis), paresthesias/weakness (myelitis), incoordination (cerebellar), and/or diplopia (brainstem) • Labs: Oligoclonal bands positive, MBP elevated • Not specific, new tests improving sensitivity/specificity • Early MS vs Clinically definite • MBP elevated in various disease processes • MRI: T2 intense foci in white matter (UBOs), juxtacortical (G-W junction involving U-fibers), periventricular lesions, involve corpus collosum (perpendicular extensions)

  17. MS C-spine

  18. Neuromyelitis Optica/Devic • Clinical: Visual loss eye(s) w/myelopathy, usually over several months; may occur simultaneously. Course can be monophasic, but ~2/3 relapsing. • In children: usually monophasic & preceded by infection. Recovery is often complete. ADEM variant. • Labs: Oligoclonal bands negative. NMO-IgG serum can be sent (indirect immunofluorescence assay) • MRI: Spinal cord lesions extend > 3 levels, w/o brain lesions. SC lesions are cavitating, necrotic, w/acute axonal pathology.

  19. NMO MRI T2

  20. Transverse Myelitis • Clinical: focal inflammatory disorder of spinal cord; acute or subacute combination of motor, sensory, and autonomic deficits. • Up to 50% pt will have LE paralysis/paresis • >80% have numbness, paresthesias, or dysesthesias, often with a well-defined sensory level • vast majority experience impaired bladder function. • 1/3 complete recovery, 1/3 moderate disability, 1/3 severe disability. • Labs/MRI/Treatment: Variable

  21. Transverse Myelitis • Findings that herald diagnosis of MS: • asymmetric clinical findings • predominance of sensory deficits • MRI lesions <3 spinal cord segments, • MRI Brain: occult white matter lesions (“dirty white matter”) • CSF: Oligoclonal bands positive

  22. Acute Disseminated Encephalomyelitis(ADEM) • Clinical: “Postinfectious Encephalomyelitis” • Presents as ACUTE neurological symptoms • Fever, nausea, vomiting, positional vertigo, and altered consciousness (drowsy or lethargic) • Children following viral infxn or immunization • Labs: ESR, CRP, CSF pleocytosis • MRI: extensive lesions, many enhance w/gad (ring-enhancing lesions) • Basal ganglia and/or thalami involvement • Spares corpus collosum • Molecular mimicry: viral epitope & myelin epitope

  23. ADEM MRI - Brain

  24. ADEM MRI - Spine

  25. Other Differentials • Sjögren’s syndrome: mimics MS by producing recurrent multifocal neurological manifestations; white-matter lesions on MRI and oligoclonal bands in CSF. • Differentiate by: systemic manifestations (sicca-dry eyes, mouth & rheumatic features), abnormal serology (antinuclear, SSA-Ro, or SSB-La antibodies), and findings of inflammatory foci on salivary gland biopsy.

  26. Other Differentials • Behçet’s disease: Most common CNS presentation is a subacute brainstem syndrome. • Differentiate by: recurrent oral ulcerations (at least 3 times in 12 months), MRI in Behçet’s disease: brain abnormalities tend to be large, diffuse lesions confined to the brainstem.

  27. Red Flags Atypical features that may portend alternative diagnosis (not MS): • Onset: too early/late (before 15-20y, after 50y) • Early onset may point to a genetic etiology • Family history: less likely MS (weak genetic assoc) • Normal CSF, MRI, and/or Evoked potential studies • Systemic signs • Anemia • Angiokeratomas • Cardiomyopathy • Proteinuria • Metabolic acidosis

  28. Summary

  29. Summary • History & Physical Exam, Labs & MRI findings, AND exclusion of alternative diagnoses. • ~90% of patients ultimately diagnosed with MS initially present with a clinically isolated syndrome (CIS), such as optic neuritis. • MRI can be valuable in differentiating. • American Academy of Neurology: on value of MRI predicting eventual conversion of a clinically isolated syndrome to clinically definite MS: • >3 white-matter lesions in T2 MRI is sensitive predictor (>80%) of clinically definite MS within 7-10 years • In Normal MRIs, <20% will have 2nd attack w/in 10y

  30. The Future • Development of more highly specific criteria for this spectrum of diseases. • Development of more reliable biomolecular markers. Always consider demyelinating diseases in the differential diagnosis of enhancing intramedullary lesions. • If index of suspicion is high, further testing may be warranted.

  31. 1.Natowicz MR, Bejjani B. Genetic disorders that masquerade as multiple sclerosis. Am J Med Genet. 1994;49:149-169. 2.Trojano M, Paolicelli D. The differential diagnosis of multiple sclerosis: classification and clinical features of relapsing and progressive neurological syndromes. Neurol Sci. 2001;22(suppl 2):S98-S102. 3.Frohman EM, Goodin DS, Calabresi PA, et al. The utility of MRI in suspected MS: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2003;61:602-611. 4.Brinar VV. Non-MS recurrent demyelinating diseases. Clin Neurol Neurosurg. 2004;106:197-210. 5.Wingerchuk DM. Postinfectious encephalomyelitis. Curr Neurol Neurosci Rep. 2003;3:256-264. 6. Alexander EL, Malinow K, Lejewski JE, Jerdan MS, Provost TT, Alexander GE. Primary Sjogren’s syndrome with central nervous system disease mimicking multiple sclerosis. Ann Intern Med. 1986;104:323-330. 7 Siva A, Altintas A, Saip S. Bechet’s syndrome and the nervous system. Curr Opin Neurol. 2004;17:347-357. 8.Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B. Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients. Neurology. 2001;56:1313-1318. 9.Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002;59: 499-505. 10.Sakakibara R, Hattori T, Yasuda K, Yamanishi T. Micturition distur- bance in acute transverse myelitis. Spinal Cord. 1996;34:481-485. 11.Bakshi R, Kinkel PR, Mechtler LL, et al. Magnetic resonance imaging findings in 22 cases of myelitis: comparison between patients with and without multiple sclerosis. Eur J Neurol. 1998;5:35-48. 12.Cree BAC, Goodin DS, Hauser SL. Neuromyelitis optica. Semin Neurol. 2002;22:105-122. 13.Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53:1107-1114. 14.Rodriguez M, Siva A, Cross SA, O’Brien PC, Kurland LT. Optic neuritis: a population-based study in Olmsted County, Minnesota. Neurology. 1995;45:244-250. 15.Wingerchuk DM, Pittock SJ, Lennon VA, Lucchinetti CF, Weinshenker BG. Neuromyelitis optica diagnostic criteria revisited: validation and incorporation of the NMO-IgG serum autoantibody [abstract]. Neurology. 2005;64(suppl 1):A38. 16.Pittock SJ, Wingerchuk DM, Krecke K, Lennon VA, Lucchinetti CF, Weinshenker BG. Brain abnormalities in patients with neuromyelitis optica (NMO) [abstract]. Neurology. 2005;64(suppl 1):A39. 17.Lucchinetti CF, Mandler RN, McGavern D, et al. A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica. Brain. 2002;125:1450-1461. 18.Bergamaschi R, Tonietti S, Franciotta D, et al. Oligoclonal bands in Devic’s neuromyelitis optica and multiple sclerosis: differences in repeated cerebrospinal fluid examinations. Mult Scler. 2004;10:2-4. 19.Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364:2106-2112. 20.Sourander P, Walinder J. Hereditary multi-infarct dementia. Morphological and clinical studies of a new disease. Acta Neuropathol (Berl). 1977;39:247-254. 21.Haritoglou C, Rudolph G, Hoops JP, Opherk C, Kampik A, Dichgans M. Retinal vascular abnormalities in CADASIL. Neurology. 2004;62: 1202-1205. 22.Viitanen M, Kalimo H. CADASIL: hereditary arteriopathy leading to multiple brain infarcts and dementia. Ann N Y Acad Sci. 2000;903:273-284. 23.Desmond DW, Moroney JT, Lynch T, Chan S, Chin SS, Mohr JP. The natural history of CADASIL: a pooled analysis of previously published cases. Stroke. 1999;30:1230-1233. 24.Chabriat H, Levy C, Taillia H, et al. Patterns of MRI lesions in CADASIL. Neurology. 1998;51:452-457. 25.Joutel A, Vahedi K, Corpechot C, et al. Strong clustering and stereo- typed nature of Notch3mutations in CADASIL patients. Lancet. 1997; 350:1511-1515. 26.Joutel A, Dodick DD, Parisi JE, Cecillon M, Tournier-Lasserve E, Bousser MG. De novo mutation in the Notch3gene causing CADASIL. Ann Neurol. 2000;47:388-391. 27.De Strooper B, Annaert W, Cupers P, et al. A presenilin-1-dependent -secretase-like protease mediates release of Notch intracellular domain. Nature. 1999;398:518-522. 28.Brex PA, Ciccarelli O, O’Riordan JI, Sailer M, Thompson AJ, Miller DH. A longitudinal study of abnormalities on MRI and disability from multiple sclerosis. N Engl J Med. 2002;346:158-164. 29.Rudick RA, Schiffer RB, Schwetz KM, Herndon RM. Multiple sclerosis: the problem of incorrect diagnosis. Arch Neurol. 1986;43:578-583. 30. Images from wikicommons, mmorris.com, devic.org References

More Related