Usa confirance


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Usa confirance

  1. 1. INVESTIGATIONS Mayo clinic extensive work –up negative Serology, CT Body, Mammogram, testicular U/s Pet Congectival, Lung parenchymal biopsies. ACE,ANA ssB minimally elevated one patient each Para neoplatic screen negative in all 5 of 6 mayo Pets tested (4 serum: 2CSF) NMO-lgG Negative in 4 all checked
  2. 2. PATHOLOGY 4 Patient biopsied (3 Mayo: Belgium):3 cerebellum 1 Pons Marked lymphocytic infiltrate (Predominantly CD3 reactive T lymphocytes, Some CD20 positive B lymphocytes. CD68 positive histiocytes and activated microglia present) White matter with peri vascular predominance and more diffuse parenchyma inflammatory infiltrate Myelin intact, special stains for fungi, Mychobecteria negative No characteristic finding of sarcoidosis, histiocytosis, lymphoma, lymphomatoid, granulom atosis Multiple sclerosis or other disease
  3. 3. TREATMENT AND OUT COME 7 Patient 1g Iv methylprednisolone all improved initially. PO prednisone in 1 without marked early improvement. Varied long-term outcome- ranged from excellent to incomplete with substantial deficits remaining Myelopathy in Belgium case.
  4. 4. CONCLUSION Definable, treatable, inflammatory, CNS brainstem- predominant syndrome Similar clinical, Radiological, Pathological Syndrome responsive to Immunosuppression especially steroids No other diseases found despite extensive and prolonged follow –up Difficulty biopsy: rule out other competing, diseases, consider biopsy Therapy with high dose corticosteroids. Prolonged therapy commonly needed, immunosuppression with steroid- sparing
  5. 5. EMERGING THERAPIES IN MS Natalizumab: Monitoring advances serum JCV antibody test projecting PML risk Combination Therapy: Await combi Rx interferon B -1a IM/glatiramer acetate Fingolimod: first approved oral MS Medication: long term safety
  6. 6. EMERGING THERAPIES IN MS - ORAL MEDICATION Cladribine: approved in Russia and Australia disappoint reviews at FDA Europe: helted development Laquinimode: mixed results: BRVO missed reducing relapse (Primary end point) BG00012: Promising results MRI and relapse Teruflunimide: early promising results MRI and relapse
  7. 7. DIFFERENTIAL DIAGNOSIS OF ACUTE TRANSVERSE MYELITISDemyelinating Disorders Parasitic Systemic sclerosisMultiple sclerosis Neurocysticercosis NeurocysticercosisNeuromyelitis optica Schistosoma Behçet syndromeAcute disseminated Gnathostoma Vascular DisorderEncephalomyelitis Angiostrongylus Anterior and posterior spinal artery infarctionIdiopathic Toxocara Arteriovenous fistula
  8. 8. Postvaccinial Viral (human T-cell Hematomyelia lymphotropic virus and (arteriovenousRabies HIV cause more chronic malformation, myelopathies) cavernoma,Diphtheria-tetanus-polio bleeding diathesis, Herpesvirus: herpes Osler-Weber-RenduSmallpox simplex virus, syndrome)Measles varicella-zoster virus cytomegalovirus,Rubella human herpes virus Fibrocartilaginous disk types 6 and 7 embolismJapanese B encephalitis Epstein-Barr virus NeoplasticEpstein-Barr virus Flaviviruses: Dengue Primary intramedullary feverJapanese B B tumors (lymphoma,Pertussis encephalitis, st Louis ependymoma, encephalitis tickborn actrocytoma, andInfluenza encephalitis, West hemangionlastoma Nile virus or metastaticHepatitis B intramedullary tumors
  9. 9. Infection Orthomyxovirus: Paraneoplastic (mayBacterial Influenza A virus also cause chronic Spinal cord abscess myelopathy)(epidural or Paramyxovirus:intraparenchymal) Measles virus Lung and breastdue to spread from Mums virus carcinomas mostsystemic infection common Picornaviruses:Myco plasma Coxackievirus types A Amphiphysin andBorrelia burgdorferi AndB, echoviruses Collapsein Enteroviruses 70 and 71 Response- mediatorTreponema pallidum hepatitis A and C protein 5-lgG most common autoantibodyMyconacterium Poliovirus types I,2 associationsTuberculosis And 3Actinomyces Other inflammatory DisordersFungal Systemic lupus Blastomyces Erythematosus Coccidiodes Siogren syndrome Cryptococcus Mixed connective tissue Aspergillus disorder
  10. 10. DIFFERENTIAL DIAGNOSIS OF CRONIC MYELOPATHIESIdiopathic Inflammatory Spinocerebellar ataxiasDemyelinating Diseases ALSPrimary progressive Vascularmultiple sclerosis Cerebral autosomalAutoimmune dominantParaneoplastic myelopathyarteriopathy Anteriopathy withOther autoimmune subcortical infarcts andMyelopathy LeukoencephalopathyAutoimmune/paraneoplasticmotor neuron disorders Dural arteriovenous malformatiorv’fistula
  11. 11. Infectious Compression/StructuralHIV myelopathy Tumor SpondylosisHuman T-cell lymphotropic TumorSyrinx Syrix virus—associatedmyelopathy/ Inflammatory tropical spastic paraparesis SarcoidosisBorrellosis VasculitisSchistosomiasis Bebcet syndromeBorrelia burgdorferi Sjogren syndromeHereditary/Degenerative Deficiency status Vitamin- B12Hereditary spastic CopperparaparesisFriedreich ataxiaAdrenomyeloneuropathy
  12. 12. COMPARIS0NON OF MS, NEUROMYELITIS, OPTIC, ACUTE DISSEMINATED ENCEPHALOMYELITIS, ANDPARANEOPLASTIC MYELOPATHIES AND ACUTEIMMUNE/OARANEO PLASTIC MOTOR NEURON DISEASECharacteristic Multiple Neuromyelitis Acute Paraneoplastic Auto immune/ Sclerosis Optica disseminated Myelopathy Paraneoplastic Motor neuron DiseaseAntecedent Variable Variable Typical No Noinfection orImmunizationMedian age 29 29 Children to 62 Vriableof onset(years)sex( F:M) 2;1 3-9:1 Similar Similar Slight female PredominateFrequency Common Intermediate Intermediate Rare Extremely rareEpidemiology White Disproportion Any Unknown Unknown ately
  13. 13. Characteristic Multiple Neuromyelitis Acute Paraneoplastic Auto immune/ Sclerosis Optica disseminated Myelopathy Paraneoplastic Motor neuron DiseaseNeural None Neuromyelitis None Collapsin Variableautoantibody optica lgG response-associations mediator protein5 and amphiphysin lgGs most commonBrain MRI Periventricular Hypothalamic, Subcortical, Normal Normal white matter periventricular, may involve lesions particularly deep gray thirdffourth matter ventricle; cloudlike enhancement
  14. 14. Characteristic Multiple Neuromyelitis Acute Paraneoplastic Auto immune/ Sclerosis Optica disseminated Myelopathy Paraneoplastic Encephalomyel Motor neuron iti DiseaseChronic Interferon Azathioprine None Cancer Cancertreatment Beta steroids, treatment. treatment. glatiramer mycophenolate steroids, IVIg. steroids, IVIg, acetate, mofetil, plasmapheresis cyclophospham natalizumab in rituximab ,cyclophospha ide, severe cases mide,azathiopri azathioprine, ne,mycophenol mycophenolate ate mofetil mofetilPrognosis Majority Moderate to Good; Poor; most Poor response ambulatory severe monophasic Wheelchair to after 20 years disability dependent treatment; may over time; within 2 to 5 have slower most patients years oroaression disabled than ALS