Highly active multiple sclerosis
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Highly active multiple sclerosis Malignant multiple sclerosis aggressive multiple sclerosis
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Highly active multiple sclerosis
- 1. Amr Hassan MD,FEBN Associate professor of Neurology Cairo University Highly Active Multiple Sclerosis
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- 5. Case vignette • H.M.H. • Male patient • 34 years old • Single • Physician • No special habits of medical importance • Rt Handed
- 6. 2006 2008 2011 2014 2017 Diplopia Ataxia Received IV MP treatment Back to normal Timeline
- 7. 2006 2008 2011 2014 2017 Recurrent attacks of left sided weakness ↓ Pulse IV MP ↓ Improvement ↓ Residual left sided weakness Timeline
- 8. 2006 2008 2011 2014 2017 Left focal fits VPA was added S.C. INF B 1B For 1 year with no improvement Cyclophospha mide for another 1 year Timeline
- 9. 2006 2008 2011 2014 2017 SE LVT was added Cognitive impairment Left work Timeline
- 10. Timeline
- 11. 2006 2008 2011 2014 2017 Still fits but infrequent Progressive Cognitive impairment Left sided weakness Rituximab 30th May Fever DCL 29th June Timeline
- 12. Which type of MS ?? Aggressive Malignant Fulminant Highly active RRMS --------------------------------------- SPMS
- 13. Sunny OR Stormy ? 13 GOOD EPIDEIOLOGICAL FACTORS BAD Female Sex Male < 40 y Age > 40 y
- 14. Sunny OR Stormy ? 14 GOOD RELAPSES BAD Mild, monofocal 1st relapse Severe , multifocal Sensory, ON Clinical presentation Motor, cerebellar Full recovery Response to ttt Residual Long Time to 2nd relapse Short Low Relapse rate High
- 15. Sunny OR Stormy ? 15 GOOD DISABILITY BAD Long Time to EDSS 4-5 Short GOOD MRI BAD Low Lesion load High Absent CEL Present
- 16. Predictors of Highly Active MS Freedman et al., 2016
- 17. Predictors of Highly Active MS Freedman et al., 2016
- 18. Predictors of Highly Active MS Freedman et al., 2016
- 19. Predictors of Highly Active MS Freedman et al., 2016
- 21. Malignant (fulminant) MS • Forms of MS that deteriorate so rapidly and progressively from the beginning that they are almost monophasic illnesses and can result in death within a very short time Otto Marburg (1874 –1948)
- 23. Malignant MS • Multiple sclerosis (MS) that causes patients to require assistance for ambulation (EDSS 6) within 5 years from symptom onset is generally termed malignant. • Malignant status can be transient (TM) or sustained until year 5 (SM). Gholipour T, Healy B, & Baruch NF, et al. Demographic and clinical characteristics of malignant multiple sclerosis. Neurology 2011;76(23):1996–2001
- 24. TM OR SM ? 24 TM SM Brain stem Male Younger age Older age Smoking Gholipour T, Healy B, & Baruch NF, et al. Demographic and clinical characteristics of malignant multiple sclerosis. Neurology 2011;76(23):1996–2001 Malignant MS
- 25. Aggressive MS
- 26. The following criteria for diagnosing AOMS: 1) two or more relapses in the year after onset and two or more gadolinium-enhancing lesions on brain MRI scan; or 2) one relapse if it results in sustained baseline EDSS score of 3 along with two or more gadolinium-enhancing lesions. Aggressive onset MS
- 27. Highly active MS • EDSS score of 4.0 within 5 years of onset • Poor response to at least 1 full year of therapy with one or more disease-modifying therapies, not because of intolerance • Breakthrough disease over at least 1 year of disease-modifying therapy consisting of: – Two or more disabling relapses with incomplete resolution – Two or more MRI studies showing new or enlarging T2 lesions or gadolinium-enhancing lesions Freedman et al., 2016
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- 29. Timing of the therapy key to preventing disability Time (Years) Relapsing Remitting Multiple sclerosis Transitional Secondary Progressive MSCISPre- Clinical Demyelination Remission State of no disease activity, the period during which diminution of symptoms occurs due to the cessation of inflammatory processes and some degree of reparative remyelination of affected axons Relapses Acute Inflammation Demyelination First Clinical Attack Axonal loss Inflammation Brain Volume MRI Activity Disability progression Reflects reactive astrogliosis, Axonal Loss and Brain volume loss. Starts Reversible (remyelination) and ends in permanent disability Time window for early treatment Mark S. Freedman: Induction vs. escalation of therapy for relapsing Multiple Sclerosis: the evidence, Neurol Sci (2008) 29:S250–S252
- 30. Timing of the therapy key to preventing disability Time (Years) Relapsing Remitting Multiple sclerosis Transitional Secondary Progressive MSCISPre- Clinical Demyelination Axonal loss Inflammation Brain Volume MRI Activity Time window for early treatment Mark S. Freedman: Induction vs. escalation of therapy for relapsing Multiple Sclerosis: the evidence, Neurol Sci (2008) 29:S250–S252 Aggressive MS
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- 34. Fazekas et al., 2013
- 35. Fazekas et al., 2013
- 36. Treatment Algorithm Freedman et al., 2016
- 37. Conclusions • To date, aggressive multiple sclerosis has no uniform definition. • Multiple sclerosis has several well-known clinical and MRI factors for poor prognosis. • Effectively treating multiple sclerosis early preserves central nervous system reserve for aging later in life. • Patients with aggressive MS have a narrower therapeutic window. • Aggressive multiple sclerosis warrants aggressive treatment.
- 38. THANK YOU