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UB Dept of Neurology UBMD Neurology/JNI

brains &GAINS November 2, 2013 9-12 a.m. Center For Tomorrow. UB Dept of Neurology UBMD Neurology/JNI. http://ubnjacobsneuro.wordpress.com/home/. https://www.facebook.com/UBMDNeurology. @ wolfegil. Gil I. Wolfe, MD. Irvin and Rosemary Smith Professor and Chair since Jan 2012

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UB Dept of Neurology UBMD Neurology/JNI

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  1. brains&GAINS November 2, 2013 9-12 a.m. Center For Tomorrow UB Dept of Neurology UBMD Neurology/JNI http://ubnjacobsneuro.wordpress.com/home/ https://www.facebook.com/UBMDNeurology @wolfegil

  2. Gil I. Wolfe, MD • Irvin and Rosemary Smith Professor and Chair since Jan 2012 • 9 new Dept. of Neurology faculty • 3 stroke • 1 multiple sclerosis • 1 adult epilepsy • 1 child neurology • 1 neurocritical care • 2 neuroimaging (100% research) • Clinical and investigative career in neuromuscular medicine • Myasthenia Gravis (MG) • ECU-MG-301: Double-blind, placebo –controlled trial of eculizimab in refractory generalized MG • Clinical chair of international, NIH supported study of thymectomy in MG • Peripheral Neuropathy • GBS longitudinal follow-up study (IGOS) • Others • INSIGHTS • IV immunoglobulin usage patterns across U.S.

  3. Aging, Alzheimer’s Disease & Memory • Kinga Szigeti, MD, PhD • Director, Memory Disorders Program

  4. Disease Classification Motor neuron Neuronopathies & Neuropathies ALS, PLS, SMA, diabetic neuropathy, toxic neuropathy, GBS, CMT Axon Myelin Neuromuscular Junction Disorders MG, botulism NMJ Myopathies Muscular dystrophy, polymyositis, dermatomyositis, IBM Muscle

  5. Eculizimab in MG • Monoclonal antibody that binds complement C5, preventing cleavage to C5a • Complement activation is key component to damage of muscle membrane in MG • Cobra venom blocks complement and prevents induction of experimental autoimmune MG (EAMG) • Membrane attack complex (MAC) present on muscle membrane in EAMG animals and MG patients • Up to 10% of MG patients refractory

  6. MG and complement-mediated injury Control AChRAb MuSKAb MuSKAb • Shiraishi et al. Ann Neurol 2005;57:289 (biceps biopsies)

  7. Phase II Study of Eculizimab • n=14 (30-72 yrs) • AChRAb+ and failed 2 immunosuppressives, including prednisone, for ≥ 1 yr • No IVIg for 8 weeks or plasma exchange for 12 weeks • Results • Reduction by >3 QMG points during eculizimab vs. placebo marginally significant (p=0.0577) • Side effects • Mild to moderate • Nausea, back pain, headache, pharyngitis • 3 serious adverse advents on both active and placebo treatment Howard JF et al. Muscle Nerve 2013;48:76--84 600 mg IV qwk for 4 wks 900 mg IV at 5th q2wks for 12 wks

  8. ECU-MG-301: Eculizimab in refractory generalized MG • Phase 3, randomized (1:1), double-blinded, placebo-controlled trial • Treatment duration 26 wks • AChRAb+, age ≥ 18 yrs • MG-ADL score ≥ 6 • MG-ADL is the primary outcome measure • QMG, MGC, QOL also measured • Failed ≥2 immunotherapies for at least 1 yr • No IVIg or PE within 4 wks • Can continue on current immunosuppressive treatments without dose changes

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