Ocular Myasthenia GravisPast, Present, and Future.ppt

Ocular Myasthenia Gravis:Past, Present, and Future Victoria S. Pelak, MD Departments of Neurology and Ophthalmology University of Colorado Health Sciences Center Ocular Myasthenia Gravis 1. Definition and Natural History 2. Epidemiology 3. Anatomy Pathophysiology 4. Clinical features Differential Dx 5. Diagnostic tests 6. Treatment 7. Future Options Definition Weakness and fatigability of cranial, limb, respiratory muscles “generalized” Levator palpebrae superioris, EOMs, and orbicularis oculi “ocular” 15% purely “ocular” Natural History Ocular symptoms in Myasthenia Gravis: 50% present solely with 75-80% have on presentation 90% eventually develop Natural History (Grob et al. ’81) ~2/3 will generalize Who? When? first 7 months OMG @ 1 year: 84% will NOT OMG @ 2 years: 88% OMG @ 3 years: 92% Historical Perspective Thomas Wills 1672 Samuel Wilks 1877 Ernst Sauerbrch 1912 Mary Walker 1934 C.E. Chang 1962 1970s Epidemiology: Incidence Incidence MG: 4-14/100,000 age and gender related generalized: early peak late peak ocular: late peak Generalized Myasthenia (Grob et al. ‘81) Epidemiology: Mortality (Grob et el. ’87) 1915-34: 70% 1935-39: 40% 1940-57: 33% 1958-65: 14% 1966-85: 7% 1934: anticholinesterase 1939: assisted ventilation 1960: pressure or volume 1966: steroid use Epidemiology: Associated Conditions Thyroid dysfunction Rheumatoid Arthritis Ankylosing spondylitis Anatomy Pathophysiology Anatomy Neuromuscular junction Pathophysiology Causes Autoimmune Anatomy Central nervous system Peripheral nerve Neuromuscular junction Muscle Combination Neuromuscular Junction Electrical impulse Chemical impulse Electrical impulse Neuromuscular Junction Disorders Myasthenia Gravis Lambert Eaton-Myasthenic Syndrome (LEMS) Toxic or Metabolic Botulism Hypermagnesemia Drugs (D-Penicillamine) Organophosphate toxicity Snake, spider, scorpion bites Pathophysiology: Causes Autoimmune Neon