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Myasthenia and Myopathies - An Ocular Perspective.

September 2006.. www.riogohchennai.ac.in. 2 / 28 . MYASTHENIA. Ptosis or DiplopiaInitial symptom in 2/3FluctuationSpontaneous improvementRestricted weakness 10-40%Progression in 2 years. September 2006.. www.riogohchennai.ac.in. 3 / 28 . Findings. One or more ocular musclePupils sparedVariabl

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Myasthenia and Myopathies - An Ocular Perspective.

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    1. September 2006. www.riogohchennai.ac.in 1 / 28 Myasthenia and Myopathies - An Ocular Perspective. Dr. V. Natarajan. Additional Professor of Neurology Institute of Neurology Government General Hospital and Madras Medical College

    2. September 2006. www.riogohchennai.ac.in 2 / 28 MYASTHENIA Ptosis or Diplopia Initial symptom in 2/3 Fluctuation Spontaneous improvement Restricted weakness 10-40% Progression in 2 years

    3. September 2006. www.riogohchennai.ac.in 3 / 28 Findings One or more ocular muscle Pupils spared Variable weakness, fatigability Shift of ptosis – Pathognomonic Ocular “quiver” – Superfast saccades

    4. September 2006. www.riogohchennai.ac.in 4 / 28 Lid Twitch Lifting ptotic lid may cause the other lid to fall Covering of ptotic lid relieves contraction of opposite frontalis Ice pack test

    5. September 2006. www.riogohchennai.ac.in 5 / 28 Investigations Anti Ach R antibody titre False positive is rare 50% of OMG – Antibody negative 15% of GMG – Antibody negative Anti Musk Ab 50% sero negative Ach R

    6. September 2006. www.riogohchennai.ac.in 6 / 28 Edrophonium (Tensilon) Test Superseded Small risk of respiratory arrest, cardiac arrhythmias Resuscitation facilities Pre dose with Atropine

    7. September 2006. www.riogohchennai.ac.in 7 / 28 Ideal dose cannot be predetermined escalating dose 2mg, 3mg, 5mg IV IM Neostigmine Positive in MND and OMN lesions Negative test does not exclude MG

    8. September 2006. www.riogohchennai.ac.in 8 / 28 Neurophysiologic Tests RNS Supra maximal 3 Hz decrement SF-EMG More sensitive 2 muscle fibers in 1 unit Inter potential interval variability Jitter / Block

    9. September 2006. www.riogohchennai.ac.in 9 / 28 Treatment Ach EI – Symptom relief Steroids – Individualized Thymectomy Debatable Young patients Recent onset Poor response to Ch EI

    10. September 2006. www.riogohchennai.ac.in 10 / 28

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    12. September 2006. www.riogohchennai.ac.in 12 / 28

    13. September 2006. www.riogohchennai.ac.in 13 / 28 OCULO PHARYNGEAL MUSCULAR DYSTROPHY Same age group as MG 5th & 6th decade EOM & Ptosis asymmetrical Swallowing difficulty Facial & limb weakness EMG & Muscle biopsy

    14. September 2006. www.riogohchennai.ac.in 14 / 28

    15. September 2006. www.riogohchennai.ac.in 15 / 28

    16. September 2006. www.riogohchennai.ac.in 16 / 28 Myotonic Dystrophy Ptosis, no EOM involvement Facial appearance – wasting Associated features Cardiac Cataract Endocrine & Testicular atrophy Myotonia on percussion EMG

    17. September 2006. www.riogohchennai.ac.in 17 / 28 Mitochondrial myopathy Progressive external ophthalmoplegia with or without limb weakness RP, heart block, endocrinopathies Lactate, Pyruvate CSF proteins Muscle biopsy

    18. September 2006. www.riogohchennai.ac.in 18 / 28

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    21. September 2006. www.riogohchennai.ac.in 21 / 28 Congenital Myopathies Centro muscular or myotubular myopathy X linked recessive- infantile; AD- in later life Ptosis, EOM, facial and limb muscles EMG – Spontaneous activity Muscle biopsy

    22. September 2006. www.riogohchennai.ac.in 22 / 28 Tolosa Hunt Syndrome Inflammatory pathology in anterior cavernous sinus, SOF Usually painful EOM paresis, asymmetrical Steroid responsive Recurrent Associated V, II helpful

    23. September 2006. www.riogohchennai.ac.in 23 / 28 Partial III Nerve Superior branch Ptosis, Superior rectus Pupil spared Idiopathic, viral, aneurysm of IC, PCom

    24. September 2006. www.riogohchennai.ac.in 24 / 28 Congenital Myasthenic Syndromes (CMS) Heterogenous group Genetic defects affecting NMT Presynaptic, synaptic and post synaptic defects Muscle weakness – Increases by exertion

    25. September 2006. www.riogohchennai.ac.in 25 / 28 Early onset Opthalmoplegia, ptosis, bulbar Amyotrophy, tendinous retraction facial malformation Family history

    26. September 2006. www.riogohchennai.ac.in 26 / 28

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