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Neuro Disorders

1. MYASTHENIA GRAVIS Presenter NYUMBU

2. TABLE OF CONTENTS Introduction Epidemiology Causes Pathophysiology Clinical features Diagnosis management

3. INTRODUCTION Myasthenia gravis is an autoimmune disease that causes chronic, progressive damage of the neuromuscular junction. The underlying defect is a decrease in the number of available acetylcholine receptor at neuromuscular junction due to an antibody mediated autoimmune attack. It worsens with activities and improves with resting Its not a brain disorder –brain function normally

4. EPIDEMIOLOGY The estimated prevalence is approx 77 to 150 Cases per 100,000 population Incidence increases with advancing with age

5. AETIOLOGY Autoantibodies that destroys acetylcholine receptors Autoantibodies against muscle specific kinase Thymus tumor Drugs Genetic inheritance

6. IMAGE

7. PATHOPHYSIOLOGY Autoimmune response Development of auto-antibodies Antibodies attack AchR at the motor end plate Impair the ability of Ach to bind to receptors Resulting in voluntary muscle weakness that escalates with continued activity

8. CLINICAL FEATURES EYE MUSCLES Ptosis Double vision FACE AND THROAT MUSCLES Problem in chewing Limited facial expression Dysphasia

9. Cont RESPIRATORY SYMPTOMS Weakness of intercostal muscles and diaphragm Weakness of pharyngeal muscles PALATE MUSCLE WEAKNESS Swallowing maybe difficult and regurgitation of food can occur Limb muscle weakness is often proximal and asymmetric

10. Images

11. CLINICAL CLASSIFICATION

12. DIAGNOSIS Physical examination Serological test Imaging techniques Endrophonium test

13. PHYSICAL EXAMINATION Repeated blinking of 60-180 seconds this results in ptosis of one or both eyes Sustained abduction of the arms this will lead to weakness Asking a patient to make a high pitched sound this results in hoarseness of the voice this is due to weakness of the laryngeal muscle

14. ICEPACK TEST Ptosis may improve after applying ice to the eyelids since cold temperatures may facilitate neuromuscular transmission

15. THE TENSILON TEST This involve the use of endrophonium an acetycholinesterase inhibitor that inhibits the breakdown of acetylcholine in the neuromuscular junction

16. IMAGES CT or MRI of the chest to screen for thymoma Single fiber electromyograph detects impaired nerve to muscle transmission

17. Labs Ach receptor antibodies Muscle specific kinase antibodies

18. Management Medica Surgical

19. Medical mgt Immunosuppresive therapy- with prednisolone start at 5mg/kg single dose Acetylcholinesterase inhibitors –first line neostigmine bromide

20. Surgical mgt Thymectomy surgical removal of thymus gland this can produce antigen specific immunosuppression and result in clinical improvement

21. Complications Mysthenic crisis This acute exacerbation causing respiratory failure requiring intubation,it can be triggered by infection,surgery or drugs such as quinolones,macrolides calcium channel blockers RX Steroids, mechanical respiratory support and treating the triger

22. Thank you