Myasthenia Gravis and  Guillan Barre Syndrome Submitted to  AskTheNeurologist.Com   in 2007 Author Anon.
Myaesthenia Gravis “  A disease consisting of muscle weakness due to a variable block of neuromuscular transmission in whi...
 
Neuromuscular transmission <ul><li>Action potential arrives at synapse </li></ul><ul><li>Calcium enters via Ca channels (L...
Associated factors <ul><li>Female > Male </li></ul><ul><li>Often associated conditions: </li></ul><ul><li>- Thymoma </li><...
Symptoms <ul><li>Diplopia </li></ul><ul><li>Ptosis </li></ul><ul><li>Dysarthria </li></ul><ul><li>Dysphagia </li></ul><ul>...
Examination <ul><li>Weakness of EOM’s </li></ul><ul><li>Ptosis </li></ul><ul><li>Limb weakness </li></ul><ul><li>Fatiguabi...
Exacerbating factors <ul><li>Infection </li></ul><ul><li>Hypothyroidism </li></ul><ul><li>Poor compliance </li></ul><ul><l...
Diagnostic tests <ul><li>Tensilon ( edrophonium ) </li></ul><ul><li>EMG ( decrement ) </li></ul><ul><li>Ach receptor Ab’s ...
Further Investigations <ul><li>Chest CT scan </li></ul><ul><li>Monitoring of Ach R Ab level </li></ul><ul><li>VITAL CAPACI...
Treatment <ul><li>Anticholinesterase drugs </li></ul><ul><li>- Pyridostigmine ( Mestinon) </li></ul><ul><li>Thymectomy </l...
Prognosis <ul><li>Most patients can be managed successfully with drug treatment </li></ul><ul><li>Occasionally fatal due t...
Case 1 <ul><li>75 year old man with malignant thymoma and metastasis </li></ul><ul><li>Seropositive MG for 2 years ( contr...
Case 2 <ul><li>19 year old male soldier, admitted to ER with 3 hour history of worsening diplopia, dysphagia and general w...
Case 2 continued <ul><li>2 hours later, another soldier admitted with a similar problem ( from same base) </li></ul>
Guillan Barre Syndrome <ul><li>“ Acute Demyelinating Polyneuropathy” </li></ul>AXON MYELIN
Guillan Barre Syndrome “  Acute Demyelinating Polyneuropathy” AXON MYELIN
History <ul><li>Often follows minor infection </li></ul><ul><ul><li>- URTI  </li></ul></ul><ul><ul><li>- Diarrhoea ( Campy...
Examination <ul><li>Limb weakness </li></ul><ul><li>May ascend to affect facial muscles </li></ul><ul><li>Decreased or abs...
Autonomic instability <ul><li>Tachycardia </li></ul><ul><li>Other cardiac irregularities </li></ul><ul><li>Labile blood pr...
Investigations <ul><li>LP – High protein with normal cell count </li></ul><ul><li>Nerve conduction studies reveal slowing ...
Treatment <ul><li>IVIG and plasmapharesis </li></ul><ul><li>Steroids contraversial </li></ul><ul><li>Respiratory support i...
Monitoring <ul><li>Neurological examination </li></ul><ul><li>Complaints of dyspnea </li></ul><ul><li>Cardiac monitor </li...
Prognosis <ul><li>Self limiting </li></ul><ul><li>Improvement expected to begin by 4 weeks </li></ul><ul><li>About ¾ recov...
Submitted to  AskTheNeurologist.Com   in 2007 Author Anon.
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Myasthenia Gravis and Guillan Barre Syndrome (Acute Inflammatory Demyelinating Polyneuropathy)

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Myasthenia Gravis and Guillan Barre Syndrome

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Myasthenia Gravis and Guillan Barre Syndrome (Acute Inflammatory Demyelinating Polyneuropathy)

  1. 1. Myasthenia Gravis and Guillan Barre Syndrome Submitted to AskTheNeurologist.Com in 2007 Author Anon.
  2. 2. Myaesthenia Gravis “ A disease consisting of muscle weakness due to a variable block of neuromuscular transmission in which there is an immune-mediated decrease in the number of functioning acetylcholine receptors”
  3. 4. Neuromuscular transmission <ul><li>Action potential arrives at synapse </li></ul><ul><li>Calcium enters via Ca channels (LEMS) </li></ul><ul><li>ACh vesicles released </li></ul><ul><li>ACh binds to postsynaptic receptors </li></ul><ul><li>Muscle AP resulting in contraction </li></ul><ul><li>Enzyme ( ACHE ) breaks down ACh </li></ul>
  4. 5. Associated factors <ul><li>Female > Male </li></ul><ul><li>Often associated conditions: </li></ul><ul><li>- Thymoma </li></ul><ul><li>- Thyrotoxicosis </li></ul><ul><li>- SLE </li></ul><ul><li>- RA </li></ul><ul><li>- Other autoimmune conditions </li></ul>
  5. 6. Symptoms <ul><li>Diplopia </li></ul><ul><li>Ptosis </li></ul><ul><li>Dysarthria </li></ul><ul><li>Dysphagia </li></ul><ul><li>Limb weakness </li></ul><ul><li>Fluctuative fatiguability is hallmark </li></ul><ul><li>NO SENSORY SYMPTOMS </li></ul>
  6. 7. Examination <ul><li>Weakness of EOM’s </li></ul><ul><li>Ptosis </li></ul><ul><li>Limb weakness </li></ul><ul><li>Fatiguability </li></ul><ul><li>SENSATION IN TACT </li></ul>
  7. 8. Exacerbating factors <ul><li>Infection </li></ul><ul><li>Hypothyroidism </li></ul><ul><li>Poor compliance </li></ul><ul><li>Drugs - Aminoglycosides </li></ul><ul><li>- Beta Blockers </li></ul><ul><li>- Many others! </li></ul><ul><li>IV CONTRAST </li></ul>
  8. 9. Diagnostic tests <ul><li>Tensilon ( edrophonium ) </li></ul><ul><li>EMG ( decrement ) </li></ul><ul><li>Ach receptor Ab’s </li></ul>
  9. 10. Further Investigations <ul><li>Chest CT scan </li></ul><ul><li>Monitoring of Ach R Ab level </li></ul><ul><li>VITAL CAPACITY during exacerbations </li></ul>
  10. 11. Treatment <ul><li>Anticholinesterase drugs </li></ul><ul><li>- Pyridostigmine ( Mestinon) </li></ul><ul><li>Thymectomy </li></ul><ul><li>Steroids ( may worsen initially) </li></ul><ul><li>Azathioprine ( Imuran) </li></ul><ul><li>Cyclosporine </li></ul><ul><li>IVIG or plasmapharesis </li></ul>
  11. 12. Prognosis <ul><li>Most patients can be managed successfully with drug treatment </li></ul><ul><li>Occasionally fatal due to respiratory complications including aspiration pneumonia </li></ul>
  12. 13. Case 1 <ul><li>75 year old man with malignant thymoma and metastasis </li></ul><ul><li>Seropositive MG for 2 years ( controlled with pyridostigmine only) </li></ul><ul><li>Hospitalised in ITU because of aspiration pneumonia </li></ul><ul><li>Severe deterioration, family consulted regarding intubation </li></ul>
  13. 14. Case 2 <ul><li>19 year old male soldier, admitted to ER with 3 hour history of worsening diplopia, dysphagia and general weakness. </li></ul><ul><li>On examination BP 70 / 30, Pulse 40 </li></ul><ul><li>blurred vision when each eye examined separately </li></ul><ul><li>Pupils dilated and not reactive </li></ul>
  14. 15. Case 2 continued <ul><li>2 hours later, another soldier admitted with a similar problem ( from same base) </li></ul>
  15. 16. Guillan Barre Syndrome <ul><li>“ Acute Demyelinating Polyneuropathy” </li></ul>AXON MYELIN
  16. 17. Guillan Barre Syndrome “ Acute Demyelinating Polyneuropathy” AXON MYELIN
  17. 18. History <ul><li>Often follows minor infection </li></ul><ul><ul><li>- URTI </li></ul></ul><ul><ul><li>- Diarrhoea ( Campylobacter) </li></ul></ul><ul><li>Progressive symmetrical limb weakness </li></ul><ul><li>Usually affects legs first and ascends </li></ul><ul><li>Proximal > Distal weakness </li></ul><ul><li>Frequent sensory complaints </li></ul><ul><li>Progresses over no more than 4 weeks </li></ul>
  18. 19. Examination <ul><li>Limb weakness </li></ul><ul><li>May ascend to affect facial muscles </li></ul><ul><li>Decreased or absent reflexes </li></ul><ul><li>In severe cases where there is axonal damage muscle wasting may occur </li></ul>
  19. 20. Autonomic instability <ul><li>Tachycardia </li></ul><ul><li>Other cardiac irregularities </li></ul><ul><li>Labile blood pressure </li></ul><ul><li>Sweating </li></ul><ul><li>Pulmonary dysfunction </li></ul><ul><li>Sphincter disturbances </li></ul><ul><li>Paralytic ileus </li></ul>
  20. 21. Investigations <ul><li>LP – High protein with normal cell count </li></ul><ul><li>Nerve conduction studies reveal slowing of nerve conduction </li></ul><ul><li>Both abnormalities may lag behind clinical course </li></ul>
  21. 22. Treatment <ul><li>IVIG and plasmapharesis </li></ul><ul><li>Steroids contraversial </li></ul><ul><li>Respiratory support if necessary </li></ul><ul><li>General supportive care e.g. s.c heparin </li></ul>
  22. 23. Monitoring <ul><li>Neurological examination </li></ul><ul><li>Complaints of dyspnea </li></ul><ul><li>Cardiac monitor </li></ul><ul><li>Blood pressure </li></ul><ul><li>Vital capacity </li></ul>
  23. 24. Prognosis <ul><li>Self limiting </li></ul><ul><li>Improvement expected to begin by 4 weeks </li></ul><ul><li>About ¾ recover completely </li></ul><ul><li>20% remain with mild deficits </li></ul><ul><li>5% die - Respiratory failure </li></ul><ul><li>- Autonomic instability </li></ul>
  24. 25. Submitted to AskTheNeurologist.Com in 2007 Author Anon.

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