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Dr.Mohammad Shaikhani DISORDERS OF THE NEUROMUSCULAR JUNCTION  MYASTHENIA GRAVIS
Definition: ,[object Object]
Aetiology / pathology: ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
Clinical features   ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical features   ,[object Object],[object Object],[object Object],[object Object]
 
Investigations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Investigations
Management   ,[object Object],[object Object],[object Object]
Management : 1.Ach estrase inhibs ,[object Object],[object Object],[object Object],[object Object]
Management: 2. IMMUNOLOGICAL Trt ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Prognosis ,[object Object],[object Object],[object Object],[object Object],[object Object]
OTHER MYASTHENIC SYNDROMES   ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Differences between MG & Myasthenic (Eaton-Lamberts) syndrome:     MS MG 1.Autoimmune immunological attack on  presynaptic  membrane limiting the release of ACH. 2.Mosrly  paraneoplastic  : in 2/3 of cases due to oat cell Ca. of the lungs > 40 ys . of age & 1/3  non neoplastic  occuring at any age. 3.Realative sparing of  extra ocular & bulbar muscles . 4.Autonomic dysfunction occur  in 50% of cases. 5. EMG  shows decreases amplitude of contraction with single nerve stimulation  , but repetitive stimulation at frequencies > 10htz produces normal amplitude. 6. Treatment: ACH estrase inhibitors has minimal effect. Paraneoplastic type needs management of underlying cancer with guanidine & diaminopyridine having some benefits. For nonneoplastic type steroids + atzathioprine  can control immuologocal attack.   1. Postsynaptic  ACH receptors Abs.     2.All  autoimmune  type 3.       3.Common .   4.Not present,   5.Just  contrary  to that. 6.Good response to ACH estrase inhibitors.          
Disease of muscles: MYOPATHIES     ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Disease of muscles: MUSCULAR DYSTROPHIES       ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Disease of muscles: MUSCULAR DYSTROPHIES       ,[object Object],[object Object],[object Object],[object Object],[object Object]
Disease of muscles: MUSCULAR DYSTROPHIES       Type Genetics onset Muscles affected Other features Myotonic dystrophy (DM1) AD; triplet repeat chr19q Any Face (ptosis), sternomastoids, distal limb, generalised later Myotonia, cognitive dulling, cardiac conduction abnormalities, lens opacities, frontal balding, hypogonadism Proximal myotonic myopathy (PROMM; DM2) AD; chr 3q Adult Proximal, esp thigh, sometimes muscle hypertrophy As for DM1 but cognition not affected, Muscle pain Duchenne XL; deletd dystrophin gene 1st 5ys Proximal/ limb girdle Pseudohypertrophy of calves Cardiomyopathy, Gower sign +ve:patient climbs thigh by hand to stand Becker XL; deled dystrophin gene LC/EA Proximal/limb girdle Pseudohypertrophy of calves Cardiomyopathy Gower +ve.
Disease of muscles: MUSCULAR DYSTROPHIES       Limb girdle AD (type 1) AR (type 2) different chromosomes Childhood/early adult Limb girdle Some have calf hypertrophy Some have cardiac conduction abnormalities Facioscapulohumeral (FSH) AD; chromosome 4q 7-30 ys Face/ upper limb girdle Pain in shoulder girdle common Oculopharyngeal AD/AR recessive; chromosome 14q 30-50 years Ptosis, external ophthalmoplegia, dysphagia, tongue weakness Mild lower limb weakness Emery-Dreifuss XLR; mutations in emerin gene 4-5 years Humero-peroneal, proximal limb girdle later Contractures early Cardiac involvement leads to sudden death
Disease of muscles: MUSCULAR DYSTROPHIES       ,[object Object],[object Object],[object Object],[object Object]
1.Myasthenia gravis can be associated with: A. Presynaptic antibodies. B. Thymoma. C. Thymic hyperplasia. D. Skeletal muscle antibodies. E. Other autoimmune diseases.
2.M2yasthenia gravis &Thymic hyperplasia: A. Most cases have the disorder. B. Chest X Ray is indicated in every cases. C. Thymic surgery is indicated in every case. D. Thymic hyperplasia makes prognosis worse. E. Thymic hyperplasia occur in elderly patients.
3.Thymoma with myasthenia gravis: A. Makes prognosis worse. B. Occurs in elderly. C. Associated with skeletal muscle antibodies. D. More common than Thymic hyperplasia. E. Makes the disease more severe.
4.Diagnosis of myasthenia gravis can be supported by: A. Positive tensilon test. B. Ice on Eyes test. C. Number counting test. D. Ach antibodies. E. Anti skeletal muscle antibodies.
5. The following suggest cholinergic crisis rather than myasthenic crisis: A. Sweating. B. Salivation. C. Severe Paralysis. D. Pallor. E. Respiratory failure.
Myasthenia Gravis
 
 
 
 

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CNS myasthenia gravis davidson 10 MCQs.

  • 1. Dr.Mohammad Shaikhani DISORDERS OF THE NEUROMUSCULAR JUNCTION MYASTHENIA GRAVIS
  • 2.
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  • 10.
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  • 15. Differences between MG & Myasthenic (Eaton-Lamberts) syndrome:     MS MG 1.Autoimmune immunological attack on presynaptic membrane limiting the release of ACH. 2.Mosrly paraneoplastic : in 2/3 of cases due to oat cell Ca. of the lungs > 40 ys . of age & 1/3 non neoplastic occuring at any age. 3.Realative sparing of extra ocular & bulbar muscles . 4.Autonomic dysfunction occur in 50% of cases. 5. EMG shows decreases amplitude of contraction with single nerve stimulation , but repetitive stimulation at frequencies > 10htz produces normal amplitude. 6. Treatment: ACH estrase inhibitors has minimal effect. Paraneoplastic type needs management of underlying cancer with guanidine & diaminopyridine having some benefits. For nonneoplastic type steroids + atzathioprine can control immuologocal attack. 1. Postsynaptic ACH receptors Abs.     2.All autoimmune type 3.       3.Common .   4.Not present,   5.Just contrary to that. 6.Good response to ACH estrase inhibitors.          
  • 16.
  • 17.
  • 18.
  • 19. Disease of muscles: MUSCULAR DYSTROPHIES     Type Genetics onset Muscles affected Other features Myotonic dystrophy (DM1) AD; triplet repeat chr19q Any Face (ptosis), sternomastoids, distal limb, generalised later Myotonia, cognitive dulling, cardiac conduction abnormalities, lens opacities, frontal balding, hypogonadism Proximal myotonic myopathy (PROMM; DM2) AD; chr 3q Adult Proximal, esp thigh, sometimes muscle hypertrophy As for DM1 but cognition not affected, Muscle pain Duchenne XL; deletd dystrophin gene 1st 5ys Proximal/ limb girdle Pseudohypertrophy of calves Cardiomyopathy, Gower sign +ve:patient climbs thigh by hand to stand Becker XL; deled dystrophin gene LC/EA Proximal/limb girdle Pseudohypertrophy of calves Cardiomyopathy Gower +ve.
  • 20. Disease of muscles: MUSCULAR DYSTROPHIES     Limb girdle AD (type 1) AR (type 2) different chromosomes Childhood/early adult Limb girdle Some have calf hypertrophy Some have cardiac conduction abnormalities Facioscapulohumeral (FSH) AD; chromosome 4q 7-30 ys Face/ upper limb girdle Pain in shoulder girdle common Oculopharyngeal AD/AR recessive; chromosome 14q 30-50 years Ptosis, external ophthalmoplegia, dysphagia, tongue weakness Mild lower limb weakness Emery-Dreifuss XLR; mutations in emerin gene 4-5 years Humero-peroneal, proximal limb girdle later Contractures early Cardiac involvement leads to sudden death
  • 21.
  • 22. 1.Myasthenia gravis can be associated with: A. Presynaptic antibodies. B. Thymoma. C. Thymic hyperplasia. D. Skeletal muscle antibodies. E. Other autoimmune diseases.
  • 23. 2.M2yasthenia gravis &Thymic hyperplasia: A. Most cases have the disorder. B. Chest X Ray is indicated in every cases. C. Thymic surgery is indicated in every case. D. Thymic hyperplasia makes prognosis worse. E. Thymic hyperplasia occur in elderly patients.
  • 24. 3.Thymoma with myasthenia gravis: A. Makes prognosis worse. B. Occurs in elderly. C. Associated with skeletal muscle antibodies. D. More common than Thymic hyperplasia. E. Makes the disease more severe.
  • 25. 4.Diagnosis of myasthenia gravis can be supported by: A. Positive tensilon test. B. Ice on Eyes test. C. Number counting test. D. Ach antibodies. E. Anti skeletal muscle antibodies.
  • 26. 5. The following suggest cholinergic crisis rather than myasthenic crisis: A. Sweating. B. Salivation. C. Severe Paralysis. D. Pallor. E. Respiratory failure.
  • 28.  
  • 29.  
  • 30.  
  • 31.  

Editor's Notes

  1. I am going to be talking with you today about synaptic transmission. Since, this is a topic that involves many areas in neuroscience, and could easily be made into a semester length course, I am just going to give you a survey of what is involved in synaptic transmission. In later courses, you will go into much greater detail on each topic I discuss today. So, what is synaptic transmission and what does it entail? Well, simply put, it is the method by which neurons communicate with one another. In order for any movement, sensation, thought, or emotion to occur, many neurons have to relay and integrate messages to one another. This involves many processes both on the electrophysiological and molecular levels. If you have any questions along the way, please do not hesitate to ask. I do not mind being interrupted. If you would like background reference information to this lecture please read Part 2 of Principles of Neural Science (Kandel & Schwartz).
  2. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  3. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  4. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  5. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  6. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  7. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  8. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  9. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  10. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  11. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  12. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  13. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  14. So far, I have mentioned the nt Ach, but there are many other substances in the nervous system that are considered nts. So, what makes something a nt? Well, there are three main criteria that a substance must meet in order to be considered a nt…
  15. A fundamental structure necessary for synaptic transmission is the synapse. This is the point where a neuron relays its message to an adjacent neuron. In the CNS there are two major types of synapses: electrical and chemical. This chart outlines some of the major differences between the two…..
  16. A fundamental structure necessary for synaptic transmission is the synapse. This is the point where a neuron relays its message to an adjacent neuron. In the CNS there are two major types of synapses: electrical and chemical. This chart outlines some of the major differences between the two…..
  17. A fundamental structure necessary for synaptic transmission is the synapse. This is the point where a neuron relays its message to an adjacent neuron. In the CNS there are two major types of synapses: electrical and chemical. This chart outlines some of the major differences between the two…..
  18. A fundamental structure necessary for synaptic transmission is the synapse. This is the point where a neuron relays its message to an adjacent neuron. In the CNS there are two major types of synapses: electrical and chemical. This chart outlines some of the major differences between the two…..
  19. A fundamental structure necessary for synaptic transmission is the synapse. This is the point where a neuron relays its message to an adjacent neuron. In the CNS there are two major types of synapses: electrical and chemical. This chart outlines some of the major differences between the two…..
  20. A fundamental structure necessary for synaptic transmission is the synapse. This is the point where a neuron relays its message to an adjacent neuron. In the CNS there are two major types of synapses: electrical and chemical. This chart outlines some of the major differences between the two…..
  21. A fundamental structure necessary for synaptic transmission is the synapse. This is the point where a neuron relays its message to an adjacent neuron. In the CNS there are two major types of synapses: electrical and chemical. This chart outlines some of the major differences between the two…..
  22. In the final section of my lecture I am going to give you an overview of a disease that disrupts synaptic transmission a the NMJ. MG is an autoimmune disease in which antibodies to nicotinic Ach receptors are produced. These antibodies either destroy the Ach receptors or bind to and block others. So, when you destroy or block Ach receptors at the NMJ you end up with less functional receptors which leads to decreased synaptic transmission at the NMJ. One of the symptoms that an individual with MG often presents with is muscle weakness.
  23. In addition to muscle weakness, the patient also presents with head and neck symptoms since this disease typically affects the cranial muscles. Some of the symptoms that a patient can present is…….
  24. In addition to muscle weakness, the patient also presents with head and neck symptoms since this disease typically affects the cranial muscles. Some of the symptoms that a patient can present is…….
  25. In addition to muscle weakness, the patient also presents with head and neck symptoms since this disease typically affects the cranial muscles. Some of the symptoms that a patient can present is…….
  26. In addition to muscle weakness, the patient also presents with head and neck symptoms since this disease typically affects the cranial muscles. Some of the symptoms that a patient can present is…….