Multiple Sclerosis - by MHR Corp

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Multiple Sclerosis - by MHR Corp

  1. 1. MULTIPLE SCLEROSIS<br />MOHD HANAFI RAMLEE<br />
  2. 2. DEMYLINATION<br />“A disease process whose prominent feature is the loss of myelin sheath surrounding axons in the central nervous system ”<br />Multiple sclerosis is the most common example<br />
  3. 3. EPIDEMIOLOGY<br />Prevalence is >50 per 100,000 in US<br />Age range- 10 to 60 years<br />Peak incidence- 20 to 30 years<br />Female predominance<br />Genetic predisposition <br />20 times higher in first-degree relatives<br />Prevalence directly proportional to distance from equator<br />
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  5. 5. ETIOLOGY<br />Cause is still unknown<br />Identified factors:<br />Autoimmune causes<br />Human Leukocyte Antigens<br />Viral causes<br />Roseola virus<br />
  6. 6. PATHOPHYSIOLOGY<br />Scattered areas of demyelination= <br />“Plaques”<br />Plaques are more common in:<br />Optic tracts<br />Spinal cord<br />Brain stem<br />Basal Ganglia<br />
  7. 7. PATHOPHYSIOLOGY<br />Demyelinated axons<br />Do not conduct normal action potentials<br />Hyperexcitable (generate action potentials with minimal stimuli)<br />Lesions are “scattered in space and time”<br />
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  10. 10. CLASSIFICATION<br />TYPE OF MS<br />Benign MS- 10%<br />Relapsing-remitting MS- 40%<br />Secondary chronic progressive- 40% of patients with original relapsing-remitting MS<br />Primary progressive MS- 10%<br />
  11. 11. CLINICAL PRESENTATION<br />A relapsing-remitting pattern is characteristic for this disease. <br />EARLY STAGE:<br />Double or blurred vision<br />Numbness<br />Weakness in one or two extremities<br />Instability in walking<br />Tremors<br />Problems with bladder control<br />Heat intolerance. <br />
  12. 12. CLINICAL PRESENTATION<br />MOTOR SYMPTOMS<br />Upper motor neuron signs<br />Mild spasticity<br />Hyperreflexia<br />Monoparesis (one extremity)<br />Quadriparesis (all four extremities)<br />
  13. 13. CLINICAL PRESENTATION<br />SENSORY SYMPTOMS<br />Ascending numbness starting in the feet <br />Bilateral hand numbness<br />Hemiparesthesia<br />Reduction of vibration<br />Reduction of proprioception <br />
  14. 14. CLINICAL PRESENTATION<br />OCULAR SYMPTOMS<br />Optic Neuritis<br />Frequent presenting symptom of MS (30%)<br />Inflammation of the optic nerve head<br />Fundus exam- swelling, edema, preservation of venous pulsations<br />Blurred vision<br />
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  16. 16. CLINICAL PRESENTATION<br />OCULAR SYMPTOMS<br />Optic neuritis<br />Change in color perception<br />Visual field defect (central scotoma)<br />Headaches and retro-orbital pain precipitated by eye movements<br />“Uhthoff’s phenomenon”= visual acuity worsens with increase in body temperature<br />
  17. 17. CLINICAL PRESENTATION<br />OCULAR SYMPTOMS<br />Internuclear opthalmoplegia (INO)<br />Interruption of fibers in the medial longitudinal fasciculus that connect III and VI nuclei<br />Abnormal adduction of involved eye<br />Horizontal nystagmus on abduction of contralateral eye<br />Usually bilateral<br />Healthy young person with INO= think of MS<br />VI nerve paresis and palsy <br />III and IV nerves palsy (uncommon) <br />
  18. 18. CLINICAL PRESENTATION<br />Ongoing symptoms and signs<br />Motor system<br />Weakness (variable severity mono- and paraparesis, hemiparesis, quadriparesis) <br />Increased spasticity resulting in spastic gait <br />Pathologic signs (Babinski's, Chaddock's, Hoffmann, Oppenheim's)<br />Dysarthria <br />
  19. 19. CLINICAL PRESENTATION<br />Ongoing symptoms and signs<br />Cerebellar signs<br />Incoordination (dysdiadochokinesia, problems with heel-to-shin test) <br />Slowing of rapid repeating movements <br />Ataxic gait<br />Abnormal speech <br />Loss of balance<br />
  20. 20. CLINICAL PRESENTATION<br />Ongoing symptoms and signs<br />Sensory systems<br />Lhermitte's sign <br />Paresthesia<br />Numbness <br />Dorsal column signs (severe decrease or loss of vibratory sense and proprioception, positive Romberg's test)<br />
  21. 21. CLINICAL PRESENTATION<br />Ongoing symptoms and signs<br />GU<br />urinary incontinence<br />incomplete emptying<br />increased frequency of urination<br />urinary tract infections <br />Ocular<br />optic disc pallor and atrophy<br />blurred vision<br />diplopia<br />nystagmus<br />intranuclear ophthalmoplegia<br />central scotomas/ visual field defects <br />
  22. 22. CLINICAL PRESENTATION<br />Ongoing symptoms and signs<br />Cognitive and emotional abnormalities <br />Emotional lability<br />Depression<br />Anxiety <br />Fatigue <br />
  23. 23. Patterns of MS<br />Relapsing - remitting<br />Attacks with complete/incomplete recovery<br />Stable between attacks<br />Secondary - progressive<br />Initially relapsing-remitting<br />Then progression +/- attacks<br />Progressive - relapsing<br />Initial gradual detioriation<br />Subsequent episodes<br />Primary progressive<br />Gradual decline<br />No attacks<br />
  24. 24. RELAPSING/REMITTING<br />
  25. 25. SECONDARY PROGRESSIVE<br />
  26. 26. PROGRESSIVE RELAPSING<br />
  27. 27. PRIMARY PROGRESSIVE<br />
  28. 28. DIFFERENTIAL DIAGNOSIS<br />Postinfectious Encephalomyelitis<br />Primary CNS Vasculitis<br />Lyme Disease<br />Systemic Lupus Erythematosus<br />Tropical Spastic Paraparesis<br />
  29. 29. DIFFERENTIAL DIAGNOSIS<br />Behçet Syndrome<br />Sarcoidosis <br />Vitamin B-12 deficiency<br />Tertiary Syphilis<br />Progressive Multifocal Leukoencephalopathy<br />
  30. 30. Principal Differential Diagnosis of Multiple Sclerosis<br />Infection<br />Lyme, Syphilis, Progressive Multifocal Leukoencephalopathy, HIV, HTLV-1<br />Inflammatory<br />SLE, Sjogren syndrome, vasculitis, Sarcoidosis, Bechet’s disease<br />Metabolic<br />B12 deficiency, lysosomal disorders, adrenoleukodystrophy, mitochondrial disorders, other genetic diseases<br />Neoplastic<br />CNS lymphoma<br />Spine disease<br />Vascular malformations, degenerative spine disease<br />
  31. 31. CRITERIA FOR DIAGNOSIS<br />Probable MS with laboratory support<br />History of two attacks<br />Positive oligoclonal bands or Increased IgG in CSF<br />No clinical evidence of a disease<br />Clinically Probable MS<br />History of two attacks without laboratory abnormalities<br />
  32. 32. CRITERIA FOR DIAGNOSIS<br />Laboratory-supported definite MS<br />History of two attacks<br />Clinical evidence of one lesion<br />Oligoclonal bands or increased IgG present in CSF<br />Clinically-definite MS<br />History of at least two attacks<br />Clinical evidence of at least one lesion<br />
  33. 33. ED PRESENTATONS<br />Exacerbation of previous deficits<br />Development of new deficits<br />Development of complications<br />Initial presentation<br />
  34. 34. Treatments<br />Rehabili-tation<br />Surgical<br />Treatments<br />Oral<br />Medications<br />Local<br />Treatments<br />
  35. 35. TREATMENT<br />General<br />Specific therapy<br />Preventive therapy for relapses<br />Supportive therapy<br />
  36. 36. TREATMENT<br />GENERAL<br />Exercise<br />Physical therapy<br />Nutrition<br />Pregnancy<br />Treatment for fever/infections<br />
  37. 37. TREATMENT<br />SPECIFIC THERAPY<br />Steroids<br />Mild to moderate exacerbations<br />Oral prednisone1mg/kg/day<br />Severe exacerbations<br />IV methylprednisone 500 to 1000 mg/day for 3 to 5 days with taper<br />
  38. 38. TREATMENT<br />PREVENTIVE THERAPY FOR RELAPSES<br />Immunosuppressive agents<br />Interferon <br />
  39. 39. TREATMENT<br />SUPPORTIVE THERAPY<br />Fatigue<br />Vertigo<br />Muscle spasms<br />Tremors<br />Pain<br />Cognitive Dysfunction<br />Urinary dysfunction<br />Psychological problems<br />
  40. 40. PROGNOSIS<br />FAVORABLE FACTORS:<br />Females <br />Low rate of relapses per year <br />Complete recovery from the first attack <br />Long interval between first and second attack <br />Symptoms predominantly from afferent systems (i.e. sensory symptoms) <br />Younger age of onset <br />
  41. 41. PROGNOSIS<br />FAVORABLE FACTORS:<br />Low disability at 2 to 5 years from the disease onset <br />Later cerebellar involvement <br />Involvement of only one CNS system at the time of onset <br />
  42. 42. PROGNOSIS<br />UNFAVORABLE FACTORS:<br />Males <br />High rate of relapses per year <br />Incomplete recovery from the first attack <br />Short interval between first and second attack <br />Symptoms predominantly from efferent systems (i.e. symptoms of motor tract involvement) <br />Older age of onset <br />
  43. 43. PROGNOSIS<br />UNFAVORABLE FACTORS:<br />Significant disability at 2 to 5 years from the onset acute onset <br />Early cerebellar involvement <br />Involvement of more than one CNS system at the time of onset <br />
  44. 44. PROGNOSIS<br />Average life span after diagnosis is 25 to 35 years<br />Suicide rate is 7.5 times higher <br />Common causes of death<br />Compromised swallowing and breathing<br />Severe infections (e.g. Urosepsis, Aspiration pneumonia)<br />

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