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