multiple sclerosis
TRANSCRIPT
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MULTIPLE SCLEROSIS
SAADIA AKHTAR, MDDEPARTMENT OF
EMERGENCY MEDICINEBIMC
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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
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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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ETIOLOGY
• Cause is still unknown
• Identified factors:– Autoimmune causes
• Human Leukocyte Antigens
– Viral causes• Roseola virus
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PATHOPHYSIOLOGY
• Scattered areas of demyelination= – “Plaques”
• Plaques are more common in:– Optic tracts– Spinal cord– Brain stem– Basal Ganglia
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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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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%
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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.
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CLINICAL PRESENTATION
• MOTOR SYMPTOMS– Upper motor neuron signs
• Mild spasticity• Hyperreflexia• Monoparesis (one extremity)• Quadriparesis (all four extremities)
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CLINICAL PRESENTATION
• SENSORY SYMPTOMS– Ascending numbness starting in the feet – Bilateral hand numbness– Hemiparesthesia– Reduction of vibration– Reduction of proprioception
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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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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
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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)
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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
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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
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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)
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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
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CLINICAL PRESENTATION
• Ongoing symptoms and signs– Cognitive and emotional abnormalities
• Emotional lability• Depression• Anxiety
– Fatigue
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RELAPSING/REMITTING
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SECONDARY PROGRESSIVE
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PROGRESSIVE RELAPSING
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PRIMARY PROGRESSIVE
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DIFFERENTIAL DIAGNOSIS
• Postinfectious Encephalomyelitis
• Primary CNS Vasculitis
• Lyme Disease
• Systemic Lupus Erythematosus
• Tropical Spastic Paraparesis
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DIFFERENTIAL DIAGNOSIS
• Behçet Syndrome
• Sarcoidosis
• Vitamin B-12 deficiency
• Tertiary Syphilis
• Progressive Multifocal Leukoencephalopathy
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DIAGNOSIS
• History
• Physical Exam
• Laboratory tests
• Lumbar puncture
• MRI
• Electrophysiological tests (visual- evoked potentials)
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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
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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
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ED PRESENTATONS
• Exacerbation of previous deficits
• Development of new deficits
• Development of complications
• Initial presentation
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TREATMENT
• General
• Specific therapy
• Preventive therapy for relapses
• Supportive therapy
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TREATMENT
• GENERAL– Exercise– Physical therapy– Nutrition– Pregnancy– Treatment for fever/infections
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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
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TREATMENT
• PREVENTIVE THERAPY FOR RELAPSES– Immunosuppressive agents
• Interferon
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TREATMENT
• SUPPORTIVE THERAPY– Fatigue– Vertigo– Muscle spasms– Tremors– Pain– Cognitive Dysfunction– Urinary dysfunction– Psychological problems
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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
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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
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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
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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
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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)
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QUESTIONS?