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MULTIPLE SCLEROSIS

• SAADIA AKHTAR, MD
• DEPARTMENT OF
• EMERGENCY MEDICINE
• BIMC

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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 gt50 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
• Uhthoffs 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?