MULTIPLE SCLEROSIS

MULTIPLE SCLEROSIS SAADIA AKHTAR, MD DEPARTMENT OF EMERGENCY MEDICINE BIMC

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

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

ETIOLOGY
Cause is still unknown
Identified factors:
Autoimmune causes
Human Leukocyte Antigens
Viral causes
Roseola virus

PATHOPHYSIOLOGY
Scattered areas of demyelination=
“ Plaques”
Plaques are more common in:
Optic tracts
Spinal cord
Brain stem
Basal Ganglia

PATHOPHYSIOLOGY
Demyelinated axons
Do not conduct normal action potentials
Hyperexcitable (generate action potentials with minimal stimuli)
Lesions are “scattered in space and time”

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%

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.

MOTOR SYMPTOMS
Upper motor neuron signs
Mild spasticity
Hyperreflexia
Monoparesis (one extremity)
Quadriparesis (all four extremities)

SENSORY SYMPTOMS
Ascending numbness starting in the feet
Bilateral hand numbness
Hemiparesthesia
Reduction of vibration
Reduction of proprioception

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

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

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)

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

Cerebellar signs
Incoordination (dysdiadochokinesia, problems with heel-to-shin test)
Slowing of rapid repeating movements
Ataxic gait
Abnormal speech
Loss of balance

Sensory systems
Lhermitte's sign
Paresthesia
Numbness
Dorsal column signs (severe decrease or loss of vibratory sense and proprioception, positive Romberg's test)

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

Cognitive and emotional abnormalities
Emotional lability
Depression
Anxiety
Fatigue

RELAPSING/REMITTING

SECONDARY PROGRESSIVE

PROGRESSIVE RELAPSING

PRIMARY PROGRESSIVE

DIFFERENTIAL DIAGNOSIS
Postinfectious Encephalomyelitis
Primary CNS Vasculitis
Lyme Disease
Systemic Lupus Erythematosus
Tropical Spastic Paraparesis

DIFFERENTIAL DIAGNOSIS
Behçet Syndrome
Sarcoidosis
Vitamin B-12 deficiency
Tertiary Syphilis
Progressive Multifocal Leukoencephalopathy

DIAGNOSIS
History
Physical Exam
Laboratory tests
Lumbar puncture
MRI
Electrophysiological tests (visual- evoked potentials)

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

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

ED PRESENTATONS
Exacerbation of previous deficits
Development of new deficits
Development of complications
Initial presentation

TREATMENT
General
Specific therapy
Preventive therapy for relapses
Supportive therapy

TREATMENT
GENERAL
Exercise
Physical therapy
Nutrition
Pregnancy
Treatment for fever/infections

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

TREATMENT
PREVENTIVE THERAPY FOR RELAPSES
Immunosuppressive agents
Interferon

TREATMENT
SUPPORTIVE THERAPY
Fatigue
Vertigo
Muscle spasms
Tremors
Pain
Cognitive Dysfunction
Urinary dysfunction
Psychological problems

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

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

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

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

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)

QUESTIONS?

MULTIPLE SCLEROSIS

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