MULTIPLE SCLEROSIS

MULTIPLE SCLEROSIS

SAADIA AKHTAR, MDDEPARTMENT OF

EMERGENCY MEDICINEBIMC

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.

CLINICAL PRESENTATION

• 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


• 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


• 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)

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


• Ongoing symptoms and signs– 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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