Multiple Sclerosis - by MHR Corp

MULTIPLE SCLEROSISMOHD HANAFI RAMLEE

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)


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

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

Principal Differential Diagnosis of Multiple Sclerosis

Infection Lyme, Syphilis, Progressive Multifocal

Leukoencephalopathy, HIV, HTLV-1

Inflammatory SLE, Sjogren syndrome, vasculitis, Sarcoidosis,

Bechet’s disease

Metabolic B12 deficiency, lysosomal disorders,

adrenoleukodystrophy, mitochondrial disorders, other genetic diseases

Neoplastic CNS lymphoma

Spine disease Vascular malformations, degenerative spine disease

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

Treatments

Oral

Medications

Rehabili-tation

Local

Treatments

Surgical

Treatments

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)

Health & Medicine

Multiple Sclerosis - by MHR Corp