Multiple Sclerosis and Guillian Barre Syndrome

multiple sclerosis (MS)

• A chronic disease involving the demyelinaton of the central nervous system (CNS)

• Hard plaques (scar tssue) are formed over the nerve, limitng transmission of signals to & from the body

cns

brain & spinal cord (optic nerve is CNS)

pns

Nerves that extend from the brain & spinal cord to the rest of the body

exacerbation

when symptoms intensify or when new symptoms emerge & may be an episode as minor as fatgue & slight sensory loss or as extensive as total paralysis of all extremites & loss of bladder control

remission

when symptoms diminish or resolve, may involve total resoluton of the symptoms, slight return of some functon with the symptoms remaining, or a short plateau in which no new symptoms occur but the current symptoms remain

MS characteristics 

• Inflammaton, primary demyelinaton, & neurodegeneraton are the dominant features of both relapsing & progressive MS

• Inflammatory & non-inflammatory lesions of the myelin sheath (demyelinaton)

• Loss of myelin throughout the central nervous system (white & gray mater)

• Disrupton of neurotransmission (axonal conducton)

• Inflammaton as seen by the accumulaton of white blood cells around the CNS blood vessels

diagnosis of MS

Combinaton of clinical, imaging, & laboratory evidence

• Eliminaton of other/alternate neurological conditons that share symptomatology

‒ Systemic lupus

‒ Neuromyelits

‒ Neurosarcoidosis

• Relapses of clinical symptoms

• Evidence of objective lesions (spinal cord, brain)

‒ Large lesions, known as plaques, are ofen seen in the spinal cord (50%), optc nerves (25%), brainstem/cerebellum (20%), & periventricular white mater

• CSF (oligoclonal bands)

etiology of MS

• Combination of genetics, epigenetic, infections including Epstein-Barr virus (EBV), nutrition, climate, other environmental influences, sunlight exposure, & smoking

  • EBV is the most agreed upon MS risk factor especially if it arises afer childhood and is symptomatic

• Smoking was found to be a modest risk factor of MS, while the involvement of vitamin D & sun exposure needs to be further investigated

factors associated with exacerbations 

• These are not causatve

• Extreme fatigue

• Trauma

• Increased body heat

        ‒ Outdoor temperature

        ‒ Fever

        ‒ Hot bath or shower

signs and symptoms of MS

• Vary widely

• Several are covert symptoms

• The type of disease process will efect the expression of symptoms

• Dependent upon where the demyelinaton occurs within the CNS

• Visual*, motor, sensory, cognitve, psychological, & bowel & bladder system

impairments

‒ Visual defcits such as diplopia or unilateral optc neurits: among the earliest signs of MS

‒ Oculomotor control may also be afected due to lesions of the supranuclear connecton to the oculomotor nuclei in the brainstem; as a result, the person loses horizontal eye movement either unilaterally or bilaterally

80%

Nearly _ of all persons who have MS have some loss of visual acuity

CNS

MS can affect nearly any part of the _

• motor

• sensroy

• visual

• autonomic systems

40%-70%

approx. _ of individuals with MS experience some change in their cognitive ability

epidemiology of MS

Most common nontraumatc neurological disorder among adults under 40 years of age

• Diagnosed between ages 20 - 40 years, mainly in the 30s, average age 32

• Prevalence among women with ~149 per 100 000 women & 50 per 100,000 men in the United States

‒ Women are more afected than men- 3:1

• Rare in African descent

• High prevalence in North America & Europe to low rates in Eastern Asia & sub-Saharan Africa

countries affected by MS

Occurs in areas furthest from the equator

‒ Southern United States, the rate of incidence is 20-39 for every 100 000 persons

‒ Northern United States & Canada, the rate is ~100 out of every 100 000 persons

‒ Previous studies described a lattude gradient, with increasing risk from the equator to North &

South Poles, but this theory is still controversial

• Epidemiologists have seen an increased prevalence of MS in countries at high lattudes, where the sunlight is limited & where the populations have vitamin D defciency & high melatonin levels

• Familial recurrence rate of 20%-30% & is more common in first-degree relatives vs more distant relatives

relapsing & remetting

65-85% of the MS populaton, leads to episodes of exacerbaton & remission of symptoms that result in a slow, stepwise progression as the defcits accumulate

secondary progressive

begins with a pattern of relapses & remissions but evolves into the progressive form of the disease given time

• before the introduction of disease-modifying drugs, approximately 50% of clients with relapsing & remitting MS progressed to the secondary progressive form of the disease

• Will develop in approximately 60% of children

primary progressive

10% of the MS populaton, is distnguished by a downward course with episodes of exacerbatons or remissions

progressive relapsing

the least common of the four types of MS

• This type of MS accounts for approximately 5% of the MS populaton

• Experience a steady worsening of symptoms but with episodes of exacerbatons

clinical isolated syndrome (CIS)

A first episode of neurologic symptoms typical of an MS relapse in a person not known to have MS

‒ 63% progress to MS

course and prognosis of MS 

10 years post onset: ~10% will be wheelchair bound & about 50% will be unable to work

• Median life expectancy: 74.7 years (compared to healthy, 81.8 years)

‒ Female age > male

‒ Primary progressive < other forms

‒ Individuals with MS typically live 6 years less than those without MS

‒ Approximately 50% of individuals with MS live for at least 30 years afer onset of the disease, 50% die of complications of MS

• Changes in cogniton & mood ofen occur

complex attention, processing speed, & verbal memory

The volume of lesions seen on MRI correlates with cognitve decline in the areas of _

typical medical interventions

Ant-inflammatory medicatons, such as prednisone and methylprednisolone, help reduce symptoms & shorten the duraton of the exacerbaton

disease modifying drugs

• Interferon B1b

• Glatramer acetate

• Azathioprine

• Fingolimod

• Disesear-modifying: promote anibodies

• Natalizumab

• Rituzimab

• Alemtuzumab

beta blockers 

spastcity

anticholinergic

swallowing

steroids

nerve inflammation

cannabis extract

pain & spastcity

cognitve symptoms

Acetylcholinesterase inhibitors (donepezil, rivastgmine, galantamine [developed for Alzheimer’s disease]

fatigue

the most common symptom

• A covert problem

• Originated as nerve fber fatgue

• Stronger muscle groups overwork

• Overall cardiac ftness will affect this symptom

weakness

Results from:

‒ UMN weakness

‒ Fatigue

‒ Disuse

‒ Overriding antagonist spastcity

• Strengthening can not alter the progression of the disease

spasticity 

• May be caused by hypersensitvity

• Also may be a case of primal reflexes becoming uninhibited

• Occurs in larger muscle groups

• Can be painful and debilitatng

• Ofen requires medicaton

balance

• Cerebellar problems are common

• Ataxia, incoordinaton, dysmetria, & tremor exist in the trunk & extremites

• Vertigo is a less-seen problem

• Increases the risk of falls and self-injury in the populaton

sensory dysfunction

• Hypersensitvity or diminished sensaton may be seen

• Loss of pain & temperature is common

• Increases risk of self injury

• Several chronic pain issues in MS originate from this

cognitive dysfunction

• High level cognitve dysfuncton is common

• Most problems in memory, reasoning, & insight into disability

• Processing speed may be affected

• Due to loss of communication in integration areas of cortex

psychosocial components 

• Depression is more common

• Increased anxiety in the patent & their family

• Increased family stressors

• Grieving is ofen repeated

• Previous coping & characteristcs of disease impact psychosocial efect

Guillain Barre Syndrome

• ‘Acute inflammatory demyelinatng polyradiculoneuropathy’

• Acute, short-term motor neuropathy and flaccid paralysis

‒ The body’s own immune system atacks part of the peripheral nervous system

• No known cause or cure, and no one knows why this disorder strikes some people and not others

• Ofen occurs afer an upper respiratory or gastrointestnal viral infecton, usually within a few days or weeks

• The syndrome also occasionally occurs afer surgery, injury, or vaccinatons & occasionally occurs with no known precipitatng event

course of GBS

‘

1 to 2 cases per 100,000

• Variant types, acute motor or sensory axonal neuropathy, Miller Fisher syndrome

• What happens

‒ Immune system destroys Myelin sheath...afects signal transmission

‒ Inflammaton

‒ Schwann cell

• Why happen

‒ Presence of previous infectons...autoimmune response...campylobacter Jejuni, cytomegalovirus

epidemiology of GBS 

the incidence of GBS increases with age, & people over age 50 are at greatest risk for developing GBS

• On average, each year about 3000 to 6000 people in the United States develop GBS whether or not they received a vaccinaton

• Afects males slightly more ofen than females

• Incidence of GBS in the United States is approximately 1 to 2 persons in 100,000

vaccination & GBS

>99% of GBS clients have not had the flu vaccine recently

• Rumors of an associaton come from 1976 & swine flu vaccine of that time

• GBS clients ofen report flu-like symptoms prior to onset of weakness

• Patents with a history of GBS are cautioned about vaccines

GBS characteristics

Inital symptoms of GBS most ofen include varying degrees of weakness & sensory changes in the legs

• GBS is characterized by rapidly progressive ascending symmetric weakness of bilateral extremites, usually proceeding from distal to proximal (feet to trunk)

‒ Descending paralysis with predominant proximal muscle weakness rarely appears

‒ Typically, the weakness & abnormal sensatons frst notced in the legs progress to the arms & upper body, & some muscles are almost totally paralyzed

‒ As the demyelinaton contnues, the client may experience problems with breathing, speaking, swallowing, blood pressure, or heart rate

‒ The client may require use of a respirator to assist with breathing & is monitored closely for an abnormal heart rhythm, infectons, blood clots, & high or low blood pressure

‒ Afer the inital clinical manifestatons of the disease, the symptoms can progress over the course of days or weeks. oy the third week of the illness, 90% of all clients are at their weakest.

inital or acute phase

begins with the client’s first conclusive symptoms & lasts untl there is no further decline in physical status

‒ This phase may last for up to 4 weeks

plateau phase

begins when the client’s physical state stabilizes, with no further deterioraton of physical status and no evidence of physical recovery

‒ Generally lasts a few weeks, during which the physical status of the client remains unchanged

recovery phase

period when the client slowly begins to recover physical abilites & symptoms gradually decrease (usually 2–4 weeks afer disease progression ceases)

‒ Recovery can occur over 6 months up to 2 years, depending on the extent of paralysis

20%–30%

although complete recovery is possible, many individuals (_ of those diagnosed with GBS) will experience long-term residual defcits

symptoms of GBS

• Ascending symmetrical weakness

• Sensory symptoms

• Autonomic dysfuncton

• Diminished deep tendon reflexes

• Cranial nerve involvement