pa tho physiology of guillain barre syndrome

8/3/2019 Pa Tho Physiology of Guillain Barre Syndrome

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PATHOPHYSIOLOGY OF GUILLAIN

BARRE SYNDROME

ASSOCIATED WITHCAMPILOBACTER JEJUNI INFECTION


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PRECIPITATING FACTOR PREDISPOSING FACTOR

ETIOLOGY

Campilobacter Jejuni

Invasion of C. Jejuni through the GI tract

Activation of Immune Response

Migration to Regional Production of Activated Production of ActivatedLymph Nodes T-cells B-cells


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Formation of antigenpresenting cell in conjunction

to histocompatability complex

molecules

( homologous or identical

amino acids of pathogen and

GM1 ganglioside of peripheral

myelin sheath)

Production of activated T cells

Activation of CD4 that

recognizes antigens from the

infectious agent

Production of activated B cells

IgG IgM

activation of complement system

molecular mimicry

failure of the immune system

autoimmunity activation (activated T-cell

facilitates opening of the blood brain barrier)

production of antibodies that cross react with

peripheral myelin sheath

migration of lymphocyte and macrophage adjacent to the area


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A B C

macrophage cytokine TNFcell-mediated activation of

C5b-C9 membrane complex attack

attacks GM1 gangliosides of

peripheral myelin sheath

edema between myelin lamelae and vesicular disruption

Dull aching pains in the lower back, flank and proximal legsdemyelination of nerve segments

disruption in the propagation of electrical nerve impulses

peripheral nerve denrvation and atrophy

Autonomic Nervous System dysfunction

GUILLAIN BARRE SYNDROME


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A B C

IF TREATED EARLY IF TREATED LATE IF UNTREATED

Cell body survives

Regeneration of peripheral nerves

RECOVERY OF MOTOR FUNCTION

Cell body dies

collateral reinnervation from

surving axona

AXON REGENERATION

extensive axxonal destruction

bad prognosis

ascending weakness progresses

EARLY: muscle weakness, sensory changes,

paresthesia in feet or hands,

loss of reflexes begins with the peripheries

PROGRESSIVE: blurred vision, clumsiness and falling,

difficulty moving facial muscles,

muscle contraction, palpitations

EMERGENCY: Breathing temporarily stops,

Unable to take a deep breath,

DOB, Drooling, Fainting

Treatment and Medication


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W

eakening of the diaphragmand respiratory muscles

Respiratory insufficiency RDS

Dyspnea Respiratory arrest

Shock

DEATH


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Prepared by:Ralph Rhandall R. Espejo

Sheana V. Malillin

Gretchen I. MaguddayaoRafael C. Palattao


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-Age ( Age 16-25, 45-60y/o)

-Sex (more prevalent to men)

BACK


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Post infection to C. Jejuni

Hodgkins Lymphoma

Mononucleosis

poor hygiene

lifestyle/stress food poisoning

BACK


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Signs and Symptoms

Dull aching pains in the lower back, flank and proximal legs

Clinical History Assessment:

Paresthesia, paralysis-(+)

CSF findings(+)

Electromyogram-detects tiny electrical impulse in the muscle

BACK


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Signs and Symptoms

-Loss of sweating

-Sinus tachycardia

-Hyper/hypotension

CSF examination-unusually protein level of 600 mg/ml Cellular abnormality

Nerve conduction test= (-)PTR

MRI

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Diagnosis

diagnosis of GBS usually depends on findings such as rapid

development of muscle paralysis, hyporeflexia, absence offever, and a likely inciting event.

Cerebrospinal fluid analysis - typical CSF findings includealbumino-cytological dissociation. As opposed to infectiouscauses, this is an elevated protein level (1001000 mg/dL),without an accompanying increased cell count pleocytosis. A

sustained increased white blood cell count may indicate analternative diagnosis such as infection.

Electrodiagnostics

Electromyography (EMG) and nerve conduction study (NCS)may show prolonged distal latencies, conduction slowing,

conduction block, and temporal dispersion of compoundaction potential in demyelinating cases. In primary axonaldamage, the findings include reduced amplitude of the actionpotentials without conduction slowing.


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Diagnostic criteria

-Required:

-Progressive, relatively symmetrical weakness of two or more limbs due to neuropathy-Areflexia

-Disorder course < 4 weeks

Supportive:

-relatively symmetric weakness accompanied by numbness and/or tingling

-mild sensory involvement-facial nerve or other cranial nerve involvement

-absence of fever

-typical CSF findings obtained from lumbar puncture

-electrophysiologic evidence of demyelination from electromyogram

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SPEECH THERAPY

PHYSICAL THERAPY

EXCERCISE

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Ventilatory support(ventilator)

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Campylobacter jejuni is a species of curved, helical-shaped,

non-spore forming, Gram-

negative, microaerophilic bacteria commonly found in animalfeces. It is one of the most common causes of

human gastroenteritis in the world. Food poisoning caused

by Campylobacter species can be severely debilitating, but is

rarely life-threatening. It has been linked with subsequent

development of Guillain-Barr syndrome (GBS), which usually

develops two to three weeks after the initial illness.

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TREATMENT:

Patients who are diagnosed with GBS should be admitted to a hospital for close monitoring until ithas been determined that the course of the disease has reached a plateau or undergone reversal.

Although the weakness may initially be mild and no disabling, symptoms can progress rapidly overjust a few days. Continued progression may result in a neuromuscular emergency with profoundparalysis, respiratory insufficiency, and/or autonomic dysfunction with cardiovascularcomplications.

Approximately one third of patients require admission to an intensive care unit (ICU), primarilybecause of respiratory failure. After medical stabilization, patients can be treated on a generalmedical/neurologic floor, but continued vigilance remains important in preventing respiratory,cardiovascular, and other medical complications. Patients with persistent functional impairments

may need to be transferred to an inpatient rehabilitation unit.

Continued care also is needed to minimize problems related to immobility, neurogenic bowel andbladder, and pain. Early involvement of allied health staff is recommended.

Early recognition and treatment of GBS also may be important in the long-term prognosis,especially in the patient with poor clinical prognostic signs, such as older age, a rapidly progressingcourse, and antecedent diarrhea.

Immuno modulatory treatment has been used to hasten recovery. Intravenous immunoglobulin andplasma exchange have proved equally effective.


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MEDICATIONS

Immunomodulatory therapy, such as plasmapheresis or the administrationof intravenous immunoglobulins (IVIGs), is frequently used in GBS

patients.The efficacy of plasmapheresis and IVIGs appears to be about

equal in shortening the average duration of disease. Combined treatment

has not been shown to produce a further, statistically significant reduction

in disability.

The decision to use immunomodulatory therapy is based on the disease's

severity and rate of progression, as well as on the length of time between

the condition's first symptom and its presentation. Risks, such as

thrombotic events associated with intravenous immunoglobulin (IVIG),

should be taken into consideration. Patients with severe, rapidly

progressive disease are most likely to benefit from treatment, with faster

functional recovery.

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pa tho physiology of guillain barre syndrome

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