sgb retno ms

7/29/2019 Sgb Retno Ms

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GUILLAN-BARRE SYNDROME

(GBS) LANDRYS ASCENDING PARALYSIS,

ACUTE IDIOPATHIC POLYRADICULITIS

is an inflammatory disorder of the peripheralnerves.

Is characterized by weakness and numbness ortingling in the legs and arms, and possible loss ofmovement and feeling in the legs, arms, upper

body, and face.


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Guillain-Barre Syndrome

Synonyms Acute inflammatory demyelinating

polyradiculoneuropathy-AIDP Landry-Guillain-Barr syndrome,

Landry-Guillain-Barr-Strohl syndrome,

Acute idiopathic neuropathy,

Acute demyelinating neuropathy,

Infectious polyneuritis,

Acute polyradiculoneuritis,

Axonal Guillain-Barr syndrome, Acute motor axonal neuropathy,AMAN,

acute motor-sensory axonal neuropathy-AMSAN

Miller-Fisher syndrome,

Pharyngeal-cervical-brachial GBS


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Variants with rapid progressive phase:

AIDP(Acute Inflammatory Demyelinating

Polyradiculoneuropathy) = GBS

AMSAN (Acute Motor Sensory Axonal

Neuropathy

AMAN (Acute Motor Axonal Neuropathy)

MFS (Miller-Fisher Syndrome)Also known asMiller Fish syndrome, Miller's syndrome and

Acute Disseminated Encephalo-

myeloradiculopathy


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Variants with slow progressive phase:

CIDP (Chronic Inflammatory

Demyelinating Polyneuropathy)

MMN (Multifocal Motor Neuropathy

MMSD (Multifocal Motor SensoryDemyelinating Neuropathy

MADSAN (Multifocal Acquired

Demyelinating Sensory AcquiredNeuropathy

PDN (Paraproteinaemic Demyelinating

Neuropathy


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AIDP (Acute Inflammatory Demyelinating

Polyradiculoneuropathy) = GBS

About 80% of the patients get this form.

- AMSAN (Acute Motor Sensory AxonalNeuropathy

A serious axonal form of GBS that attacks

motor and sensory nerves. Fulminantcourse with slow and incomplete

recovery.

AMAN (Acute Motor Axonal Neuropathy) particularly severe form, attacks the motornerves primarily, causing rapid progressiveweakness often with respiratory failure. Pure

axonal cases may occur more frequently in

other parts of the world outside Europe and

North America. AMAN cases also may be

different from cases of axonal GBS describedin the West.

Many cases have been reported in rural areas

of China, especially in children and young

adults during the summer months.

Prognosis is often quite favorable and

recovery is rapid.


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Incidence

In developed countries, Guillain-Barre

Syndrome (GBS) is the most common causeof acute neuromuscular paralysis.

about 1 to 2 cases in every 100,000 people

per year ( 0,01%-0,02% of populations) in the United States afflicting about 5,000

persons annually

Age:All ages, Bimodal distribution, withpeaks in age ranges of 15-35 years and 50-

75 years

Sex:Male-to-female ratio is 1.5:1


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Incidence variant SGB(race)

demyelinating

polyneuropathy

Europe

Africa

Axonal degeneration

Axonal degenerationAsia

South America

demyelinating

polyneuropathy

North America

demyelinatingpolyneuropathy

demyelinating

polyneuropathy

Australia


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Causes autoimmune disease triggered by a preceding

bacterial or viral infection. Campylobacter jejuni,cytomegalovirus, Epstein-Barr virus andMycoplasma pneumoniae Chlamydia HepatitisB,mononucleosis, AIDS, and herpes simplex.

Sometimes Guillain-Barre occurs following

surgery or vaccinations (such as rabies and swineflu vaccines) or in association systemic lupuserythematosus orHodgkin's disease.

are commonly identified antecedent pathogens

A small number of casesto occur after a medicalprocedure, such as minor surgery.

Camden, NJ. Review provided by VeriMed Healthcare Network. Update

Date: 4/25/2004
http://www.nlm.nih.gov/medlineplus/ency/article/000594.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/001324.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000580.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000580.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/001324.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000594.htm


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Causes

GBS is a postinfectious immune-mediated diseasecellular and humoral immune mechanisms

Most patients report an infectious illness in the weeks priorto the onset of GBS.

Guillain-Barr syndrome may be an autoimmunedisorder in which the body produces antibodies thatdamage the myelin sheath that surrounds peripheral nerves.

The myelin sheath is a fatty substance that surroundsaxons. It increases the speed at which signals travel alongthe nerves.


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INFECTION COMMON INSIDEN = 13 - 72% SEVERE MOTOR

AKSONAL NEUROPHATY

Ab GM1

C jejuniHadden, 2001

INF C jejuni PREDICT PROGNOSIS severe inflammation 0f axonal

disruption and loss.


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PATOG : SEL T, MAGR, SITOKINE

MIP-1 a

MCP-1

EAN

Sitokine gerakleuk & macr & ek

MIP-1 a dan MIC-1Gold-2000, Kiesier-2000,

Fujioka-99


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Cytokines

Macrophage

TNF

IL-1

IL-1raIL-6

IL-12

IFN-gTNF, IL-6 IL-1, IL-1ra IL-12


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Histologic Findings

Lymphocyte and macrophage infiltration ofperipheral nerves. Macrophage influx

multifocal demyelination .

in severe inflammatory changesCellular

infiltratesWallerian degeneration

cranial nerves, nerve roots, dorsal root

ganglions, and peripheral nerves.


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GENE TRANSCRIPTION

NF-kBmRNA proteinmacrofag

Immun mediator

Damage peripheral nerve

1. Ekspression NF-kB correlationswith macropagh

2. Magr imun reactions Andorfer, 2001Jander, 2001


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Pathophysiology 4:

APOPTOSIS SEL Th1 Apoptosis genetic proggramed cell death Apop sel T cell (auto reactive) healing

(Gold et al., 1999).

Apop Schwann cell remyelinization nerveregenerations

Th1 TNF-a Apoptosis Schwann cell (Weishaupt et al., 2001).


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APOPTOSIS

Auto reactive

myelin-directed T cell

imun response-inflamation

Gold, 1999

Schwann cell

Remyelinisation +

Nerve regeneration Weishaupt, 2001


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Pathophysiology 5:

molecular mimicry

Campylobacter jejuniantecedent illness andGBS can be typified by infections.

The virulence presence of specific antigens in

its capsule. Immune responses ( against the capsular

components) produce antibodiesreact withmyelin demyelination.

Ganglioside GM1cross-react with C jejunilipopolysaccharide antigens immunologicdamage to the peripheral nervous system.


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INFECTION _ NEUROPHATY

MOLECULAR MIMICRYCONCEPT


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MOLECULAR MIMICRY CONCEPT

C jejuni

H. Influ

C jejuni sialicacid synthesaGM1,GD1, GT1 (Yuki, 2001)

Anti-GT1 IgG post infection H Infl

(Koga, 2

C jejunigene cst II activity(transkrsialic acid) (Van Belkum, 2001)


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Signs and Symptoms

The first symptoms: numbness or tingling(paresthesia) in the toes and fingers ( Gloveand stocking ) progressive weakness inthe arms and legs over the next few days.

Some paresthesia only in their toes and legs

Others only experience symptoms on oneside of the body.


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________________% of patient

Sensory symptoms

numbness 72pain 37

Sensory sign 62

Reflexes

normal 5

partial loss 17

complete loss 78


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__________________% 0f patient

urinary dysfunction 25

rectal disfunction 14 hypotension 14

hypertension 31

sinus tachycardia 36 Arrhythmia 16

hyponatremia (SIADH) 9


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Pain 50% described the pain severe and distressing due to direct

nerve injury or the paralysis and prolonged immobilization

inflamed nerve roots.back and leg pain as aching or throbbing, myalgic with

cramping, local muscle tenderness, visceral pain, and painassociated with conditions of immobility (eg, pressure nerve

palsies, decubitus ulcers).

Dysesthetic symptoms 50% of patients frequently are described asburning, tingling, or shocklike sensationsmore prevalent in thelower extremities persist indefinitely in 5-10% of patients.

Intensity of pain on admission correlates poorly with neurologicdisability on admission and end outcome.

C i l i l


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Cranial nerve involvement

Cranial nerve involvement 45-75%

Common complaints : Diplopias,Dysarthria,Facial droop, Dysphagia

Facial and oropharyngeal weakness usually

appears after the trunk and limbs areaffected.


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Weakness

Ascending and symmetricaldevelops acutely andprogresses over days to weeks mild weakness tocomplete tetraplegia with ventilatory failure.

Peak deficits are reached by 4 weeks . Recoveryusually begins 2-4 weeks after progression ceases.

The lower limbs usually are involved before theupper limbs. Proximal muscles may be involvedearlier and more severe than the distal onesTrunk,bulbar, and respiratory muscles can be affected.

Diagnosis


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Diagnosis The diagnosis of Guillain-Barr syndrome is based on

typical clinical features, electrodiagnostic examination,

and examination of the cerebrospinal fluid. An EMG (a test of electrical activity in muscles) showslack of nervous stimulation.

A CSF (cerebrospinal fluid) examination may beabnormal, showing increase in protein without increase in

white blood cell count. Electromyogram (EMG)diagnostic tool recordsmuscle activity, the loss of reflexes (slowing of nerveresponses. )

Nerve conduction velocity (NCV) records the speed

at which signals travel along the nerves. AnNCV (nerveconduction velocity) shows demyelination.

Lumbar puncture : An elevated level of protein in thefluid is characteristic of GBS.

Diagnostic Criteria for Typical Guillain-Barr
http://www.nlm.nih.gov/medlineplus/ency/article/003929.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003625.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003927.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003927.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003625.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003929.htm


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Diagnostic Criteria for Typical Guillain-Barr

Syndrome Features required for diagnosis

Progressive weakness in both arms and legs

Areflexia Features strongly supporting diagnosis

Progression of symptoms over days, up to four weeks

Relative symmetry of symptoms

Mild sensory symptoms or signs

Cranial nerve involvement, especially bilateral weakness of facialmuscles

Recovery beginning two to four weeks after progression ceases

Autonomic dysfunction

Absence of fever at onset

High concentration of protein in cerebrospinal fluid, with fewer

than 10 cells per cubic millimeter Typical electrodiagnostic features

Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barr syndrome. Ann Neurol 1990;27(suppl):S21-4.


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GBS EMERGENCY

SEVERE &ACUT PARALYSIS

Severe Weakness

BULBAR PARALISIS

RESP Failure

OTONOMIC Complications

MORTALITY (Hahn, 1998). (Raphael et al, 2000).

Respiratory involvement


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Respiratory involvement

40% of patients have respiratory and oropharyngeal weakness.

Typical complaints may include the following:

Dyspnea on exertion

Shortness of breath

Difficulty swallowing Slurred speech

Ventilatory failure required respiratory support in up to one

third of patients .


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Guillain Barre Disability Score

(Greenwood, Osterman, GBSG studies)

1=minor symptoms

2=able to walk 5 meters without assistance

3=able to walk 5 meters with assistance

4=confined to bed or chair5=requiring

assisted ventilation for at least part of the

day or night


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MANAGEMENT

Early intensive care

R) spesifik against otoantibody &reaktivitas el imunokomp IVIg (dosis & plasmaph(PE)

(Hartung et al., 2002).

R) General supportif & Complications


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GENERAL1. Respiratoy failure : Intubation; volume control

ventilation; antibiotics; glycopyrrolate (forcontrolling secretion & diarrhea).

2. Nutrition3. Prophylaxis against deep vein thrombosis and

pulmonary embolism4. Stress ulcer prophylaxis5. Prevention of decubitus ulcers6. Keeping patients head 30-45% above the bed7. Others (treat hypokalemia; hypophosphatemia; HCt 30-40/menit.

Tracheostomi bulbar palsy (aspiration) (Sharshar et al, 2003).


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ASSESSMENT OF SYMPTOMS FORINTUBATION AND MECHANICALVENTILATION (Leshner 1984)

SYMPTOM RESULT__________________________________________________________

___ Vital capacity

3 times predicted tidal volume Poor cough

2 times predicted tidal volume Intubation indicated Maximum respiratory forces

PE max 40 cm H2O Inability to clear secretionsPI max 20 cm H2O Marked weakness inspiratory

muscles Dysphagia with bulbar paralysis Danger of aspiration Arterial blood gases:

Hypoxia with normal PCO2 Probable severe ventilatoryimpairment

____________________________________________________________

PE = max. pressure on expiration; PI = max. pressure on inspiration


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Postponing intubation may lead to so-called "crash"intubation in the middle of the night which may haveadditional complications.

Time on the ventilator varies but most modern intensivecare series report a median of 30 days.

A tracheostomy is soon considered. In the vast majority ofpatients, this is usually postponed until

the third week after intubation,

bulbar symptoms or in thosesevere damage to the axonstracheostomy considered earlier

IVIg


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IVIg

Indication : severe paralysis, resp failure, bulbar

Dose = 0.4 g /kgBW/day (5 days)

2g Efektive 2 first week

R) gagal = 25%

trial Ig &prednisolon

Side effect (10%) headache (respon NSAID),myialgia,

aseptic meningitis(5%), Self limiting 24-48 hours

Profilak100mg Solu-Cortef IV anafilaksis (def IgA) rare (Raphael et al, 2000).


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High dose prednisone and imuran again put

me in remission, this time for two years. In

May 1995, I was started on IV-IG (80g) andimuran (175mg). The imuran did nothing

and was stopped after a year. In 1997, I was

put on low dose


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PLASMAPHERESIS = PE

CHANGE humoral immunity ventil time , motoris , squele Efektive < 7 days

Indication:paralisis prog, bulbar/resp

Dose = 200-250 ml/bw (5 X/10-14days)

albumin low Na 5%

Compl phlebitis & hypotension (Raphael et al, 2000).


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Complications:

Breathing difficulty (respiratory failure)

aspiration of food/fluids into the lungs pneumonia

increased risk of infections

deep vein thrombosis permanent loss of movement of an area

contractures of joints or other deformity

Complications
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Complications

Monitor heart rate, blood pressure, and cardiacarrhythmias allows early detection of life-threatening situations.

Antihypertensives and vasoactive drugs patientswith autonomic instability.

Enteric or parental feedings for patients onmechanical ventilation and severe dysphagia

to ensure that adequate caloric needs are met whenthe metabolic demand is high.


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Complications

Bowel and bladder dysfunction Initial

managementsafe evacuation and

prevention of overdistension.

Monitoring for secondary infections, such

as a urinary tract infection.


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Mortality/Morbidity:

In epidemiologic surveys, the overall death

rates range from 2-12% of patients.

GBS-associated mortality rates increasemarkedly with age. Though the death rate

increases with age

Males have a death rate 1.3 times greaterthan females after the age of 40 years.


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