GUILLAIN BARRЀ SYNDROME

8/8/2019 GUILLAIN BARRЀ SYNDROME

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San Beda College – College of Nursing ORTHOPEDICNURSING

GUILLAIN – BARRЀ SYNDROMEI. Definition:

Guillain-Barre syndrome is anautoimmune disorder affecting peripheralnervous system (PNS), usually triggered by

an acute infectious process. Disorder in which the body’s defense system

attacks its own body cells. The defensesystem attacks myelin, the insulationcovering the nerves. The myelin is damagedand nerve can’t send signals, which in turnparalyzes the muscles.

GBS is a demyelinating disease, meaningthat segments of myelin are stripped fromtheir insulating position around nerves,reducing the propagation of electrical nerveimpulses.

This causes loss of reflexes, muscle weakness, and temporary paralysis (loss of musclestrength).

characterized by the acute onset of peripheral and cranial nerve dysfunction This is a serious disorder because of the extent to which Nervous system is affected. The

cause is IDIOPATHIC but this is believed to be due to autoimmune, meaning the body’sdefense system attack its own body cells

II. Synonyms:

Landry-Guillain-Barre syndrome; GBS; Acute idiopathic polyneuritis; Acute Inflammatory

Polyradiculoneuropathy ; Post-infectious Polyneuritis

III. Anatomy and Physiology:NERVOUS SYSTEM

The Nervous System consists of brain, spinal cord and complex network of neurons. Thissystem is responsible for sending, receiving and interpreting information from all parts ofthe body.

It monitors and coordinates internal organ function and responds to changes in externaenvironment

This system can be subdivided into 2 parts - The CENTRAL and PERIPHERAL NERVOUSSYSTEM. Let’s take a look at the Peripheral Nervous System:FUNCTION: Controll of all motor, sensory, autonomic, cognitive and behaviora

activities.




The NEURONFunctional Unit of the Nervous Systemo Dendrites: receive neural messages and transmit towards cell bodyo Axon: transmits neural messages away from cell bodyo Cell body: contains nucleus, mitochondria ad other organelleso Myelin Sheath produced by Schwann cells: Covering of axon, insulator and facilitate

conduction of neural impulses; fatty and protein material that surrounds certain nervefibers of brain and spinal cord

o glial cells forming the myelin sheaths are called oligodendrocytes in CNS while it iscalled Schwann cells in PNS

o Rapid rate of conduction is called Saltatory Conductiono Nodes of Ranvier: gaps between Myelin Sheath where saltatory conduction (jumping of

impulse between Myelin Sheaths) takes place

Types of Neurons:• Sensory Neurons typically have long dendrites and short axon, carry messages

from sensory receptors → CNS

• Motor Neurons have a long axon and short dendrites, transmit messages fromCNS → muscles (or to glands)

• Interneurons are found only in CNS were they connect neuron to neuron

• Afferent Neurons: from tissues and organs into the CNS (sensory neurons)

• Efferent Neurons: from CNS to the effector cells (motor neurons

Peripheral Nervous System Divisions: The PNS is divided into following sections:

o Somatic NS – controls skeletal muscle as well as external sensory organso Autonomic NS – controls involuntary muscles, such as smooth and cardiac

muscles

Sympathetic NS – controls activities that increase energy expenditures Parasympathetic NS - controls activities that conserve energy

expenditures

Peripheral Nervous System – nerves and ganglia outside the CNS

A.Nerve Structure and connective tissue sheath:




Fiber – endoneuriumFiber bundle/ Fascicle – perineuriumNerve – epineuriumAll Spinal nerves are mixed – sensory (afferent arm) and motor (efferent arm)

Spinal Cord – continuation of the brain stem; conduction pathway to and from the braincovered by meninges; Major reflex center; gives rise to 31 pairs of Spinal nervesConnection between the brain and peripheryApprox. → 45 cm (18 inches); thickness of the finger

surrounded by a clear fluid called Cerebrospinal Fluid that acts like a cushion The nerves within the spinal cord are grouped together in different bundles called

Ascending and Descending tracts.o Ascending tracts – carry information from the body, upward to the brain, such as

touch, skin temperature, pain and joint position.o Descending tracts - carry information from the brain, downwards to initiate

movement and control body functions

NORMAL CSF FINDINGSPressure <20 cm H2OColor/Appearance Clear, ColorlessCells 0-5 small lymphocyte/mm2

Protein 15-45 mg/dlImmune gammaglobulin (IgG)

3-12% of total CHON

Albumin/Globulin ratio 8:1Glucose 50-75 mg/dl or 60-70% of

blood glucose levelLactic acid 10-25 mg/dl

Spinal Nerves / Nerve roots come off the spinal cord and pass out through a hole ineach of the vertebrae called Foramen to carry the information from the spinal cord to therest of the body, and from the body back up to the brain.

O Cervical Nerves “C” : nerves in neck; supply movement and feeling to the arms,neck and upper trunk

O Thoracic Nerves “T”: nerves in upper back; supply trunk and abdomenO Lumbar Nerves “L”, Sacral Nerves “S”: nerves in lower back; supply the legs,

bladder, bowel and sexual organs

SPINAL NERVES

Plexus ImportantNerve

Body Areas Served Result of Damage

CervicalC1 – 5

Phrenic nerve DiaphragmShoulders and neck muscles

Respiratory paralysis

BrachialC5-8 T1

Axillary nerve Deltoid muscle Paralysis

Radial nerve Triceps brachii, extensors of forearm

Wristdrop – inability to extendhand or wrist

Median nerve Flexors of forearm and hand Inability to pick up smallobjects (pincer grasp)Ape hand

Musculocutaneou Flexors of arm Inability to flex forearm or arm




sUlnar nerve Wrist and hand muscles Claw hand – inability to spread

fingers apartLumbarL1-4

Femoral nerve Lower abdomen, buttocks,Ant. thighs, skin of anteromedialleg and thigh

Inability to extend leg and flexhip, loss of cutaneoussensation

Obturator Adductor muscle of medial thighSkin of medial thigh and hip joint

Inability to adduct thigh

SacralL4-5 S1-4

Sciatic nerve Lower trunk, posterior thigh andleg

Sciatica, inability to extend hipand flex knee

Commonperoneal / fibular

Lateral aspect leg and foot Footdrop – inability to dorsiflexthe foot

Tibial nerve Posterior aspect leg and foot Shuffling gait – inability toplantarflex and invert foot

Superior andInferior glutealnerve

Gluteus muscles Inability to extend hipInability to abduct andmedially rotate the thigh

B.Cranial Nerves

CN Name Function TestI Olfactory Purely sensory

Carries Impulses for sense of smellSniffing different aromas

II Optic Purely sensoryCarries impulses for vision

Eye chart and visual field testingUse of ophthalmoscope

III Oculomotor Motor fibers to eye muscles,eyelid, lensControls pupil size/lens shape

Following moving objects Test for convergence; papillaryreflexes

IV Trochlear Motor fibers to the superior obliquemuscle

Following moving objects

V Trigeminal Sensory fr. skin of face, nose andmouthMotor to chewing muscles

Sensation on face,corneal reflextesting, opening mouth againstresistance, moving jaw

VI Abducens Motor to lateral rectus muscle Following moving objects – lateral eyemovements

VII Facial Motor to muscles of facialexpressionto tear glands, salivary glandsSensory fr. taste buds on anteriortongue

Taste tongue for tastes – sweet, salty,sour, bitterClose eyes, smile and whistle

VIII VestibulocochlearOr Acoustic

Purely sensory – sense of balance(vestibular) and hearing (cochlear)

Hearing test using tuning fork forbone and air conduction

IX Glossopharyngeal

Motor to pharynx; swallowing andsaliva productionSensory for taste buds – posteriortongue

Gag reflex; swallowing and coughing Test tongue for tastes

X Vagus Sensory and motor – pharynx,larynx, thoracic visceraMotor parasympathetic fibers –regulate digestion and cardiacactivities

Gag reflex and swallowingcoughing

XI Accessory Motor to Sternocleidomastoid and Trapezius

Rotate head, shrug shoulders againstresistance




XII Hypoglossal Motor to tongue, sensory fromtongue

Stick out tongue

IV. Pathophysiology:

Predisposing

Factors:Age (All ages)Sex – common inmales

Precipitating Factors:

Post infection of Campylobacter jejuniPoor immunologic statusViral infection; CMV, EBV, VZVMycoplasma (bacteria that causePNM)Post GIT and lung infection; Stress

Bacteria, otherviruses (e.g. C.

jejuni)

Enters the body by the use of multifenestrated cells or othermechanisms

Initiate immune response; activation of the CD4 Tcells and B cells that recognize the presence of pathogens – this produces cell- mediated andhumoral response against the pathogen

Infectious organisms contain an aminoacid that mimics the peripheral nervemyelin proteins

Molecular

The immune system cannot distinguish betweenthe two CHONs and attacks and destroys the

Lymphocytes and macrophagescirculate in the blood and eventually

Immune response directed against the capsularcomponents produce antibodies that cross-react

Lymphocytic infiltration of the spinal roots andperipheral nerves and followed by macrophages-mediated multifocal stripping of the myelin and




IF treated:IF Not treated:

Defects on the propagation of electrical nerve impulses, with

GUILLAIN BARRЀSYNDROME

Sensory Changes:

• Paresthesia ornumbness in feetand hands

Acute progressiveASCENDINGWEAKNESS:-start from the legsand progresses upward• lower limbs

• upper limbs

• hyporeflexia• diminished reflexes

Demyelination of thenerves that innervatethe diaphragm andintercostals musclesresult inneuromuscular

Dull aching pain of the lower back,flank, proximal legsd/t demyelination of sensory fibers

Cranial Nerveinvolvement:

• 7th, 9th & 10th CN Difficulty forming

words (Dysarthri Dysphagia Difficulty of

breathing,speaking

Blindness (opticnerve /CN 2damage)

Vagus nervedamage –instability of CV

Don’t affect

LOC/Cognitive

Function

Common:

Typical GBS

>Plasmapheresis>IVIG>Physical therapyand exercise>Medications

GOOD PROGNOSIS

Extensive axonaldestruction

Ascending weaknessprogresses

Weakness of thediaphragm and therespiratory muscles

Respiratory Distress

BAD PROGNOSIS

Respiratory ArrestShockDeath




V. Laboratory and Diagnostic Procedures:Guillain-Barre syndrome can be difficult to diagnose in its earliest stages. Its signs

and symptoms are similar to those of other neurological disorders and may vary fromperson to person.

The first step in diagnosing Guillain-Barre syndrome is to take a careful medicahistory to fully understand the cluster of signs and symptoms you're experiencing.DIAGNOSTIC CRITERIA FOR GBS:

Features required for diagnosis - Progressive weakness of both legs

& arms areflexia

Clinical features supportive of diagnosis

Progression over days to 4 wks Relative symmetry of signs

Mild sensory symptoms or signs Cranial nerve involvement (bifacial

palsies)

Recovery beginning 2-4 wks afteprogression ceases

Autonomic dysfunction

Absence of fever at onset Laboratory features supportive o

diagnosis Elevated cerebrospinal fluid protein

with <10 cells/µl Electro diagnostic features of nerve

conduction slowing or block

Spinal tap (lumbar puncture)This procedure involves withdrawing a small amount of fluid from your spinal canal

at your low back (lumbar) level. This cerebrospinal fluid is then tested for a specific type ofchange that commonly occurs in people who have Guillain-Barre syndrome.A spinal tap (lumbar puncture) and nerve function tests are commonly used to help

confirm a diagnosis of Guillain-Barre syndrome.

Nerve function tests Your doctor may want informationfrom two types of nerve function tests —electromyography and nerve conductionvelocity:

o Electromyography reads electricalactivity in your muscle to determine if your weakness is caused by muscledamage or nerve damage.

o Nerve conduction studies assesshow your nerves and muscles respondto small electrical stimuli.

o A delay in F waves is present,implying nerve root demyelination.

o Nerve motor action potentials may be decreased. This is technically difficult todetermine until the abnormality is severe.

o The extent of decreased action potentials correlates with prognosis.

If Guillain-Barré syndrome is suspected, patients should be admitted to a hospital for




electrodiagnostic testing, CSF analysis, and monitoring by measuring forced vital capacity every 6 to 8 h.

Initial electrodiagnostic testing detects slow nerve conduction velocities andevidence of segmental demyelination in 2/3 of patients; however, normal results donot exclude the diagnosis and should not delay treatment.

CSF analysis may detect albuminocytologic dissociation (increased protein >45mg/dl but normal WBC count), but it may not appear for up to 1 wk and does not developin 10% of patients.

MRI

o MRI is a sensitive but nonspecific test.

o Spinal nerve root enhancement with gadolinium is a nonspecific feature seen ininflammatory conditions and is caused by disruption of the blood-nerve barrier.

o Selective anterior nerve root enhancement appears to be strongly suggestive of GBS.

o The cauda equine nerve roots are enhanced in 83% of patients.

Forced vital capacityo Forced vital capacity (FVC) is very helpful in guiding disposition and therapy.

o Patients with an FVC less than 15-20 mL/kg, maximum inspiratory pressure less than30 cm H2 O, or a maximum expiratory pressure less than 40 cm H2 O generallyprogress to require prophylactic intubation and mechanical ventilation.

Many different abnormalities may be seen on ECG, including second-degree and third-degree atrioventricular (AV) block, T-wave abnormalities, ST depression, QRS widening,and a variety of rhythm disturbances.

VI. Medical-Surgical Management:

a. Plasmapheresis. AKA Plasma Exchange, is used to remove proteins, calledantibodies, from the blood. The process involves taking blood from the body, usually fromthe arm, pumping it into a machine that removes the antibodies, then sending it back intothe body.o type of "blood cleansing" in which damaging antibodies are removed from your

blood.o removing the liquid portion of your blood (plasma) and separating it from the actual

blood cells. The blood cells are then put back into your body, which manufacturesmore plasma to make up for what was removed.

o plasmapheresis rids plasma of certain antibodies that contribute to theimmune system attack on the peripheral nerves.

o helps when done early in the syndrome; it is used if γ-globulin is ineffective.

Plasmapheresis is relatively safe, shortens the disease course and hospital stay, andreduces mortality risk and incidence of permanent paralysis. Plasmapheresis removesany previously administered γ-globulin, negating its benefits.

b. Intravenous immunoglobulin. Immunoglobulin contains healthy antibodiesfrom blood donors. High doses of immunoglobulin can block the damaging antibodies thatmay contribute to Guillain-Barre syndrome.

o used to reduce the severity and length of Guillain-Barre symptoms.

o the immunoglobulins are added to the blood in large quantity, blocking theantibodies that cause inflammation; promote remyelination

http://www.nlm.nih.gov/medlineplus/ency/article/002223.htm

http://www.nlm.nih.gov/medlineplus/ency/article/002223.htm




c. If the diaphragm is week, breathing support or even a breathing tube and mechanicaventilator may be needed. – ptx. is weaned in when spontaneous respiration isestablished.

d. Pain is treated aggressively with anti-inflammatory medicines andnarcotics, if needed.

e. Proper body positioning or a feeding tube may be used to prevent choking during feedingif the muscles for swallowing are weak.

f. Anticoagulant may be used to prevent blood clots. – heparin as DVT prophylaxis.

g. Intubation should be performed on patients who develop any degree of respiratory failure. Clinical indicators needed for intubation: hypoxia, rapidlydeclining respiratory function, poor or weak cough, and suspected aspiration.

Typically, intubation is indicated when the FVC is less than 15 mL/kg.h. Monitor closely for changes in blood pressure, heart rate, and other arrhythmias.

o Treatment rarely is needed for tachycardia. But may give Digitalis, Ca+ ChannelBlockers, VND drugs – Verapamil, Nefedipine, Diltiazem and Beta blockers , OLOLdrugs- Atenolol, Nadolol, Metoprolol

o Atropine is recommended for symptomatic bradycardia. (may also useIsoproterenol)

o Because of the lability of dysautonomia, hypertension is best treated with short-acting

agents, such as a short-acting beta-blocker or nitroprusside (peripheralvasodilator).

o Hypotension of dysautonomia usually responds to intravenous fluids andsupine positioning.

o Temporary pacing may be required for patients with second-degree and third-degreeheart block.

VII. Nursing Management:

Monitor Vital Signs, esp. BP and HR toidentify autonomic dysfunction; vitalcapacity, breath sounds, and ABG

Keep airway and tracheostomy set at thebed side

Suction, provide emotional support to theclient and family because of the severityand lengthy convalescent period

If patient can’t swallow due to bulbarparalysis (immobility of muscles),gastrostomy tube may be placed toadminister nutrients

Assess return of gag reflex and bowelsound before resuming oral nutrition

Prevent complications of immobilityo Skin Careo Padding may be placed at the

bony prominenceso ROM exerciseso Position changeso Coughing and deep breathing

exerciseso Antiembolism stockings

Provide explanations of disease processand care

Encourage client to verbalize feelings

GUILLAIN BARRЀ SYNDROME

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