Guillain-Barré SyndromeGuillain-Barré Syndrome “Ascending Paralysis “ “Ascending Paralysis “

AI-Destruction-Nodes of RanvierAI-Destruction-Nodes of Ranvier

Guillain-Barré SyndromeGuillain-Barré Syndrome

Acute inflammatory demyelinating polyneuropathy (AIDP) caused by an autoimmune disorder affecting the peripheral nervous system,usually triggered by an acute infectious process

characterized by ascending paralysis.

Demyelination of Nerve Demyelination of Nerve FibersFibers

Positive conduction abnormalities

generations of ectopic impulses, spontaneous and abnormal “crosstalk” between demyelinated axons

Negative conduction abnormalities

slowed axonal conduction, variable conduction blocks occur in the presence of high- but not -low frequency volleys of impulse.

55IMMUNE RESPONSE

ImmunopathogenesisImmunopathogenesisAcute autoimmune disorderAcute autoimmune disorder

There is involvement of T There is involvement of T and B lymphocytes –and B lymphocytes –↑↑cytokines and cytokine receptors cytokines and cytokine receptors in serum (IL 2, soluble IL 2 in serum (IL 2, soluble IL 2 receptor) and CSF (IL 6, TNF receptor) and CSF (IL 6, TNF αα, , interferon) interferon)

Brain is unable to send Brain is unable to send messagesmessages

Legs and arms are Legs and arms are commonly affectedcommonly affected

EtiologyEtiology 75% of cases are preceded by 75% of cases are preceded by

an acute infectious process an acute infectious process usually GI or Respiratory in usually GI or Respiratory in originorigin

20-35% of cases are preceded 20-35% of cases are preceded by a by a Campylobacter jejuni, HV, Campylobacter jejuni, HV, EBV EBV infection.infection.

Recent:Recent: swine influenza swine influenza vaccine vaccine

Destruction most often occurs Destruction most often occurs in segments between the in segments between the Nodes of RanvierNodes of Ranvier

Source: Harrison Internal medicine pp 2509

Immune response to foreign antigens that are mistargeted at host nerve tissues instead.

Why Nodes of Ranvier are Why Nodes of Ranvier are the target of attack? the target of attack?

Neural targets are likely to be gangliosides

Gangliosides are complex glycosphingolipids that contain one or more sialic acid residue

Gangliosides are present in large quantities in human nervous tissues andin key sites: NODES OF RANVIER

Pathophysiology of GBS Pathophysiology of GBS

Antigens enterinto the body by

multifenestrated cells

Innate immune response results in the uptake of the

pathogens by immature APC

Production of antibodies and Phagocytosis of the bacteria

B cells are activated by newly activated Th2 cells. This

produces a cell-mediated and humoral response against the

pathogen.

Etiology Autoimmue Campylobacter jejuni Virus

EBVHVSIV

Molecular mimicry

Immune responses directed against the capsular components,

produce antibodies that cross-react with myelin.

Lymphocytes and macrophages circulate in the blood and eventually

find myelin.

Migration to lymph nodes , a mature, differentiated APC

activate CD4 T cells that recognize antigen from the

infectious pathogen

Lymphocytic infiltration of spinal roots and peripheral nerves,

followed by macrophage-mediated, multifocal stripping of myelin causing

axonal damage

Defects in the propagation of electrical nerve impulses with eventual conduction blocks

Guillain–Barré syndrome

Sensory changes:

Paresthesia or numbness in the hands/feet

Dull aching pains of the lower back,

flank or lower legs

Acute progressive ascendingweakness

Cranial nerve involvement:

facial droop(VII), dysphagia (V),

M. Fishers Syndrome

Number Name General Function Specific FunctionI Olfactory Sensory SmellII Optic Sensory VisionIII Oculomotor Motor, Parasympathetic Motor to four of six eye muscles and

upper eyelid; parasympathetic: constricts pupil; thickens lens

IV Trochlear Motor Motor to one eye muscleV Trigeminal Sensory, Motor Sensory to cornea face and teeth; motor

to muscles of mastication

VI Abducens Motor Motor to one eye muscleVII Facial Sensory,Motor,

ParasympatheticSensory: taste; motor to muscles of facial expression; parasympathetic to salivary and tear glands

VIII Vestibulo-cochlear Sensory Hearing and balance

IX Glossopha-ryngeal Sensory,Motor, Parasympathetic

Sensory: taste and touch to back of tongue; motor to pharyngeal muscles; parasympathetic to salivary glands

X Vagus Sensory,Motor, Parasympathetic

Sensory to pharynx, larynx, and viscera; motor to palate, pharynx, and larynx; parasympathetic to viscera of thorax and abdomen

XI Accessory Motor Motor to 2 neck and upper back muscles

XII Hypoglossal motor Motor to tongue muscles

Cranial Nerves and Their Functions Test

Fatigue Scale Scale Assessment Assessment

Muscle Strength Muscle Strength Assessment Assessment

ComplicationsComplications

Breathing difficulties Breathing difficulties Residual numbness or other sensations Residual numbness or other sensations Long term complications:Long term complications: Serious, permanent problems with sensation and Serious, permanent problems with sensation and

coordination, including some cases of severe coordination, including some cases of severe disability disability

A relapse of Guillain-Barre syndrome A relapse of Guillain-Barre syndrome Rarely, death from complications such as Rarely, death from complications such as

respiratory distress syndromerespiratory distress syndrome

Nursing DiagnosisNursing Diagnosis

1. Acute Pain r/t stimulation of free nerve endings 1. Acute Pain r/t stimulation of free nerve endings 2ndary to nonsynaptic transmission of nerve axons.2ndary to nonsynaptic transmission of nerve axons.

2. Self care deficit r/t decrease strength and endurance. 2. Self care deficit r/t decrease strength and endurance.

3. Low Self–Esteem r/t disruption in how client perceive 3. Low Self–Esteem r/t disruption in how client perceive one’s own body. one’s own body.

4. Ineffective airway clearance r/t neuromuscular 4. Ineffective airway clearance r/t neuromuscular dysfunction dysfunction

5. Bathing/hygiene, feeding, toileting self-care 5. Bathing/hygiene, feeding, toileting self-care deficit related to decrease energy production. deficit related to decrease energy production.

6. Fear related to sudden onset of illness. 6. Fear related to sudden onset of illness.

7. Impaired spontaneous ventilation r/t denervation 7. Impaired spontaneous ventilation r/t denervation of intercostal muscles. of intercostal muscles.

Cont..Nursing DiagnosisCont..Nursing Diagnosis

Diagnosis Diagnosis Diagnosis is made by recognizing the Diagnosis is made by recognizing the

pattern of rapidly evolving paralysis pattern of rapidly evolving paralysis with areflexia.with areflexia.

Absence of fever or other systemic Absence of fever or other systemic symptoms and characteristics of symptoms and characteristics of antecedent events. antecedent events.

Diagnostics Diagnostics

In lumbar puncture “LP” CSF is withdrawn through a In lumbar puncture “LP” CSF is withdrawn through a needle inserted into the subarachnoid space of needle inserted into the subarachnoid space of the spinal canal between the L3-L4 or L4-L5 the spinal canal between the L3-L4 or L4-L5 lumbar vertebrae.lumbar vertebrae.

Measure CSF pressure Measure CSF pressure determine viral or bacterial origin determine viral or bacterial origin Increase in WBC countIncrease in WBC count presence of cytokines (presence of cytokines (IL 6, TNF IL 6, TNF αα, interferon) , interferon) Cx: inc. ICP Cx: inc. ICP →→ rapid decrease in pressure within rapid decrease in pressure within

CSF around spinal cordCSF around spinal cord→→ brain herniation brain herniation

Lumbar PunctureLumbar Puncture

- Needle electrodes inserted into the muscle .Needle electrodes inserted into the muscle .- Pattern of electrical activity in the muscle both at Pattern of electrical activity in the muscle both at

rest and during activity may be recorded.rest and during activity may be recorded.- Relaxed muscles are normally electrically silent Relaxed muscles are normally electrically silent

except in motor end plates. except in motor end plates. - Abnormal spontaneous activity with denervation Abnormal spontaneous activity with denervation

or inflammatory changes in the affected muscle. or inflammatory changes in the affected muscle. - Fibrillation potentials and positive sharp waves – Fibrillation potentials and positive sharp waves –

reflect muscle irritability. reflect muscle irritability.

ElectromyographyElectromyography

IgM and IgG are highest in the early IgM and IgG are highest in the early course of the disease. course of the disease.

Serum Antibody titerSerum Antibody titer

Nerve conduction studiesNerve conduction studies (Sensory) (Sensory) determining the determining the

conduction velocity and conduction velocity and amplitude of APs where these amplitude of APs where these fibers are stimulated at one point.fibers are stimulated at one point.

Helpful in determining whether Helpful in determining whether sensory symptoms arising from sensory symptoms arising from pathology are proximal or distal pathology are proximal or distal to the root of ganglia to the root of ganglia

Diagnostic TestsDiagnostic Tests

Normal conduction: - Arms: 50-70 m/s - Legs: 40-60 m/s

TreatmentTreatment

There is no cure for Guillain-Barré Syndrome, but there There is no cure for Guillain-Barré Syndrome, but there are treatments available…are treatments available…

PlasmapharesisPlasmapharesis Immunoglobulins Immunoglobulins

Question?Question?

referencesreferences

(1) Guillain-Barre Syndrome. (1) Guillain-Barre Syndrome. Davids, Dr. Heather. University of Colorado Davids, Dr. Heather. University of Colorado School of Medicine. 2006. Viewed at: School of Medicine. 2006. Viewed at: http://www.emedicine.com/pmr/topic48.htmhttp://www.emedicine.com/pmr/topic48.htm

"Guillain-Barré Syndrome Fact Sheet.""Guillain-Barré Syndrome Fact Sheet." NINDS. NIH Publication No. 05-2902. NINDS. NIH Publication No. 05-2902. Viewed at http://www.ninds.nih.gov/disorders/gbs/detail_gbs.htmViewed at http://www.ninds.nih.gov/disorders/gbs/detail_gbs.htm

Van Doorn, P. A., (March 2003). Gullain-Barré syndrome. Retrieved September Van Doorn, P. A., (March 2003). Gullain-Barré syndrome. Retrieved September 2, 2008 from http://www.orpha.net/data/patho/GB/uk-Guillain.pdf2, 2008 from http://www.orpha.net/data/patho/GB/uk-Guillain.pdf

Thomas. C. L. (18). (1997). Thomas. C. L. (18). (1997). Taber’s Encyclopedic Medical DictionaryTaber’s Encyclopedic Medical Dictionary. . Philadelphia: F.A. Davis Company.Philadelphia: F.A. Davis Company.

Http://www.healthscout.com/ency/68/653/main.htmlHttp://www.healthscout.com/ency/68/653/main.html http://www.ninds.nih.gov/disorders/gbs/gbs.htmhttp://www.ninds.nih.gov/disorders/gbs/gbs.htm http://www.neurologychannel.com/guillain/treatment.shtmlhttp://www.neurologychannel.com/guillain/treatment.shtml http://www.emedicine.com/pkm/topic48.htm#section~treatmenthttp://www.emedicine.com/pkm/topic48.htm#section~treatment