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