GB Syndrome

Introduction Guillain-Barre syndrome is a rare but serious autoimmune

disorder in which the immune system attacks healthy nerve cells of the
peripheral nervous system. It is an acute inflammatory demyelinating
polyneuropathy that is the most common cause of acute or subacute
generalized paralysis. GBS is considered to be a postinfectious immune-
mediated disease targeting peripheral nerves.
 Definition of Guillain-Barre syndrome
• Guillain-Barre syndrome (GBS) is an acute autoimmune disease
marked by inflammation of the peripheral nerves, affecting arms and
legs and Involves destruction of the myelin sheath surrounding largest,
most myelinated sensory and motor fibers, resulting in disrupted
proprioception and weakness.
• Guillain–Barré syndrome (GBS) 0r Landry's paralysis It is a disorder in
which the body’s immune system attack part of the peripheral nervous
system. It is an acute, progressive, autoimmune, inflammatory
demyelination of polyneuropathy 0f the peripheral sensory and motor
nerves and nerve roots.
 Epidemiology
• 1-2 (1.9) per 100,000 persons . most commonly it affects young and
middle- aged adults 30 to 50 years of age. Females slightly more
affected than males. More common in devloping countries. Germany
(7.9%), Mexico (44%), India (28%).
 Etiology
• The precise cause of Guillain-Barre is unknown. Sixty percent of cases
have followed a lung infection or a gastrointestinal infection . The
following infections have been associated with Guillain-Barre:
Campylobacter jejuni infection. Influenza (the flu) Cytomegalovirus (a
strain of the herpes virus) Epstein-Barr virus infection (mononucleosis)
Mycoplasma pneumonia HIV
• Triggers of GBS Surgery Trauma Pregnancy Hodgkin’s disease HIV
• athophysiologyInfectious organism contains an amino acid – mimics peripheral nerve myelin
protein. Cell mediated immune attack on peripheral nerve myelin protein Immune system
cannot distinguish between the 2 proteins Attack and destroys peripheral nerve myelin
Inflammation and destruction of myelin sheath Axon is unable to support
• 10. Pathophysiology… Specific immune response directed against PNS antigen is initiated Auto
antigen T cells circulate & enter the PNS Auto antigen is recognised by T cells Activation of
local macrophages& B cells to secrete auto antibodies
• 11. Cont.. Blood nerve barrier breaks down, leading to entrance of specific auto antibodies
into nerve which cross react with myelin Multifocal stripping of myelin Defects in propagation
of electrical nerve impulses Conduction block &flaccid paralysis
• 12. Disease Progression 80% experience complete recovery Recovery may last from 2 months
to 2 years 3 distinct phases: Acute (4 wks) - initial rapid onset of symptoms Plateu (few days
to few weeks) - symptoms neither worsen nor improve Recovery - gradual improvement
• athophysiology Guillain-Barré is the result of a cell-mediated immune
attack on peripheral nerve myelin proteins. The best-accepted theory
is that an infectious organism contains an amino acid that mimics the
peripheral nerve myelin protein. The immune system cannot
distinguish between the two proteins and attacks and destroys
peripheral nerve myelin. the ganglioside GM1b, is the most likely
target of the immune attack. With the autoimmune attack there is an
influx of macrophages and other immune-mediated agents that attack
myelin, cause inflammation and destruction, and leave the axon
unable to support nerve conduction.
 Types
• Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common
form and accounts for around 85– 90% of cases. The clinical features are of symmetrical
ascending motor weakness with hypo- or areflexia. The underlying pathological process
involves inflammation and destruction of the myelin sheaths surrounding peripheral
nerve axons by activated macrophages. This leads to slowing and blockage of conduction
within peripheral nerves causing muscle weakness. Severe cases may develop secondary
axonal damage. Auto immune response against schwann cell.

• Acute motor axonal neuropathy (AMAN) is more common in Japan and China, amongst
young people and in the summer months. It has an association with precedent infection
with Campylobacter jejuni. Clinical features are similar to AIDP but tendon reflexes may
be preserved. Electrophysiological testing may distinguish from other variants as selective
motor nerve and axonal involvement is demonstrated. In AMAN the pathological process
involves binding of antibodies to ganglioside antigens on the axon cell membrane,
macrophage invasion, inflammation and axonal damage.Also known as Chinese paralytic
syndrome, • Attacks motor nodes of Ranvier and is prevalent in China and Mexico.
Contd.,
• Acute motor and sensory axonal neuropathy (AMSAN) is a variant of GBS in
which both motor and sensory fibres are involved and which can be demonstrated
on electrophysiological studies. It is more severe and associated with prolonged or
even partial recovery. Clinical features are similar to AMAN but also involve
sensory symptoms. The underlying pathological process is similar to that for
AMAN (i.e. antibody mediated axonal damage) • Affect the sensory with several
axonal damage.
• Miller Fisher syndrome (MFS) presents with ataxia, areflexia and
ophthalmoplegia. 25% of patients may develop limb weakness.
Electrophysiological studies show primarily sensory conduction failure.
Antiganglioside antibodies to GQ1b are found in 90% of patients and are
associated with ophthalmoplegia . There have been limited pathological studies in
MFS but demyelination of nerve roots has been demonstrated. • Rare variant •
Manifest as a descending paralysis. • Usually affects the eye muscles first and
presents with the triad of ophthalmoplegia, ataxia, and areflexia.
contd.,
• Initial nonspecific symptoms of lethargy, fatigue, headache, and
decreased initiative are followed by autonomic symptoms including
orthostatic lightheadedness, blurring of vision, abdominal pain,
diarrhea, dryness of eyes, and disturbed micturition.
• Bickerstaff's brainstem encephalitis (BBE) • It is characterized by
acute onset of ophthalmoplegia, ataxia, disturbance of consciousness,
hyperreflexia. • Large, irregular hyper intense lesions located mainly in
the brainstem, especially in the pons, midbrain and medulla, are
described in the literature.
contd.,
• Acute Panautonomic Neuropathy • Is the most rare variant of GBS,
sometimes accompanied by encephalopathy. • Frequently occurring
symptoms include impaired sweating, lack of tear formation,
photophobia, dryness of nasal and oral mucosa, itching and peeling of
skin, nausea, dysphagia, and constipation unrelieved by laxatives or
alternating with diarrhea.
contd.,
• Lumbar puncture: Cell count and glucose are usually normal with a
raised protein, although the latter may also be normal in first two
weeks.
• Nerve conduction studies: – Findings depend on subtype of GBS. –
The majority show demyelinating pattern while – some patients may
show evidence of axonal loss with little or no demyelination.
• Respiratory function tests: – These may show reduced vital capacity,
maximal inspiratory and expiratory pressures. – Arterial blood gases
may indicate progressive respiratory failure.
1) Pain.
Clinical features
2) Progressive muscle weakness.
3) Diminished reflexes of lower extremities.
4) Prickly, tingling sensations.
5) Low blood pressure.
6) Paresthesis.
7) Tenderness and muscle pain.
8) Dysthesias & muscle spasms.
9) Palpitation.
10)Hearth rate change.
11)Sweating abnormalities.
12)Cardiac arrhythmia.
13)Neuromuscular respiratory failure.
14)Difficulty in eye movement, facial movement, speaking, chewing, or swallowing.
 Clinical features
Initially Pain in the muscle Weakness of muscle The onset is gradual and
progresses over days or weeks. By the 3rd week 90% of the patient are
weak. Usually begins in the lower extremities and progressively involves
the trunk, the upper limbs, and finally the bulbar muscles. This pattern is
known as Landry ascending paralysis. Relatively symmetrically, but
asymmetry is found in 9% of patients Paresthesias occur in some cases.
Respiratory insufficiency due to Intercostal and diaphragmatic muscle
paralysis Dysphagia and facial weakness Papilledema oculomotor and
other cranial neuropathies The autonomic nervous system involvement:
lability of blood pressure and cardiac rate, postural hypotension, episodes
of profound bradycardia occasional asystole
 Diagnosis

• Physical Assessment, Vital signs (tachycardias, bradycardias, Tachypnea, Blood

pressure lability, bladder retention, paralytic ileus) Cranial nerves: Facial
weakness, facial droop, dysphagia, dysarthria,Ptosis from cranial nerve III
(oculomotor) palsy often is associated with limited eye movements. Reflexes are
absent or hyporeflexic, Pathologic reflexes, such as Babinski, are
absent.Hypotonia can be observed with significant weakness
• Lab Studies CSF studies: During the acute phase of GBS ,an elevation in CSF
protein (>0.55 g/L) without an elevation of white blood cells (<10
lymphocytes/mL). Complete blood counts Serologic studies ( increase in titre for
infectious agent)
• Electromyography (EMG) studies Abnormalities in the NCS consistent with
demyelination are sensitive and represent specific findings for classic GBS. MRI
Pulmonary function tests Histologic Findings: Lymphocyte and macrophage
infiltration is observed on microscopic examination of peripheral nerves.
 contd.,
• Serum biochemistry:evidence of SIADH or renal dysfunction
Inflammatory markers :ESR is usually raised and CRP is sometimes
• Antiganglioside antibodies : Anti-GM1 • It is positive in 25% of pts
and is a w worse outcome Anti-GD1a • AMAN subtype of GBS Anti-
GQ1b • Miller- Fisher syndrome
• Infection screen : Campylobacter jejuni, Cytomegalovirus, Epstein-
Barr virus, Herpes simplex virus, Mycoplasma pneumoniae. HIV
antibodies
• Radiological: A CT brain is indicated to exclude other causes of
symptoms and evidence of raised intracranial pressure prior to
performing a lumbar puncture. An MRI of the spine may show selective
anterior spinal nerve root enhancement with gadolinium and will
• GNOSTIC EVALUATION • Medical history- • Physical examination- • Tests -
• 15. Nerve conduction studies (NCS)
• 16. CSF examination
• 17. Electromyography (EMG)
• 18. Electrocardiogram (ECG)
• 19. Pulmonary Function Test (PFT)
• 20. Criteria- • Progressive weakness in leg & arm & areflexia. Features that
strongly support diagnosis are progressive of symptoms over days to 4
week, relatively symmetry symptoms mild sensory symptoms /signs, cranial
nerve involvement.
• ANAGEMENT Supportive Immunomodulatory
• 32. Supportive Airway and respiratory Cardiovascular Gastrointestinal Neurological Psychological
Rehabilitation
• 33. Immunomodulatory Intravenous immunoglobulin – most effective if administered within two
weeks of the onset of symptoms – Indications for IVIg include • muscle weakness and •
respiratory depression
• 34. IVIG IVIg contains pooled donor IgG antibodies and may reduce the severity of autoimmune
inflammation in GBS by blocking Fc receptors. This prevents the Fc portion of antibodies binding
and thus interrupts antibody mediated cell destruction. Complement activation is also altered.
• 35. Plasma exchange The aim of plasma exchange is to remove antibodies associated with the
underlying autoimmune response. passage of blood through an extracorporeal cell separator.
The plasma fraction of the blood is removed then and replaced with FFP or human albumin
solution. Anticoagulants are administered during the procedure.
• Recommendations for the treatment of GBS: 1)plasma exchange (PE) is recommended for nonambulant
adult patients with GBS who seek treatment within 4 weeks of the onset of neuropathic symptoms.PE
should also be considered for ambulant patients examined within 2 weeks of the onset of neuropathic
symptoms. 2) IV IG is recommended for nonambulant adult patients with GBS within 2 or possibly 4
weeks of the onset of neuropathic symptoms; 3) Corticosteroids are not recommended for the
management of GBS. 4) sequential treatment with PE followed by IV IG or immunoabsorption followed
IV IG is not recommended for patients with GBS ;and 5) PE and IV IG are treatment options for children
with severe GBS
• 37. Recommendations For Rx 1)plasma exchange (PE) is recommended for nonambulant adult patients
with GBS who seek treatment within 4 weeks of the onset of neuropathic symptoms.PE should also be
considered for ambulant patients examined within 2 weeks of the onset of neuropathic symptoms. 2) IV
IG is recommended for nonambulant adult patients with GBS within 2 or possibly 4 weeks of the onset
of neuropathic symptoms; 3) Corticosteroids are not recommended for the management of GBS. 4)
sequential treatment with PE followed by IV IG or immunoabsorption followed IV IG is not
recommended for patients with GBS ;and 5) PE and IV IG are treatment options for children with severe
GBS
• Treatment Supporti ve care Ventilato r support Autonomic dysfunctio
n manageme nt Nutrition al support Immune therapy
• 19. Management… Mechanical ventilation Trachesotomy Sustained
hypertension (ACE inhibitors, Beta blockers) Postural hypotension
Urinary difficulties Ventilator support Autonomic dysfunction
• 20. Naso gastric tube Hyperalimentation Plasma exchange Intravenous
immunoglobulin Nutritional support Immune therapy Management…
• 21. POTENTIAL COMPLICATIONS Breathing difficulties Heart and blood
pressure problems Residual numbness or other sensations Pain Bowel
and bladder function problems Blood clots Pressure sores
• Nursing management Nursing Assessment Assess pain level due to
muscle spasms and dysthesias. Assess cardiac function including
orthostatic Blood Pressure. Assess respiratory status closely to
determine hypoventilation due to weakness. Perform cranial nerve
assessment, especially ninth cranial nerve for gag reflex. Assess motor
strength.
• Nursing Management Assessment: Acute onset (hours to weeks) of progressive, usually ascending muscle weakness
and fasciculation, possibly leading to paralysis (maximal weakness is reached within 2 weeks). Paresthesia and
painful sensations. Possible hypoventilation due to chest muscle weakness. Difficulty with swallowing, chewing,
speech, and gag, indicating fifth (trigeminal) and ninth (glossopharyngeal) cranial nerve movement. Reduce or
absent deep tendon reflexes, position and vibratory perception.
• 41. Assessment: Autonomic dysfunction with orthostatic hypotension and tachycardia. Check for previous history of
viral illness or surgical procedure Urinary incontinence Check for facial nerve paralysis. Inspect the patient’s face at
rest and during conversation.Assess for any problems during talking,swallowing,and chewing. Assess for any change
in the vital signs
• 42. Maintaining respiratory function incentive spirometry and chest physiotherapy Mechanical ventilation Close
monitoring Suctioning
• 43. Enhancing physical mobility paralyzed extremities are supported in functional positions, and passive range-of-
motion exercises are performed at least twice daily Range-of-motion exercises, altering positioning, anticoagulation,
thigh-high elastic compression stockings or sequential compression boots, and adequate hydration
• 44. Providing adequate nutrition IV fluids and parenteral nutrition monitors for the return of bowel sounds
gastrostomy tube assesses the return of the gag reflex and bowel sounds before resuming oral nutrition.
• Improving communication picture cards or an eye blink system Collaboration with the
speech therapist
• 46. Decreasing fear and anxiety participate in physical care providing information
about the condition, emphasizing a positive appraisal of coping resources, teaching
relaxation exercises and distraction techniques Encouraging visitors, engaging visitors
or volunteers to read to the patient, listening to music or books on tape, and watching
television
• 47. Monitoring and managing potential complications Thorough assessment of
respiratory function at regular intervals cardiac dysrhythmias, which necessitate ECG
monitoring, transient hypertension, orthostatic hypotension, DVT, pulmonary
embolism, urinary retention, and other threats to any immobilized and paralyzed
patient. These require monitoring and attention to prevent them and prompt
treatment if indicated.
• MEDICAL TREATMENT • Plasmaphresis • High dose immunoglobin therapy •
Mechanical ventilator • Pain control • Supportive care
• 22. Prognosis • Start after the 4th week from the onset. • Approximately
80% of patient have a complete recovery.
• 23. NURSING MANAGEMENT • Treatment is non specific and symptomatic. •
Observe continuously for adequacy of respiratory effort. • Continuous ECG
monitoring. • Supportive nursing care measures indicated by the patients
degree of paralysis. • Residual efforts are rare, but prolonged flaccid
paralysis lead to muscle atrophy requiring rehabilitation and physiotherapy.
• 24. THANK YOU
• Nursing Diagnosis

• Ineffective Breathing Pattern

• May be related to

• Ascending paralysis
• Decrease lung
• Possibly evidenced by

• Altered chest expansion

• Cyanosis
• Respiratory depth changes
• Abnormal ABGs
• ursing Diagnosis Ineffective Breathing Pattern related to weakness/paralysis of respiratory
muscles Impaired Physical Mobility related to paralysis Imbalanced Nutrition: Less Than Body
Requirements, related to cranial nerve dysfunction
• 24. Cont….. Impaired Verbal Communication related to intubation, cranial nerve dysfunction
Chronic Pain related to disease pathology Anxiety related to communication difficulties and
deteriorating physical condition
• 25. Patient education Advise patient and family that acute phase lasts 1 to 4 weeks, then patient
stabilizes and rehabilitation can begin; however, convalescence may be lengthy, from 3 months
to 2 years. Instruct patient in breathing exercises or use of incentive spirometer to reestablish
normal patterns. Teach patient to wear good supportive and protective shoes while out of bed to
prevent injuries due to weakness and paresthesia.
• 26. Cont…. Instruct patient to check feet routinely for injuries because trauma may go unnoticed
due to sensory changes. Reinforce maintenance of normal weight; additional weight will further
stress the motor abilities. Encourage the use of scheduled rest periods to avoid over-fatigue.
• Convulsive Status epilepticus-
• The convulsive type is more common and more dangerous.
• It involves tonic- clonic seizures (grand mal seizures)
• In the tonic phase ( lasts less than 1 minute), body becomes stiff and person lose consciousness. Eyes roll
back into head, muscles contract, back arches, and trouble breathing.
• As the clonic phase starts, body spasms and jerks occur. Neck and limbs flex and relax rapidly but slow down
over a few minutes.
• Once the clonic phase ends, patient might stay unconscious for a few more minutes. This is the postictal
period.Non-convulsive Status epilepticus-
• Patient lose consciousness but is in an “epileptic twilight” state.
• There might not able any shaking or seizing at all, so it can be very hard for someone observing patient to
figure out what’s happening.
• A non-convulsive seizure can turn into a convulsive episode.
• Poorly controlled epilepsy
• Low blood sugar
• Stroke
• Kidney failure
• Liver failure
• Encephalitis
• HIV
• Alcohol or drug abuse
• Genetic diseases such as Fragile X syndrome and Angelman syndrome
• Head injuries