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Journey To Healthby Andy GriffithManteo, North Carolina
“Cindi had had frequent and major attacks of sore throat all of her life. So, shortly after our marriage we returned to Los Angeles and went to see a throat specialist, Dr. Robert Feder. He determined her sore throats were coming from her tonsils, and scheduled an operation. Early the next morning we drove over to Cedars-Sinai Medical Center and Dr. Feder took out her tonsils. While she was recuperating, I got a bad case of the flu. Not exactly a honeymoon of the rich and famous.
My illness was strange. As I got better, the symptoms of influenza were replaced by pain--terrible, searing pain that ricocheted through my entire body. Cindi and I joked about our invalid status and settled in that Saturday to watch the Kentucky Derby on television.
But after the race, when I stood up and took a few steps, I pitched headlong into a nightmare. I was overcome by pain so encompassing that I couldn't feel my feet. I had no control over them, and fell to the floor in agony.
We couldn't reach any of our doctors that weekend. Yet I was so desperate for relief from the pain that I took some of the codeine prescribed for Cindi for her throat. It barely made a difference.
On Monday my doctor met us at a local hospital. There a roomful of doctors attempted to find out what was wrong. For four days they hadn't a clue. Finally they did a spinal tap. When the results came in, one mystery was solved. I had Guillain-Barre syndrome, a rare form of nerve inflammation. It's thought to be caused by an allergic reaction to a viral infection, such as the flu. The nerves become inflamed and begin to send erroneous and scrambled messages to the brain. In some people it causes little pain but extensive paralysis; in cases such as mine, it causes little paralysis but intense pain.
There are no drugs or surgery to treat Guillain-Barre--so the doctor sent me home. "There is nothing we can do," he said. "You've got to ride it out. I'll prescribe some pain medication, but use as little as possible. Come back in a week."
Introduction
Guillain-Barré syndrome (GBS) is an acute, frequently severe, and fulminant polyradiculoneuropathy that is autoimmune in nature.
collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes.
1 to 2 per 100,000 population.
Males are at 1.5-fold higher risk for GBS than females,
in western countries adults are more frequently affected than children.
With poliomyelitis under control in developed countries, GBS is now the most important cause of acute flaccid paralysis
History
The French physician Jean Landry first described the disorder in 1859.
In 1916, Georges Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and described the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.
Epidemiology
Worldwide, the annual incidence is about 0.6–4 occurrences per 100,000 people.
Men are one and a half times more likely to be affected than women.
The incidence increases with age; there are approximately 1 cases per 100,000 people aged below 30 years and about 4 cases per 100,000 in those older than 75 years.
The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population.
Congenital and neonatal Guillain–Barré syndrome have also been reported
GBS is also known as
acute idiopathic polyradiculoneuritis,
acute idiopathic polyneuritis,
French polio,
Landry's ascending paralysis and
Landry Guillain Barré syndrome.
Related anatomy and physiology
PathophysiologyGuillain-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.
Research InputCampylobacter jejuni infection in Guillain-Barré syndrome: A prospective case control study in a
tertiary care hospital
A Sharma1, V Lal1, M Modi1, C Vaishnavi2, S Prabhakar1
Background: This study was carried out to determine the relationship between C. jejuni infection and GBS in an Indian setting.
Materials and Methods: This prospective study was carried out on a cohort of 50 patients with GBS who were treated in a tertiary care hospital in India. Based on electrophysiological findings the patients were divided into various subtypes. Serology for C. jejuni (Immunoglogulin G, IgG and Immunoglogulin, IgM) using ELISA was done both in patients and 40 age, sex and geographically matched controls.
Conclusions
Preceding C. jejuni infection is common among GBS patients and is often associated with the axonal variety of GBS. Axonal variety of GBS generally presents in a younger age group as compared to AIDP.
SIGNS & SYMPTOMS
Areflexic motor paralysis with or without sensory disturbance.
Bulbar weakness
Pain
Deep tendon reflexes attenuate or disappear
Cutaneous sensory deficits
Bladder dysfunction
Subtypes
• 1.Acute inflammatory demyelinating polyradiculoneuropathyAIDP
• 2.Acute motor axonal neuropathyAMAN• 3.Acute motor and sensory axonal
neuropathyAMSAN
MFS 4.Miller fisher syndrome
1.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
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.
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).
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.
INVESTIGATIONS
Serum biochemistry:evidence of SIADH or renal dysfunction
Inflammatory markers :ESR is usually raised and CRP is sometimes
INVESTIGATIONS
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
INVESTIGATIONS
Infection screen :
Campylobacter jejuni, Cytomegalovirus,
Epstein-Barr virus, Herpes simplex virus,
Mycoplasma pneumoniae. HIV antibodies
INVESTIGATIONS
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 exclude cervical nerve impingement
INVESTIGATIONS
INVESTIGATIONS
Lumbar puncture: Cell count and glucose are usually normal with a raised protein, although the latter may also be normal in first two weeks.
INVESTIGATIONS
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.
INVESTIGATIONS
Respiratory function tests:– These may show reduced vital capacity, maximal
inspiratory and expiratory pressures. – Arterial blood gases may indicate progressive
respiratory failure.
DIFFERENTIAL DIAGNOSIS
Neurological–Myasthenia gravis– Eaton-Lambert (myasthenic) syndrome– Multiple sclerosis– Transverse myelitis
DIFFERENTIAL DIAGNOSIS
Metabolic– Hypokalaemic periodic paralysis– Hypermagnesaemia– Hypophosphataemia– Acute intermittent porphyria
DIFFERENTIAL DIAGNOSIS
Infective– Post diphtheria neuropathy– Polio– Botulism– Tick paralysis
DIFFERENTIAL DIAGNOSIS
Drugs / toxins– Heavy metal poisoning (e.g. lead)– Biological toxins (including snake and scorpion
toxins)– Drugs (including stavudine, nitrofurantoin and
aminoglycosides)
DIFFERENTIAL DIAGNOSIS
Other– Acute polymyositis– Critical illness myopathy
MANAGEMENT
SupportiveImmunomodulato
ry
Supportive
Airway and respiratory
Cardiovascular
Gastrointestinal
Neurological
Psychological
Rehabilitation
Immunomodulatory
Intravenous immunoglobulin– most effective if administered within two weeks of
the onset of symptoms– Indications for IVIg include • muscle weakness and • respiratory depression
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.
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
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
PROGNOSIS
Most patients with GBS recover fully but this may take many months of intensive therapy.
15% of patients suffer persistent disability.
10% are unable to walk unaided at one year.
There may be a recurrence in 2–5% of cases.
The mortality from GBS ranges from 2–12%.
Prognosis
Common causes of death include venous thromboembolism, pneumonias, arrhythmias and complications related to dysautonomia.
Markers of poor prognosis include – age > 40 years, – rapid onset of symptoms, – severe weakness(especially if mechanically ventilation is
required or there is marked upper limb weakness),– association with precedent diarrhoeal illness or
campylobacter infection, – evidence of axonal damage on electrophysiological studies
and– lack of treatment with either plasma exchange or IVIg.
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.
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
Maintaining respiratory function
incentive spirometry and chest physiotherapy
Mechanical ventilation
Close monitoring
Suctioning
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
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
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
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.