guillain barre syndrome

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.