Guillain-Barre syndrome

Definition:
Guillain-Barre syndrome is an uncommon disorder in which your body’s immune system attacks your nerves. Weakness and numbness in your extremities are usually the first symptoms. These sensations can quickly spread, eventually paralyzing your whole body.

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The exact cause of Guillain-Barre syndrome is unknown, but it is often preceded by an infectious illness such as a respiratory infection or the stomach flu. Luckily, Guillain-Barre syndrome is relatively rare, affecting only 1 or 2 people per 100,000.

In its most severe form, Guillain-Barre syndrome is a medical emergency requiring hospitalization. There’s no known cure for Guillain-Barre syndrome, but several treatments can ease symptoms and reduce the duration of the illness.

GBS can cause symptoms that last for a few weeks. Most people recover fully from GBS, but some people have permanent nerve damage. In very rare cases, people have died of GBS, usually from difficulty breathing. In the United States, for example, an estimated 3,000 to 6,000 people develop GBS each year on average, whether or not they received a vaccination.

 

Guillain-Barre affects about 1,500 people every year in the UK, and about 150 develop CIDP. The exact mechanisms that cause the conditions aren’t clear, but about 60 per cent of those affected will have had a throat or intestinal infection, flu or major stress within the previous two weeks. This triggers the immune system, which then attacks the nerves.

It rarely occurs in first-degree relatives, but familial cases have been reported and genetic similarities noted. For example, a study of Japanese people with Guillain-Barre following an intestinal infection with the bacteria Campylobacter jejuni found they were more likely to have a rare version of the gene for an immune system chemical known as tumour necrosis factor.

Symptoms:
Guillain-Barre syndrome often begins with weakness, tingling or loss of sensation starting in your feet and legs and spreading to your upper body and arms. These symptoms may begin — often not causing much notice — in your fingers and toes. In some people, symptoms begin in the arms or even the face. As the disorder progresses, muscle weakness can evolve into paralysis.

Signs and symptoms of Guillain-Barre syndrome may include:

*Prickling, “pins and needles” sensations in your fingers, toes or both
*Weakness or tingling sensations in your legs that spread to your upper body
*Unsteady walking or inability to walk
*Difficulty with eye movement, facial movement, speaking, chewing or swallowing
*Severe pain in your lower back
*Difficulty with bladder control or intestinal functions
*Very slow heart rate or low blood pressure
*Difficulty breathing
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Most people with Guillain-Barre syndrome experience their most significant weakness within three weeks after symptoms begin. In some cases, signs and symptoms may progress very rapidly with complete paralysis of legs, arms and breathing muscles over the course of a few hours.

The disorder was first described by the French physician Jean Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.

GBS is also known as acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French polio, Landry’s ascending paralysis and Landry Guillain Barré syndrome.

Canadian neurologist C. Miller Fisher described the variant that bears his name in 1956

Causes:
Many things can cause GBS; about two-thirds of people who develop GBS symptoms do so several days or weeks after they have been sick with diarrhea or a respiratory illness. Infection with the bacterium Campylobacter jejuni is one of the most common risk factors for GBS. People also can develop GBS after having the flu or other infections (such as cytomegalovirus and Epstein Barr virus). On very rare occasions, they may develop GBS in the days or weeks after getting a vaccination.

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Typically, Guillain-Barre develops as an autoimmune reaction following an acute infection. It’s not inherited, although it’s thought that genetic factors may make some people more likely to develop autoimmune conditions.

Risk Factors:
Anyone can develop GBS; however, it is more common among older adults. The incidence of GBS increases with age, and people older than 50 years are at greatest risk for developing GBS.

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Guillain-Barre may be triggered by:

*Most commonly, infection with campylobacter, a type of bacteria often found in undercooked food, especially poultry
*Surgery
*Epstein-Barr virus
*Hodgkin’s disease
*Mononucleosis
*HIV, the virus that causes AIDS
*Rarely, rabies or influenza immunizations

Diagnosis:
The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis (through a lumbar spinal puncture) and electrodiagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.

*cerebrospinal fluid:
Typical CSF findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (100–1000 mg/dL), without an accompanying increased cell count pleocytosis. A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.

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*Electrodiagnostics
Electromyography (EMG) and nerve conduction study (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.

Diagnostic criteria Required:

*Progressive, relatively symmetrical weakness of two or more limbs due to neuropathy
*Areflexia
*Disorder course < 4 weeks
*Exclusion of other causes (see below)

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Supportive:
*relatively symmetric weakness accompanied by numbness and/or tingling
*mild sensory involvement
*facial nerve or other cranial nerve involvement
*absence of fever
*typical CSF findings obtained from lumbar puncture
*electrophysiologic evidence of demyelination from electromyogram

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Differential diagnosis:
*acute myelopathies with chronic back pain and sphincter dysfunction
*botulism with early loss of pupillary reactivity and descending paralysis
*diphtheria with early oropharyngeal dysfunction
*Lyme disease polyradiculitis and other tick-borne paralyses
*porphyria with abdominal pain, seizures, psychosis
*vasculitis neuropathy
*poliomyelitis with fever and meningeal signs
*CMV polyradiculitis in immunocompromised patients
*critical illness neuropathy
*myasthenia gravis
*poisonings with organophosphate, poison hemlock, thallium, or arsenic
*intoxication with Karwinskia humboldtiana leaves or seeds
*paresis caused by West Nile virus
*spinal astrocytoma
*motor neurone disease
*West Nile virus can cause severe, potentially fatal neurological illnesses, which include encephalitis, meningitis, Guillain-Barré syndrome, and anterior myelitis.

Treatment :
Supportive care with monitoring of all vital functions is the cornerstone of successful management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm. Early intubation should be considered in any patient with a vital capacity (VC) <20 ml/kg, a negative inspiratory force (NIF) that is less negative (i.e., closer to zero) than -25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disorder, or autonomic instability.

Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible. Either high-dose intravenous immunoglobulins (IVIg) at 400 mg/kg for 5 days or plasmapheresis can be administered, as they are equally effective and a combination of the two is not significantly better than either alone. Therapy is no longer effective two weeks after the first motor symptoms appear, so treatment should be instituted as soon as possible. IVIg is usually used first because of its ease of administration and safety profile, with a total of five daily infusions for a total dose of 2 g/kg body weight (400 mg/kg each day). The use of intravenous immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases, renal failure if used for longer than five days. Glucocorticoids have not been found to be effective in GBS. If plasmapheresis is chosen, a dose of 40-50 mL/kg plasma exchange (PE) can be administered four times over a week.

Following the acute phase, the patient may also need rehabilitation to regain lost functions. This treatment will focus on improving ADL (activities of daily living) functions such as brushing teeth, washing, and getting dressed. Depending on the local structuring on health care, a team of different therapists and nurses will be established according to patient needs. An occupational therapist can offer equipment (such as wheelchair and special cutlery) to help the patient achieve ADL independence. A physiotherapist would plan a progressive training program and guide the patient to correct functional movement, avoiding harmful compensations which might have a negative effect in the long run. There is also some evidence supporting physiotherapy in helping patients with Guillain–Barré syndrome regain strength, endurance, and gait quality,[23] as well as helping them prevent contractures, bedsores, and cardiopulmonary difficulties. A speech and language therapist would be essential in the patient regaining speaking and swallowing ability if they were intubated and received a tracheostomy. The speech and language therapist would also offer advice to the medical team regarding the swallowing abilities of the patient and would help the patient regain their communication ability pre-dysarthria. There would also be a doctor, nurse and other team members involved, depending on the needs of the patient. This team contribute their knowledge to guide the patient towards his or her goals, and it is important that all goals set by the separate team members are relevant for the patient’s own priorities. After rehabilitation the patient should be able to function in his or her own home and attend necessary training as needed.

Prognosis:
Most of the time recovery starts after the fourth week from the onset of the disorder. Approximately 80% of patients have a complete recovery within a few months to a year, although minor findings may persist, such as areflexia. About 5–10% recover with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration. However, this is a grave disorder and despite all improvements in treatment and supportive care, the death rate among patients with this disorder is still about 2–3% even in the best intensive care units. Worldwide, the death rate runs slightly higher (4%), mostly from a lack of availability of life support equipment during the lengthy plateau lasting four to six weeks, and in some cases up to one year, when a ventilator is needed in the worst cases. About 5–10% of patients have one or more late relapses, in which case they are then classified as having chronic inflammatory demyelinating polyneuropathy (CIDP).

Poor prognostic factors include: 1) age, over 40 years, 2) history of preceding diarrheal illness, 3) requiring ventilator support, 4) high anti-GM1 titre and 5) poor upper limb muscle strength

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:

http://www.bbc.co.uk/health/physical_health/conditions/guillainbarre1.shtml

http://www.riversideonline.com/health_reference/Nervous-System/DS00413.cfm

http://en.wikipedia.org/wiki/Guillain%E2%80%93Barr%C3%A9_syndrome

http://www.cdc.gov/flu/protect/vaccine/guillainbarre.htm

http://nursingcrib.com/nursing-notes-reviewer/guillain-barre-syndrome/

http://nursingcomments.com/tag/guillain-barre-syndrome/

http://www.ami20.com/tag/guillain-barre

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