- GBS at a glance -

GBS - Facts at a glance
What is a 'Syndrome'?
The many names of the syndrome
Variants of GBS
Further reading

GBS - Facts at a glance
Guillain-Barré Syndrome (GBS) is a rare inflammation of the nerves, caused by the patient's body producing antibodies against the peripheral nerves. The syndrome affects each patient differently and so the course of the disease differs for each patient.
How and why one contracts GBS is as yet unknown. The syndrome is typically observed after an infection in the lungs, diarrhoea, surgery, vaccination or childbirth.

The inflammation damages portions of the nerve cell, resulting in muscle weakness, paralysis or sensory disturbances. The damage usually involves loss of the nerve's myelin sheath (demyelination), which slows the conduction of impulses through the nerve.
The damage may also involve destruction of the axon part of the nerve cell (denervation), which blocks conduction through the nerve.

This damages the nerves that affect the patients arms, legs, lungs, throat, heart or eyes. The patient will then experience various symptoms, depending on which nerves have been affected, and to what degree.

Patients generally experience one or more of the following symptoms, caused by nerve damage: Pain, sensory irregularities, progressive weakness leading to paralysis or irregular function of the body's autonomous functions.

The disorder is characterised by progressive symmetrical paralysis and loss of reflexes, usually beginning in the legs. This means that in mild cases, the attack on the nerves may stop, going no further. The patient may experience only moderate difficulty in walking, requiring a walking stick or crutches.
In other cases, the progressing attack gives rise to symptoms of increasing severity.
In extreme cases, weakness may have progressed to the extent that the patient is almost totally paralysed. This may cause difficulty breathing, and the patient needs to be immediately hooked to a ventilator that assists breathing. If the muscles of the heart are affected, the patient will experience irregular heartbeat. He or she needs to be hooked to a cardiac monitor immediately. If the swallowing mechanism is affected, a feeding tube may be required.

There are several forms of treatment that generally reduce the duration of the disorder, but there is as yet no cure. Recovery is spontaneous and therefore impossible to predict. Most patients experience nearly complete or complete recovery, although most have lingering after-effects. The recovery process can be quick in some cases, prolonged in others, and physiotherapy is necessary to regain muscle strength and endurance.
Relapses are rare, but may occur many years later.

This website contains details of the syndrome, as well as a number of real-life stories about how GBS manifests itself, and how it affects the lives of patients and their families.
  Top

What is a 'syndrome'?
A syndrome is a medical condition, characterised by a collection of symptoms (that the patient feels) and signs (that a doctor can observe or measure), rather than by a specific organism that causes the disease.

No one knows what causes GBS. Symptoms and signs can vary a great deal in GBS patients, sometimes making it difficult to diagnose, especially in the early stages.

The terms 'syndrome', 'disease' and 'GBS' are used synonymously in this website, to indicate Guillain-Barré syndrome.
  Top

The many names of the syndrome
The syndrome was named after the French physicians Guillain, Barré and Strohl, who were the first to describe it in 1916. It is sometimes called Landry's paralysis, after the French physician who first described a variant of it in 1859. It is now known by many names. Here are some of the Latin and English names, with a short explanation:

Guillain-Barré Syndrome (GBS), pronounced 'ghee-yan bah-ray'.
Landry-Guillain-Barré syndrome (LGBS).
Guillain-Barré-Strohl syndrome.
Landry's acute ascending paralysis.
Landry's paralysis.
Neuritis.
Polyradiculitis.
Polyradiculitis acutae.
Polyradiculoneuritis.
Infectious polyneuritis.
Acute idiopathic polyneuritis.
Acute inflammatory polyneuropathy.
Acute polyradiculoneuropathy.
Polyneuropathia inflammatorica acuta.
Acquired demyelinating polyradiculoneuropathy.
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP).

The long latin names are packed with meaning: Inflammations end in -itis". As GBS is an inflammation of the peripheral nerves (neuro-), it is often called 'neuritis'.
Many nerves are involved, so the term becomes 'polyneuritis'.
More specifically, the nerve roots (the points of attachment of the peripheral nerves to the spinal cord) are attacked by GBS, which leads to the term radiculo-'.
Diseases are -pathy, making polyradiculoneuropathy an inflammation of many peripheral nerves and nerve roots.

By the way, "idiopathic" signifies that the cause of the disease is unknown. Acute means that it is rapid, while inflammatory means irritating, and finally demyelinating indicates that myelin is destroyed.
  Top

Subgroups of GBS:
GBS is not one disease - the syndrome has several variants differentiated by their symptoms, the preceding infection, the duration of the inflammatory phase, severity, etc. These forms are sometimes called subgroups of the Guillain-Barré syndrome family of nerve disorders. They are less common in children than in adults.

Variants with rapid progressive phase:
- AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy)= GBS

About 80% of the patients get this form.
In GBS most patients experience only one auto-immune attack, at the very beginning. After that they move into the healing phase.

- AMSAN (Acute Motor Sensory Axonal Neuropathy)
A serious axonal form of GBS that attacks motor and sensory nerves. Fulminant course with slow and incomplete recovery.

- AMAN (Acute Motor Axonal Neuropathy)
A particularly severe form, attacks the motor nerves primarily, causing rapid progressive weakness often with respiratory failure.
Pure axonal cases may occur more frequently in other parts of the world outside Europe and North America. AMAN cases also may be different from cases of axonal GBS described in the West.
Many cases have been reported in rural areas of China, especially in children and young adults during the summer months.
Prognosis is often quite favorable and recovery is rapid.

- MFS (Miller-Fisher Syndrome) Also known as Miller Fish syndrome, Miller's syndrome and Acute Disseminated Encephalo-myeloradiculopathy.
A very rare form of GBS that affects about 5% of GBS patients. Unlike GBS, MFS causes descending paralysis, i.e. paralysis that begins in the upper body and gradually spreads downward.
A spinal tap reveals the presence of elevated protein levels. The patient experiences the classical triad of ataxia, opthalmoplegia and areflexia: loss of tendon reflexes and coordination, difficulty walking and standing, vision problems. Also tingling, numbness, dizziness, nausea. Anti-GQ1b antibodies are produced.
The patient experiences blurred or double vision. Damage to cranial nerves weakens the eye-muscles, causing the double-vision. It also weakens the facial muscles, causing facial sagging and sometimes making speech unintelligible. Prescription glasses or contact lenses reduce the vision problems.

Treatment is often a course of corticosteroids.
Recovery takes place in the opposite order, with the cranial nerves recovering last.
Recurrence is exceptional.
See 'Links' for more information.


Variants with slow progressive phase:

- CIDP (Chronic Inflammatory Demyelinating Polyneuropathy)

Also known as chronic GBS, Chronic Idiopathic Polyneuritis, or Chronic Relapsing (Dysimmune) Polyneuropathy.
It is very much less common than GBS, but is the most common chronic form of GBS. It evolves over months or years.
Each case is unique, as in GBS.
CIDP patients normally get multiple attacks, sometimes called relapses. These can be a few weeks apart or a few months apart. Individual relapses may be confused with GBS.
Many of the treatments used for CIDP prevent or reduce the repeated attacks, whichis not the case with GBS.
See 'Links' below for more information.

- MMN (Multifocal Motor Neuropathy) A rare form of CIDP. Limbs weaken gradually in an apparently random pattern.
See 'Links' for more information.

- MMSD (Multifocal Motor Sensory Demyelinating Neuropathy) Also a rare form of CIDP. Causes weakness as well as sensory disturbances.

- MADSAM (Multifocal Acquired Demyelinating Sensory Acquired Neuropathy) Subtype of CIDP. Also known as Lewis-Sumner Syndrome or Multifocal CIDP.
It can be slowly progressive or relapsing-remitting.

- PDN (Paraproteinaemic Demyelinating Neuropathy) Subtype of CIDP.
PDN is known as MGUS (Monoclonal Gammopathy of Unknown Significance) in the USA.
It is slowly progressive in IgM, IgG and IgA but in some cases, the last two can be relapsing-remitting.
See 'Links' for more information.

  Top

Further Reading

- GBS: guide to further information
WebMD
American website with lots of really good information.
Guillain-Barré Syndrome foundation International
Founded to assist victims, through support, education and research. Newsletter. Brochures. Forums.
National Institute of Neurological Disorders & Stroke (NINDS)
Thorough.
NeurologyChannel: Guillain-Barre Syndrome
Comprehensive, concise guide.
Guillain-Barre Syndrome Support Group
UK. Information on GBS subtypes, patient care, etc.
Mayo Clinic - on GBS and Peripheral neuropathy.
MedicineNet.com: Guillain-Barre syndrome
Short but to the point.

- CIDP, MFS, PDN/MGUS and other chronic variants
(Scandinavians: Check the danish section of the website for more info).
Acquired neuropathies ( PDF file)
A chapter from the book "Acquired Neuropathies" which discusses GBS, CIDP, and other acquired neuropathies. CIDP is primarily distinguished from GBS by its clinical history, the length of time it takes to progress to its worst point, and the pattern of repeated attacks.
An overview of CIDP
GBS-Tasmania. Excellent.
CIDP
Mayo clinic, USA.
CIDP
GBS-Australia. Short guide for patients and families.
Laurie Millers CIDP website
Good information, many links.
All about GBS: Focus on MMN
This websites exclusive collection of resources, case histories and links.
PDN info
Good source, written by a PDN-sufferer.
Recurrent Guillain Barre' syndrome

- Peripheral Neuropathy (PN)
Merck.praxis: Peripheral neuropathy
Excellent information.

- Case histories
Extensive database of Danish, English and foreign-language case histories for inspiration and support. Written by patients, their families, and even health professionals.

  Top



Use of this website signifies an acceptance of the Terms of Use above. No responsibility is taken for any errors, omissions or misinterpretations. These pages should be used for information only and you are strongly advised to seek professional help particular to your circumstances.
© Copyright 2001-2007.