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.
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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.
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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.
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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.
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|
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.
|
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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.
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