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| GBS at a glance.
Names and subgroups |
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GBS - Facts at
a glance
What is a 'Syndrome'?
The many
names of the syndrome
Variants of GBS
Further reading
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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
aftereffects. 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:
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- AIDP (Acute Inflammatory
Demyelinating Polyradiculoneuropathy) = GBS
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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.
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AMSAN (Acute Motor Sensory Axonal Neuropathy)
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A serious axonal form
of GBS that attacks motor and sensory nerves. Fulminant
course with slow and incomplete recovery.
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- AMAN (Acute
Motor Axonal Neuropathy)
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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.
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| - 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' below for more
information.
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Variants with slow progressive phase:
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- CIDP (Chronic
Inflammatory Demyelinating Polyneuropathy)
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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.
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| - MMN (Multifocal
Motor Neuropathy) |
A rare form of CIDP. Limbs
weaken gradually in an apparently random pattern.
See 'Links' for more information.
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| - MMSD (Multifocal
Motor Sensory Demyelinating Neuropathy) |
Also a rare form of CIDP.
Causes weakness as well as sensory disturbances.
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| - 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.
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| - 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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Your donations
keep this site running. Please consider making
one if you've been helped thru "All about GBS"!
These donors have helped keep this site running in 2004-5:
Pam Graf, US
Konrad Tiburzy, GDR
Gabriele Wennemer,
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