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Guillain-Barré syndrome
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Guillain-Barré syndrome
Classification and external
resources

ICD-10 G61.0

ICD-9 357.0

OMIM 139393

DiseasesDB 5465

MedlinePlus 000684

eMedicine emerg/222
neuro/7 pmr/48
neuro/598

MeSH D020275

Guillain-Barré syndrome (GBS) (in French pronounced [ɡilɛ̃ baˈʁe][1][2], in English

pronounced /ˈɡiːlæn ˈbɑreɪ/[3], /ɡiːˈæn bəˈreɪ/,[4] etc.[5]) is an acute inflammatory
demyelinating polyneuropathy (AIDP), an autoimmune disease affecting the peripheral
nervous system, usually triggered by an acute infectious process. It is included in the
wider group of peripheral neuropathies. There are several types of GBS, but unless
otherwise stated, GBS refers to the most common form, acute inflammatory
demyelinating polyneuropathy (AIDP). It is frequently severe and usually exhibits as an
ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the
face along with complete loss of deep tendon reflexes. With prompt treatment by
plasmapheresis or intravenous immunoglobulins and supportive care, the majority of
patients will regain full functional capacity. However, death may occur if severe
pulmonary complications and dysautonomia are present.

Contents
[hide]
 • 1 Pathophysiology
• 2 Signs and symptoms
o 2.1 Clinical variants
• 3 Diagnosis
o 3.1 Diagnostic criteria
 3.1.1 Required
 3.1.2 Supportive
o 3.2 Differential diagnosis
• 4 Treatment
• 5 Prognosis
• 6 History
• 7 Notable sufferers
• 8 References

• 9 External links

[edit] Pathophysiology
All forms of Guillain-Barré syndrome are due to an immune response to foreign antigens
(such as infectious agents or vaccines) but mistargeted to host nerve tissues instead (a
form of antigenic mimicry). The targets of such immune attack are thought to be
gangliosides, which are complex glycosphingolipids present in large quantities on human
nerve tissues, especially in the nodes of Ranvier. An example is the GM1 ganglioside,
which can be affected in as many as 20-50% of cases, especially in those preceded by
Campylobacter jejuni infections. Another example is the GQ1b ganglioside, which is the
target in the Miller Fisher syndrome variant (see below).

The end result of such autoimmune attack on the peripheral nerves is inflammation of
myelin and conduction block, leading to a muscle paralysis that may be accompanied by
sensory or autonomic disturbances.

However, in mild cases, axonal function remains intact and recovery can be rapid if
remyelination occurs. In severe cases, such as in the AMAN or AMSAN variants (see
below), axonal degeneration occurs, and recovery depends on axonal regeneration.
Recovery becomes much slower, and there is a greater degree of residual damage. Recent
studies on the disease have demonstrated that approximately 80% of the patients have
myelin loss, whereas, in the remaining 20%, the pathologic hallmark of the disease is
indeed axon loss.

[edit] Signs and symptoms

The disease is characterized by weakness which affects the lower limbs first, and rapidly
progresses in an ascending fashion. Patients generally notice weakness in their legs,
manifesting as "rubbery legs" or legs that tend to buckle, with or without dysesthesias
(numbness or tingling). As the weakness progresses upward, usually over periods of
hours to days, the arms and facial muscles also become affected. Frequently, the lower
cranial nerves may be affected, leading to bulbar weakness, (oropharyngeal dysphagia,
that is difficulty with swallowing, drooling, and/or maintaining an open airway) and
respiratory difficulties. Most patients require hospitalization and about 30% require
ventilatory assistance. Facial weakness is also commonly a feature, but eye movement
abnormalities are not commonly seen in ascending GBS, but are a prominent feature in
the Miller Fisher variant (see below.) Sensory loss, if present, usually takes the form of
loss of proprioception (position sense) and areflexia (complete loss of deep tendon
reflexes), an important feature of GBS. Loss of pain and temperature sensation is usually
mild. In fact, pain is a common symptom in GBS, presenting as deep aching pain usually
in the weakened muscles, which patients compare to the pain from overexercising. These
pains are self-limited and should be treated with standard analgesics. Bladder dysfunction
may occur in severe cases but should be transient. If severe, spinal cord disease should be
suspected.

Fever should not be present, and if it is, another cause should be suspected.

In severe cases of GBS, loss of autonomic function is common, manifesting as wide

fluctuations in blood pressure, orthostatic hypotension, and cardiac arrhythmias.

The symptoms are similar to those for progressive inflammatory neuropathy.[6]

[edit] Clinical variants

Although ascending paralysis is the most common form of spread in GBS, other variants
also exist.

• Miller Fisher Syndrome (MFS) is a rare variant of GBS and manifests as a

descending paralysis, proceeding in the reverse order of the more common form
of GBS. It usually affects the ocular muscles first and presents as
ophthalmoplegia, ataxia, and areflexia. Anti-GQ1b antibodies are present in 90%
of cases.
• Acute motor axonal neuropathy (AMAN)[7], aka. Chinese Paralytic
Syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico.
The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies[8]
are present. Anti-GD3 antibodies are found more frequently in AMAN
• Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but
also affects sensory nerves with severe axonal damage. Recovery is slow and
often incomplete.[9]

[edit] 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. CSF and ECD is used
almost every time to verify symptoms, but because of the acute nature of the disease, they
may not become abnormal until after the first week of onset of signs and symptoms.

• CSF

typical CSF findings include albumino-cytological dissociation, this is, an

elevated protein level (100 - 1000 mg/dL) without an accompanying pleocytosis
(increased cell count), as opposed to infectious causes. A sustained pleocytosis
may indicate an alternative diagnosis such as infection.

Researchers speculate as to why protein is elevated without pleocytosis in the CSF.

Infilitration of WBCs in the myelin are responsible for demyelination in the peripheral
nerves. Specific, 2D gel electrophoresis showed proteins unspecifically affected in
different inflammatory as well as non-inflammatory neurological diseases and may be of
limited value as disease-related biochemical markers in GBS. [Proteome analysis of
cerebrospinal fluid in Guillain–Barré syndrome]

• 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.

[edit] Diagnostic criteria

[edit] Required

• Progressive, relatively symmetrical weakness of 2 or more limbs due to

neuropathy
• Areflexia
• Disease course < 4 weeks
• Exclusion of other causes (see below)

[edit] 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
[edit] Differential diagnosis

• acute myelopathies with chronic back pain and sphincter dysfunction

• botulism with early loss of pupillary reactivity
• 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
• paresis caused by West Nile Virus
• spinal astrocytoma
• Motor Neurone Disease

[edit] 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) <-25 cmH2O, more
than 30% decrease in either VC or NIF within 24 hours, rapid progression of disease, 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 400mg/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 after 2 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 (.4kg 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) is 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, there will be established a team of different therapists and
nurses according to patient needs. An occupational therapist can offer equipment (such as
wheel chair and cutlery) to help the patient achieve ADL independence. A physiotherapist
would plan a progressive training programme, and guide the patient to correct, functional
movement, avoiding harmful compensations which might have a negative effect in the
long run. 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 with
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.

[edit] Prognosis
Most of the time recovery starts after 4th week from the onset of the disease.
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 disease and
despite all improvements in treatment and supportive care, the death rate among patients
with this disease 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 4 to 6 weeks, and in some cases up to 1
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).

[edit] History
The disease was first described by the French physician Jean Landry in 1859. In 1916,
Georges Guillain, Jean Alexandre Barré, and Andre Strohl diagnosed two soldiers with
the illness and discovered the key diagnostic abnormality of increased spinal fluid protein
production, but normal cell count.[10]

GBS is also known as acute inflammatory demyelinating polyneuropathy, acute

idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio, Landry's
ascending paralysis and Landry Guillain Barre syndrome.

[edit] Notable sufferers

• Andy Griffith, actor on Andy Griffith Show, and Matlock. He contracted Guillain-
Barré in 1983. [11]
• Rachel Chagall, actress, contracted GBS in 1982. In 1987 she portrayed Gabriela
Brimmer, a notable disabilities activist. [12]
 • Joseph Heller, author, contracted GBS in 1981. This episode in his life is
recounted in the autobiographical No Laughing Matter, which contains alternating
chapters by Heller and his good friend Speed Vogel.[13]
• Franklin D. Roosevelt, U.S. president. In 2003, a peer-reviewed study[14] found
that it was more likely that Roosevelt's paralysis--long attributed to
poliomyelitis--was actually Guillain-Barré syndrome.
• Markus Babbel, former international footballer, contracted GBS in 2001,
following a period suffering from the Epstein-Barr virus. He lost almost an entire
year of his footballing career between the two illnesses and never again
demonstrated the same level of ability that won him over 50 caps for Germany.
[1]
• Serge Payer, Canadian-born professional hockey player. After battling and
overcoming the syndrome, He set up the Serge Payer foundation, which is
dedicated to raising money for research into new treatments and cures for
Guillain-Barré syndrome[15].

[edit] References
1. ^ "John Wells's phonetic blog, 23rd February, 2007".
2. ^ "See also, in the same blog, the entry of October 20th, 2008".
3. ^ Recommended by the "GBS Support Group".
4. ^ "Guillain-Barre Syndrome". Dictionary.com Unabridged (v 1.1). Random
House.
5. ^ In English, Guillain may be pronounced with an L sound as in French, but it is
common to pronounce it without one, originally based on the mistaken
assumption that the French pronunciation of the ll is IPA [ j ] and not [ l ]. In
English, both Guillain and Barré may be pronounced with the stress on either the
first or the last syllable. The nasal vowel [ɛ̃]at the end of Guillain is either kept in
English or replaced by a sequence of an oral vowel and a nasal consonant such as
[æn].
6. ^ David Brown (2008-02-04). "Inhaling Pig Brains May Be Cause of New
Illness". The Washington Post. Retrieved on 2008-02-04.
7. ^ McKhann GM, Cornblath DR, Ho T, et al (1991). "Clinical and
electrophysiological aspects of acute paralytic disease of children and young
adults in northern China". Lancet 338 (8767): 593–7. doi:10.1016/0140-
6736(91)90606-P. PMID 1679153.
8. ^ Ho TW, Mishu B, Li CY, et al (1995). "Guillain-Barré syndrome in northern
China. Relationship to Campylobacter jejuni infection and anti-glycolipid
antibodies". Brain 118 ( Pt 3): 597–605. PMID 7600081.
9. ^ Griffin JW, Li CY, Ho TW, et al (1995). "Guillain-Barré syndrome in northern
China. The spectrum of neuropathological changes in clinically defined cases".
Brain 118 ( Pt 3): 577–95. PMID 7600080.
10. ^ Guillain-Barré-Strohl syndrome and Miller Fisher's syndrome at Who Named It
11. ^ "Andy in Guideposts Magazine".
12. ^ "Gaby, A True Story (1987)". Films involving Disabilities.
 13. ^ Vogel, Speed; Heller, Joseph (2004). No Laughing Matter. New York: Simon &
Schuster. ISBN 0-7432-4717-5.
14. ^ Goldman AS, Schmalstieg EJ, Freeman DH, Goldman DA, Schmalstieg FC
(2003). "What was the cause of Franklin Delano Roosevelt's paralytic illness?". J
Med Biogr 11 (4): 232–40. PMID 14562158.
15. ^ Serge Payer Foundation, Serge Payer Foundation Mission.

[edit] External links

• GBS/CIDP Foundation International
• Information from the Guillain-Barré Syndrome Support Group (UK)
• Information from GBS Association of New South Wales (AU)
• miller_fisher at NINDS

[show]
v•d•e
Multiple sclerosis
 [show]
v•d•e
Nervous system pathology, primarily PNS (G50-G99, 350-359)

V (Trigeminal neuralgia) - VII (Facial nerve paralysis,

Cranial nerve diseaseBell's palsy, Melkersson-Rosenthal syndrome, Central
seven) - XI (Accessory nerve disorder)

Brachial plexus lesion - Thoracic outlet syndrome -

Radiculopathy, plexopathy
Phantom limb

upper limb (Carpal tunnel syndrome, Ulnar nerve

entrapment, Radial neuropathy)

Mono- neuropathylower limb (Meralgia paraesthetica, Tarsal tunnel

syndrome, Morton's neuroma)

Causalgia - Mononeuritis multiplex

Charcot-Marie-Tooth disease - Dejerine Sottas syndrome

HMSN
- Refsum's disease

autoimmune (Guillain-Barré syndrome, Chronic

Polyradiculoneuropathyinflammatory demyelinating polyneuropathy) - Alcoholic
polyneuropathy

autoimmune (Myasthenia gravis, Lambert-Eaton

Neuromuscular junction disease
myasthenic syndrome)

Congenital - dystrophin (Becker's, Duchenne) -

Distal - Emery-Dreifuss - Facioscapulohumeral -
Muscular dystrophy
Limb-girdle muscular dystrophy - Myotonic -
Oculopharyngeal

Myotonic dystrophy - Myotonia congenita -

MyotoniaThomsen disease - Neuromyotonia -
Paramyotonia congenita

Bethlem myopathy - Central core disease -

Congenital myopathyCentronuclear myopathy - Nemaline myopathy -
Zaspopathy

Mitochondrial myopathyMELAS - MERRF - KSS - PEO

Periodic paralysisHypokalemic - Hyperkalemic

 [show]
v•d•e
Immune disorders: Hypersensitivity and autoimmune diseases

Retrieved from "http://en.wikipedia.org/wiki/Guillain-Barr%C3%A9_syndrome"

Categories: Multiple sclerosis | Autoimmune diseases | Neurological disorders |
Neurology | Syndromes

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