GUILLAIN-BARRE SYNDROME (GBS)

Overview
Guillain-Barre syndrome (GBS) is an inflammatory disorder of the peripheral nerves. The
peripheral nerves convey sensory information (e.g., pain, temperature) from the body to
the brain and motor (i.e., movement) signals from the brain to the body. GBS is
characterized by weakness and numbness or tingling in the legs and arms, and possible
loss of movement and feeling in the legs, arms, upper body, and face.
Chronic inflammatory demyelinating polyradicalneuropathy (CIDP), is considered to be a
related form of Guillain-Barre syndrome. It is much less common than GBS, and evolves
much more slowly and usually is longer lasting. Some CIDP patients experience periods
of worsening and improvement, and individual relapses can be confused with GBS.
Incidence
Guillain-Barre syndrome is a rare disorder; its frequency is about 1 to 2 cases in every
100,000 people per year in the United States. Men and women, young and old, are
equally prone to contracting GBS.
Causes
Guillain-Barre syndrome is not hereditary or contagious. What causes GBS is not known;
however, in about half of all cases the onset of the syndrome follows a viral or bacterial
infection, such as the following:

Campylobacteriosis (usually from eating undercooked poultry)

Flu (influenza), common cold
Gastrointestinal viral infection
HIV
Infectious mononucleosis
Porphyria (rare disease of red blood cells)
Viral hepatitis

A small number of cases have been known to occur after a medical procedure, such as
minor surgery.
Guillain-Barre syndrome may be an autoimmune disorder in which the body produces
antibodies that damage the myelin sheath that surrounds peripheral nerves. The myelin
sheath is a fatty substance that surrounds axons. It increases the speed at which signals
travel along the nerves.
Diagnosis Because its symptoms vary and its cause is unknown, GBS can be difficult to
diagnose. If the symptoms occur uniformly across the body and progress rapidly, the
diagnosis is easier.

Observation of the patient's symptoms and an evaluation of the medical history provide
the basis for diagnosis of Guillain-Barre syndrome, although no single observation is
suitable to make the diagnosis.
Tests
Three tests can confirm a diagnosis of Guillain-Barre syndrome.
Lumbar puncture (spinal tap)The patient is given local anesthetic. Once the anesthetic
has taken effect, a needle is inserted between two lower (lumbar) vertebrae and a sample
of cerebrospinal fluid is drawn. An elevated level of protein without an increase in the
number of white blood cells (WBCs) in the fluid is characteristic of GBS.
Electromyogram (EMG)This is an effective diagnostic tool because it records muscle
activity and can show the loss of individual nerve impulses due to the disease's
characteristic slowing of nerve responses.
Nerve conduction velocity (NCV)This test is performed with EMG, and together, they
are often referred to as EMG/NCV studies. NCV records the speed at which signals travel
along the nerves. These signals are characteristically slowed in GBS, although the
findings may evolve over several weeks.
Definisi:
suatu kelainan sistem saraf akut dan difus yang mengenai radiks spinalis dan saraf
perifer, kadang-kadang juga saraf kranialis, biasanya timbul setelah infeksi.
Sinonim:
acute inflammatory or post-infective polyradiculoneuropathy
Variants SGB:
1. Acute inflammatory demyelinative polyneuropathy (AIDP)
2. Acute motor axonal neuropathy (AMAN)
3. The Miller-Fisher syndrome (MFS)
Penyebab:
1. Infeksi
2. Pada 40% kasus, penyebabnya tidak ditemukan
Faktor pemicu/pencetus (trigger) yang umum:
1. Campylobacter jejuni
2. Cytomegalovirus (CMV)
3. Mycoplasma
4. Zoster
5. Human Immunodeficiency Virus (HIV)
6. Epstein-Barr Virus (EBV)
Keluhan Utama:

1. Merasa mati rasa (parestesi) pada ujung-ujung anggota gerak tubuh (ekstremitas),
seperti tangan dan atau kaki
2. Kelumpuhan anggota gerak tubuh,
3. Atau kelumpuhan keduanya (no. 1 dan 2)
Gejala / Tanda:
1. Kelumpuhan otot-otot ekstremitas tipe LMN (lower motor neuron), biasanya dimulai
dari anggota gerak bawah, menjalar ke badan, anggota gerak atas, dan saraf krnialis
2. Sering dijumpai rasa nyeri setelah aktivitas fisik
3. Sering mengenai semua saraf kranialis terutama N. VII, kecuali Nervus I dan Nervus
VIII. Kelumpuhan otot-otot muka sering dimulai di satu sisi. Bila Nervus III atau Nervus
VI terkena, akan menjadi melihat dobel (diplopia). Bila Nervus IX atau Nervus X
terkena, maka akan menjadi sukar menelan dan disfonia
4. Dapat disertai muka jadi merah (facial flushing), hipertensi atau hipotensi yang naik
turun (berfluktuasi), hilangnya keringat (episodic profuse diaphoresis)
5. Terkadang disertai papiledema
Pemeriksaan Laboratorium:
Khas: terdapat disosiasi sito-albuminik, yaitu peninggian kadar protein dalam cairan otak
> 0,5 mg% tanpa diikuti peninggian jumlah sel dalam cairan otak.
Terapi:
1. Perawatan kulit, kandung kemih, saluran pencernaan, mulut, faring, trakea. Bila
disertai infeksi paru dan saluran kencing harus segera diobati.
2. Fisioterapi
3. Analgetik bila ada nyeri otot
4. Pertukaran plasma sebanyak 40-50 ml/kg, 35 kali per hari selama 714 hari.
5. Immunoglobulin intravena 0,4 gram/Kg berat badan/hari selama 5 hari.
6. Menurut Hadinoto S. dkk. (1996), kortikosteroid pada fase dini mungkin bermanfaat.
Sedangkan menurut Murray L (2007), kortikosteroid tidak berperan.
Diagnosis Banding:
1. Poliomielitis
2. Botulisme
3. Hysterical paralysis
4. Neuropati toksik
5. Diphtheritic paralysis
6. Porfiria intermiten akut
7. Neuropati karena timbal
8. Mielitis akut
9. Acute myelopathies dengan chronic back pain dan sphincter
10. Dysfunction botulism dengan early loss of pupillary reactivity
11. Diphtheria dengan early oropharyngeal dysfunction
12. Lyme disease polyradiculitis dan tick-borne paralyses lainnya
13. Porphyria dengan abdominal pain, seizures, psychosis
14. Vasculitis neuropathy

15. Poliomyelitis dengan demam dan meningeal signs

16. CMV polyradiculitis pada pasien-pasien immunocompromised
17. Critical illness neuropathy
18. Myasthenia gravis
19. Keracunan organophosphate, poison hemlock, thallium, atau arsenic.
20. Paresis karena West Nile Virus
21. Spinal astrocytoma
Catatan:
1. Insiden SGB adalah 1-2 orang per 100 ribu populasi per tahun.
2. Pada SGB, Biasanya antara 1-3 minggu sebelumnya ada infeksi ringan saluran
pencernaan atau saluran pernapasan, atau dapat timbul setelah pembedahan, dan dapat
pula timbul setelah vaksinasi influenza, dapat disertai infeksi virus, bakteri, atau jamur,
atau dapat disertai eksantema pada kulit, atau dapat disertai penyakit limfoma.
3. Komplikasi utama SGB adalah kegagalan pernafasan.
4. Seluruh perjalanan penyakit SGB, yang terdiri dari 3 fase ini (fase progresif, fase
plateau, fase penyembuhan), berlangsung selama 6 bulan.
5. Sekitar 20-30% kasus SGB didahului oleh infeksi karena
Campylobacter jejuni dan Cytomegalovirus (CMV).

What is Guillain-Barr Syndrome?

Guillain-Barr syndrome is a disorder in which the body's immune system attacks part of
the peripheral nervous system. The first symptoms of this disorder include varying
degrees of weakness or tingling sensations in the legs. In many instances, the weakness
and abnormal sensations spread to the arms and upper body. These symptoms can
increase in intensity until the muscles cannot be used at all and the patient is almost
totally paralyzed. In these cases, the disorder is life-threatening and is considered a
medical emergency. The patient is often put on a respirator to assist with breathing. Most
patients, however, recover from even the most severe cases of Guillain-Barr syndrome,
although some continue to have some degree of weakness. Guillain-Barr syndrome is
rare. Usually Guillain-Barr occurs a few days or weeks after the patient has had
symptoms of a respiratory or gastrointestinal viral infection. Occasionally, surgery or
vaccinations will trigger the syndrome. The disorder can develop over the course of hours
or days, or it may take up to 3 to 4 weeks. No one yet knows why Guillain-Barr strikes
some people and not others or what sets the disease in motion. What scientists do know is
that the body's immune system begins to attack the body itself, causing what is known as
an autoimmune disease. Guillain-Barr is called a syndrome rather than a disease because
it is not clear that a specific disease-causing agent is involved. Reflexes such as knee
jerks are usually lost. Because the signals traveling along the nerve are slower, a nerve
conduction velocity (NCV) test can give a doctor clues to aid the diagnosis. The
cerebrospinal fluid that bathes the spinal cord and brain contains more protein than usual,
so a physician may decide to perform a spinal tap.

Is there any treatment?

There is no known cure for Guillain-Barr syndrome, but therapies can lessen the severity
of the illness and accelerate the recovery in most patients. There are also a number of
ways to treat the complications of the disease. Currently, plasmapheresis and high-dose
immunoglobulin therapy are used. Plasmapheresis seems to reduce the severity and
duration of the Guillain-Barr episode. In high-dose immunoglobulin therapy, doctors
give intravenous injections of the proteins that in small quantities, the immune system
uses naturally to attack invading organism. Investigators have found that giving high
doses of these immunoglobulins, derived from a pool of thousands of normal donors, to
Guillain-Barr patients can lessen the immune attack on the nervous system. The most
critical part of the treatment for this syndrome consists of keeping the patient's body
functioning during recovery of the nervous system. This can sometimes require placing
the patient on a respirator, a heart monitor, or other machines that assist body function.
What is the prognosis?
Guillain-Barr syndrome can be a devastating disorder because of its sudden and
unexpected onset. Most people reach the stage of greatest weakness within the first 2
weeks after symptoms appear, and by the third week of the illness 90 percent of all
patients are at their weakest. The recovery period may be as little as a few weeks or as
long as a few years. About 30 percent of those with Guillain-Barr still have a residual
weakness after 3 years. About 3 percent may suffer a relapse of muscle weakness and
tingling sensations many years after the initial attack.
What research is being done?
Scientists are concentrating on finding new treatments and refining existing ones.
Scientists are also looking at the workings of the immune system to find which cells are
responsible for beginning and carrying out the attack on the nervous system. The fact that
so many cases of Guillain-Barr begin after a viral or bacterial infection suggests that
certain characteristics of some viruses and bacteria may activate the immune system
inappropriately. Investigators are searching for those characteristics. Neurological
scientists, immunologists, virologists, and pharmacologists are all working
collaboratively to learn how to prevent this disorder and to make better therapies
available when it strikes.

GBS - Facts at a glance

Guillain-Barr 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.
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-Barr syndrome.

The many names of the syndrome

The syndrome was named after the French physicians Guillain, Barr 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:
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.
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-Barr 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' below 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.

SINDROMA GUILLAIN-BARR (SGB)

Darto Saharso
Divisi Neuropediatri
Bag./SMF Ilmu Kesehatan Anak FK Unair/RSU Dr. Soetomo Surabaya
BATASAN
Sinonimnya: Guillain-Barr Syndrome (GBS) atau Acute Inflammatory Demyelinating
Polyneuropathy (AIDP) atau Acute Febrile Polyneuritis adalah kelemahan motorik yang
progresif dan arefleksi. Sering disertai gangguan sensorik, otonomik dan abnormalitas
batang otak. Timbulnya didahului oleh infeksi virus.
PATOFISIOLOGI
Adanya infeksi virus menyebabkan penurunan kadar supresor sel-T sehingga kadar sel-T,
sel-B dan limfosit meningkat. Kemudian sel limfosit dan makrofag melakukan infiltrasi
ke dalam membran basalis serabut saraf sehingga menimbulkan kerusakan mielin dan
degenerasi Wallerian, yang kemudian menimbulkan inflamasi saraf tepi terutama di
daerah radiks saraf.
Beberapa pemicu patogen terjadinya SGB antara lain: virus Epstein-Barr, virus
sitomegalo, hepatitis, varisela, Mycoplasma pneumonia dan Campylobacter jejuni.
Dugaan bahwa imunitas selular atau humoral berperan dalam kerusakan mielin masih
merupakan kontroversi.
CD4+ helper-inducer T-cell merupakan mediator penting terjadinya SGB. Antigen
spesifik seperti myelin P-2, ganglioside GQ1b, GM1, dan GT1a diduga ikut berperan
dalam proses penyakit.
Ada 2 bentuk SGB, yaitu:
1. Tipe demyelinating
Terjadi demielinisasi segmental saraf tepi yang disebabkan oleh infiltrasi sel-sel
radang

2. Tipe axonal
Terjadi degenerasi akson tanpa proses demielinisasi atau peradangan
GEJALA KLINIS

Kelumpuhan akut, simetris dan ascending

Nyeri dan gangguan sensori

Hipotensi ortostatik

Pengeluaran keringat abnormal

Takikardia

PEMERIKSAAN DAN DIAGNOSIS

Anamnesis:
o Riwayat infeksi virus 2-4 minggu sebelumnya
o Retensi urin (10-15%)
o Nyeri (50%), sehingga anak menjadi rewel dan irritable
Pemeriksaan fisik:
o Kelemahan otot ascending dan hilangnya refleks fisiologis (Tanda khas
SGB).
o Kelemahan kaki (dropfoot) merupakan gejala pertama. Dan kelemahan ini
dapat mengenai otot-otot pernafasan hingga membutuhkan respirator.
o Instabilitas otonom (26%). Berupa neuropati otonomik yang mengenai
sistim simpatis dan parasimpatis dengan manifestasi klinis berupa
hipotensi ortostatik, disfungsi pupil, pengeluaran keringat abnormal dan
takikardia.
o Ataksia (23%)
o Gangguan saraf kranial (35-50%)
Pemeriksaan laboratorium:
Cairan Serebro Spinal (CSS): hasil analisa CSS normal dalam 48 jam pertama,
kemudian diikuti kenaikan kadar protein CSS pada minggu II tanpa atau disertai
sedikit kenaikan lekosit (albuminocytologic dissociation).

Pemeriksaan elektrofisiologi:
EMG dan Nerve Conduction Velocity (NCV):

Minggu I: terjadi pemanjangan atau hilangnya F-response (88%), prolong

distal latencies (75%), blok pada konduksi (58%) dan penurunan
kecepatan konduksi (50%).
Minggu II: terjadi penurunan potensial aksi otot (100%), prolong distal
latencies (92%) dan penurunan kecepatan konduksi (84%).

Pemeriksaan radiologi:
MRI: Sebaiknya MRI dilakukan pada hari ke 13 setelah timbulnya gejala SGB.
Pemeriksaan MRI dengan menggunakan kontras gadolinium memberikan
gambaran peningkatan penyerapan kontras di daerah lumbosakral terutama di
kauda equina. Sensitivitas pemeriksaan ini pada SGB adalah 83%.

DIAGNOSIS BANDING

Poliomielitis
Miositis akut
Lesi medula spinalis

PENATALAKSANAAN
1. Intravenous Imunoglobulin (IVIG) 0,4 g/KgBB/hari IV, selama 5 hari. Perbaikan
klinis mulai tampak setelah hari ke 2-3. Terapi ini dapat menurunkan beratnya
penyakit dan mempersingkat lamanya sakit.
2. Plasmafaresis dilakukan 4-5 kali dalam waktu 7-10 hari (hati-hati dapat terjadi
hiperkalsemia, perdarahan karena kelainan pembekuan darah dan gangguan
otonom).
3. Dexamethasone 0,5 mg/Kg/hari dibagi dalam 3 dosis (kontroversial).
4. Rehabilitasi medis diperlukan pada penderita yang sakit lama.
5. Alat bantu pernafasan (respirator): apabila terjadi kelumpuhan pada otot-otot
pernafasan.
KOMPLIKASI

Konstipasi (40%)
Aritmia (30%)
Hipertensi (10-30%)
Pnemoni ortostatik
Syndrome inapropriate antidiuretic hormone (SIADH) (3%)
Dekubitus
Kontraktur

PROGNOSIS
Penderita SGB dapat sembuh sempurna (75-90%) atau sembuh dengan gejala sisa berupa
dropfoot atau tremor postural (25-36%).

Pada SGB tipe aksonal dengan kelumpuhan hebat prognosisnya jelek dengan angka
kematian 1-5% dan kematian biasanya disebabkan karena gagal nafas.
Bila terjadi kekambuhan atau tidak ada perbaikan pada akhir minggu IV maka termasuk
Chronic Inflammantory Demyelinating Polyradiculoneuropathy (CIDP)

Guillain-Barre Syndrome Causes

The cause of the disease is unknown. Many speculate that this is an immune-system
disorder. Symptoms often begin 5 days to 3 weeks after a viral infection, immunization,
or surgery.
The disease affects peripheral nerves, nerve roots, and cranial nerves. Evaluation of the
peripheral nerves reveals sections of the nerve with demyelination. Under microscopic
exam, the nerve tissue is infiltrated with certain types of white blood cells.

A viral infection, such as herpes, cytomegalovirus, or Epstein-Barr virus is the

cause of over two-thirds of the new cases each year.

In 1977, there were over 500 cases of Guillain-Barre syndrome associated with a
United States flu vaccination program. The cause of this outbreak was never
discovered.
5-10% of new cases will occur up to 4 weeks after surgery.

Guillain-Barre Syndrome Symptoms

Weakness on both sides of the body may develop with numbness that starts in the
legs and progresses into the trunk and moves upward to the arms and neck.

Muscles that are controlled by nerves in the head may be involved. Muscle
weakness near the involved nerves can be the most prominent sign.
Deep tendon reflexes are decreased or absent.
People can have weakness of facial muscles and some muscles in the throat.
Some may have respiratory failure due to muscle weakness. These people need to
have a breathing tube put in and be placed on a ventilator to help them breathe.
Five percent of people die from respiratory failure.
Rapid heartbeat (tachycardia), sweating, facial flushing, and variable blood
pressure are signs the nervous system is affected.
The severity of symptoms peaks by the second or third week.
In certain forms of Guillain-Barre syndrome, people have weakness of eye
muscles or unsteady gait. These symptoms overlap other syndromes such as
botulism, thiamine deficiency, and myasthenia gravis. It is important to rule out
other causes for these symptoms.

GuillainBarr syndrome (GBS) (French pronunciation: [ij bae];[1][2] in English,

pronounced /iln bre/,[3] /iln bre/,[4] etc[5]) is an acute inflammatory

demyelinating polyneuropathy (AIDP), an autoimmune disorder affecting the peripheral

nervous system, usually triggered by an acute infectious process.The syndrome was
named after the French physicians Guillain, Barr 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 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, AIDP. GBS is rare and has an incidence of 1 or 2 people per
100,000.[6] 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 autonomic
nervous system problems are present.[7] GuillainBarr is one of the leading causes of
non-trauma-induced paralysis in the world.
Classification
Six different subtypes of GuillainBarr syndrome (GBS) exist:

Acute inflammatory demyelinating polyneuropathy (AIDP) is the most

common form of GBS, and the term is often used synonymously with GBS. It is
caused by an auto-immune response directed against Schwann cell membranes.
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 eye muscles first and presents with the triad of
ophthalmoplegia, ataxia, and areflexia. Anti-GQ1b antibodies are present in 90%
of cases.
Acute motor axonal neuropathy (AMAN),[8] aka Chinese Paralytic
Syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico.
It is likely due to an auto-immune response directed against the axoplasm of
peripheral nerves. The disease may be seasonal and recovery can be rapid. AntiGD1a antibodies[9] 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. Like AMAN, it is likely
due to an auto-immune response directed against the axoplasm of peripheral
nerves. Recovery is slow and often incomplete.[10]
Acute panautonomic neuropathy is the most rare variant of GBS, sometimes
accompanied by encephalopathy. It is associated with a high mortality rate, due to
cardiovascular involvement, and associated dysrhythmias. Impaired sweating,
lack of tear formation, photophobia, dryness of nasal and oral mucosa, itching and
peeling of skin, nausea, dysphagia, constipation unrelieved by laxatives or
alternating with diarrhea occur frequently in this patient group. Initial nonspecific
symptoms of lethargy, fatigue, headache, and decreased initiative are followed by
autonomic symptoms including orthostatic lightheadedness, blurring of vision,
abdominal pain, diarrhea, dryness of eyes, and disturbed micturition. The most

common symptoms at onset are related to orthostatic intolerance, as well as

gastrointestinal and sudomotor dysfunction (Suarez et al. 1994). Parasympathetic
impairment (abdominal pain, vomiting, obstipation, ileus, urinary retention,
dilated unreactive pupils, loss of accommodation) may also be observed.
Bickerstaffs brainstem encephalitis (BBE), is a further variant of Guillain
Barr syndrome. It is characterized by acute onset of ophthalmoplegia, ataxia,
disturbance of consciousness, hyperreflexia or Babinskis sign (Bickerstaff, 1957;
Al-Din et al.,1982). The course of the disease can be monophasic or remittingrelapsing. Large, irregular hyperintense lesions located mainly in the brainstem,
especially in the pons, midbrain and medulla are described in the literature. BBE
despite severe initial presentation usually has a good prognosis. Magnetic
resonance imaging (MRI) plays a critical role in the diagnosis of BBE.

A considerable number of BBE patients have associated axonal GuillainBarr

syndrome, indicative that the two disorders are closely related and form a continuous
spectrum.
Signs and symptoms
The disorder is characterized by symmetrical weakness which usually 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.[11] 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 disorder 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.
Acute paralysis in GuillainBarr syndrome may be related to sodium channel blocking
factor in the cerebrospinal fluid (CSF). Significant issues involving intravenous salt and
water administration may occur unpredictably in this patient group, resulting in SIADH.

The symptoms are similar to those for progressive inflammatory neuropathy.[12]

Cause
All forms of GuillainBarr syndrome are due to an immune response to foreign antigens
(such as infectious agents) that are mistargeted at host nerve tissues instead. The targets
of such immune attack are thought to be gangliosides, compounds naturally present in
large quantities in human nerve tissues. The most common antecedent infection is the
bacteria Campylobacter jejuni.[13] However, 60% of cases do not have a known cause; one
study suggests that some cases are triggered by the influenza virus, or by an immune
reaction to the influenza virus.[14]
The end result of such autoimmune attack on the peripheral nerves is damage to the
myelin, the fatty insulating layer of the nerve, and a nerve conduction block, leading to a
muscle paralysis that may be accompanied by sensory or autonomic disturbances.
However, in mild cases, nerve axon (the long slender conducting portion of a nerve)
function remains intact and recovery can be rapid if remyelination occurs. In severe
cases, axonal damage occurs, and recovery depends on the regeneration of this important
tissue. Recent studies on the disorder have demonstrated that approximately 80% of the
patients have myelin loss, whereas, in the remaining 20%, the pathologic hallmark of the
disorder is indeed axon loss.
GuillainBarr, unlike disorders such as multiple sclerosis (MS) and Lou Gehrig's
disease (ALS), is a peripheral nerve disorder and does not generally cause nerve damage
to the brain or spinal cord.
Influenza vaccine
GBS may be a rare side-effect of influenza vaccines; a study of the Vaccine Adverse
Event Reporting System (VAERS) indicates that it is reported as an adverse event
potentially associated with the vaccine at a rate of about 1 per million vaccines.[15] There
were reports of GBS affecting about 500 people who had received swine flu
immunizations in the 1976 U.S. outbreak of swine flu25 of which resulted in death
from severe pulmonary complications, leading the government to end that immunization
campaign.[16] However, the role of the vaccine in these cases has remained unclear, partly
because GBS had an unknown but very low incidence rate in the general population
making it difficult to assess whether the vaccine was really increasing the risk for GBS.
Later research has pointed to the absence of or only a very small increase in the GBS risk
due to the 1976 swine flu vaccine.[17] Furthermore, the GBS may not have been directly
due to the vaccine but to a bacterial contamination of the vaccine.[18]
Since 1976, no other influenza vaccines have been linked to GBS, though as a
precautionary principle, caution is advised for certain individuals, particularly those with
a history of GBS.[19][20] On the other hand, getting infected by the flu increases the risk of

developing GBS to a much higher level (approx. 10 times higher by recent estimates[21])
and, all in all, the flu vaccination contributes protection against the risk of GBS.[22]
From October 6 to November 24, 2009, the U.S. CDC, through the VAERS reporting
system, received ten reports of Guillain-Barre syndrome cases associated with the H1N1
vaccine and identified two additional probable cases from VAERS reports (46.2 million
doses were distributed within the U.S. during this time). Only four cases, however, meet
the Brighton Collaboration Criteria for GuillainBarr syndrome, while four do not meet
the criteria and four remain under review.[23]
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 (1001000 mg/dL), without an
accompanying increased cell count pleocytosis. A sustained increased white blood
cell count may indicate an alternative diagnosis such as infection.
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)
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

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
West Nile virus can cause severe, potentially fatal neurological illnesses, which
include encephalitis, meningitis, Guillain-Barre syndrome, and anterior myelitis.
Severe Myalgic Encephalomyelitis/Chronic Fatigue Syndrome.
Management
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 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,[24][25] 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. 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 510% 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 23% 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 510% 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 >40 years, 2) history of preceding diarrheal
illness, 3) requiring ventilator support, 4) high anti-GM1 titre and 5) poor upper limb
muscle strength.
Case reports do exist of rapid patient recovery.
Epidemiology
The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population.[26]
The mother will generally improve with treatment but death of the fetus is a risk. The risk
of GuillainBarr syndrome increases after delivery, particularly during the first two
weeks postpartum. There is evidence of Campylobacter jejuni as an antecedent infection
in approximately 26% of disease cases, requiring special care in the preparation and

handling of food. Congenital and neonatal GuillainBarr syndrome have also been
reported.[27]
History
The disorder was first described by the French physician Jean Landry in 1859. In 1916,
Georges Guillain, Jean Alexandre Barr, and Andr Strohl diagnosed two soldiers with
the illness and discovered the key diagnostic abnormality of increased spinal fluid protein
production, but normal cell count.[28]
GBS is also known as acute inflammatory demyelinating polyneuropathy, acute
idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio, Landry's
ascending paralysis and Landry Guillain Barr syndrome.