Guillain-Barré syndrome is an acute, inflammatory, postinfectious polyneuropathy marked by a prodromal malaise with nausea, vomiting, headache, fever (may or may not be present) and joint pain. GBS is rapidly surmounted by a progressive and ascending paralysis which can lead to respiratory dysfunction. The acute presentation is a neurological emergency.
GBS is a form of autoimmune disease with a delayed hypersensitivity reaction. It is a rare manifestation of serum sickness or a transient syndrome resembling serum sickness with hematuria, loss of appetite, nausea, vomiting, and stomach pain accompanied by weakness (tired feeling), chills, low grade fever and possible evidence of brain involvement, indicated by lethargy and migraine headaches which may initially suggest a course of viral hepatitis, although one theory of the cause of migraine is a central nervous system (CNS) disorder. Serum sickness is of itself a type of delayed allergic response. Vaccines, broad spectrum antibiotics and "virus in stealth" to help kill cancer cells are themselves an onslaught on the immune system which can cause serum sickness leading to GBS or "provocation polio", which is a hallmark of GBS. The most serious complications of serum sickness are nerve conditions such as Guillain-Barre syndrome and peripheral neuritis.
T cells, which are mediators of immune function are also involved in the pathogenesis of most or perhaps all forms of GBS. The flaccid phase may be overthrown by the inadvertent generation of mutant T cell receptors that are anti-self; i.e., a super-immune response sending white blood cells (called T cells) rampaging through the body destroying its own tissues. We have an overactive immune system followed by an exhausted immune system. Unpleasant signs or symptoms, such as high blood pressure, headaches, backaches, and upset stomach, will take control, steering the body and the exhausted immune system into dangerous waters, ie. Sarcoidosis is caused by an overactive immune system allowing inflammation to spread out of control. Nutrient deficiency may also contribute to a depressed immune system and may ALSO be observed in patients with GBS. T cells belong to a group of white blood cells known as lymphocytes. T cell depression in serum sickness, marked by a drop in the absolute number of lymphocytes (T-cells literally become worn out) is usually associated with iatrogenic causes, most often secondary to drugs, notably penicillin based medicines and sulfonamides such as Bactrim. It is also well documented that Cipro causes drug-induced serum sickness which can rapidly progress to GBS. Guillain-Barré syndrome and clinically similar states have been reported to occur with a variety of drugs and biologics, including radiation therapy. Many of the cases seem to be triggered by a microbial infection actually follow its course with a causal link to the drugs and the treatment. Catastrophic over-stimulation of the immune system in patients with or without serum sickness can trigger GBS. Alteration in the permeability of the blood-brain barrier may predispose.
Guillain-Barré syndrome (GBS) has become an umbrella term for different disorders representing a collection of clinical syndromes, some of which may overlap each other. 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, a syndrome of ophthalmoplegia, ataxia and areflexia associated with acute idiopathic ophthalmologic neuropathy. Strictly speaking, that and only that, is MFS. The connection with GBS comes because some GBS patients have paralysed eye muscles too. Consequently, MFS and GBS can overlap. Miller Fisher-Guillain-Barre overlap syndrome is a postinfectious allergic reaction involving both peripheral nerves in the cranium and neuraxis in the spinocerebellar tract. Lesions in the spinocerebellar tracts may be responsible for cerebellar ataxia in this syndrome (see below).
The end result of such autoimmune attack on the peripheral nerves is inflammation of myolin and subsequent conduction block, leading to a rapidly evolving flaccid paralysis with or without accompanying sensory or autonomic disturbances. Autonomic neuropathy is a group of symptoms caused by damage to nerves that regulate blood pressure, heart rate, bowel and bladder emptying, digestion, and other body functions. Constipation is often a problem, due to the reduced activity of the intestines, change of diet, weakened stomach muscles that resist efforts by the patient to express the intestinal content. Bowel and bladder dysfunction with intestinal muscle paralysis; constipation, obstipation and megacolon is a consequence of defect in the nerve supply of the colon. Urinary retention and paralytic ileus may also be observed. Paralysis results because the immune system destroys the insulation that covers nerves. Nerve signals can't pass to muscles. Physical findings include delayed pupillary light response, resting tachycardia, sinus arrhythmia, and orthostatic hypotension.
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 myolin loss, whereas, in the remaining 20%, the pathologic hallmark of the disease is indeed axon loss.
CNS pathology is frequent in patients with Guillain-Barré syndrome. It involves axons with secondary myolin impairment, microglial activation and inflammatory infiltration. Changes such as degeneration of spinal posterior tracts are secondary to pathology in the peripheral nervous system (PNS). The PNS nerves connect the central nervous system (CNS), which consists of the brain and spinal cord and their associated supportive tissue, to the rest of the body. Guillain-Barré syndrome is a rare disorder that causes the immune system to attack the peripheral nervous system (PNS). Inflammatory cell reactions in the CNS are similar to those in the PNS and to CNS pathology in allergic neuritis. In the month or so before the syndrome’s appearance, patients most often have had a respiratory or digestive-tract infection.
Signs and symptoms
The symptoms of GBS vary a great deal from patient to patient, and so each can have a unique case history. The severity of symptoms can vary considerably between individuals. Symptoms can range from mild muscle weakness that resolves quickly, to complete muscle paralysis. Symptoms of Guillain-Barre get worse very quickly. It may take only a few hours to reach the most severe symptoms.
Guillain-Barre Syndrome frequently follows an antecedent flu-like illness within two weeks prior to the onset of neurological symptoms occurring in approximately 65% of cases. Fast onset of fatigue is a common symptom in patients with Guillain-Barré Syndrome and can be one of the most disabling symptoms. The clinical features range from asymptomatic to life threatening. GBS can be devastating because of its sudden and unexpected onset. GBS commences with progressive muscular weakness of extremities that may lead to paralysis. It spreads rapidly, ascending to involve the cranial nerves. Commences distally but spreads proximally and may involve the bulbar region and the diaphragm. Clinical features may include pain in the back and the legs and weakness beginning in the feet and legs and progressing upwards. Pain in the lower back, buttocks or thighs is common, and is often the earliest of symptoms. The respiratory muscles are affected in about half the cases, and this puts the patient in danger. The tendon reflexes are lost, but may still be preserved in the first hours of the illness. In certain cases, muscle weakness develops so quickly that muscle atrophy doesn’t occur, but hypotonia and areflexia do. Skeletal muscle weakness is the hallmark of most myopathies such as seen in Guillain-Barre syndrome (GBS).
Guillain-Barre syndrome (GBS) is usually easily identified with its typical presentation of ascending weakness and areflexia on examination. 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 dysthesias (numbness or tingling). As the weakness progresses upward, usually over periods of hours to days, the arms and facial muscles also become affected. GBS can develop over the course of hours or days, or it may take up to 3 to 4 weeks. Patients usually present a few days to a week after onset of symptoms. GBS may herald the onset of sleepiness in a subset of patients, presenting with mild to moderate fatigue, to 'extreme somnolence' (profound sleepiness), to "sleep drunkenness", such as seen in encephalitis, hydrocephalus, or swelling of the brain. The patient's voice may change because the vocal chords are affected. Speech may be unintelligible (slurred or whispery), because the various muscles required to form speech are weakened. Deafness is unusual but has been reported. Constipation is often a problem, due to the reduced activity of the intestines, change of diet, weakened stomach muscles that resist efforts by the patient to express the intestinal content. Frequently, the lower cranial nerves may be affected, leading to bulbar weakness (causing difficulty with eye movements, double vision), oropharyngeal dysphagia (difficulty with swallowing, drooling, and/or maintaining an open airway). Most patients require hospitalization and about 30% require ventilatory assistance.
The Guillain–Barré syndrome is an immune-mediated, acute, generalized peripheral neuropathy (eg, cranial nerve disorders) that usually causes diffuse (widely dispersed) weakness; peripheral nervous system pathology is visible by the lack of motor response to peripheral nerve stimulation. This syndrome (also called infectious polyneuritis, Landry-Guillain-Barré syndrome, and acute idiopathic polyneuritis) can occur at any age but is most common between ages 30 and 50; it affects both sexes equally. Guillain-Barre Syndrome has three stages. There is a progression phase over several days to several weeks, a plateau phase of similar duration, and then recovery over weeks to months. Onset: Mean 40 years; Seasonal: Higher frequency in Spring (March to May).
The classic Guillain-Barre syndrome is characterized by acute ascending and progressive neuropathy associated with muscle weakness and hyporeflexia. The clinical manifestations of this condition included areflexia in the cranial and spinal nerves as well as apnoea. Cranial nerves are involved in approximately 50% of cases. A special form of the syndrome is the Miller-Fisher syndrome in which signs of the cranial nerve deficit dominate in the first phase of the disease. In rare cases, acute quadriparesis may develop leading to coma and the absence of brainstem reflexes. At first the patient may become unresponsive to light with sluggishly reactive pupils which can rapidly progress to dilated or fixed pupils, usually the result of para sympatholytic or anticholinergic drugs.
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 over exercising. These pains are self-limited and should be treated with standard analgesics. Bladder dysfunction may occur in severe cases but should be transient. If spinal cord disease should be suspected, polyradiculoneuropathy should be considered. 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. Autonomic dysfunction in GBS is manifested as tachycardia and mild hypertension in the acute stage and cardiac arrhythmias associated with autonomic dysfunction are a recognised manifestation in Guillain-Barré syndrome. Marked orthostatic hypotension following paresis suggests an atypical clinical course of GBS. Notably, hypertension and tachycardia are also an indication of awareness and/or pain.
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. The brain and spinal cord are called the central nervous system (CNS), while nerves throughout the rest of the body are referred to as the peripheral nervous system (PNS). The basic building block of the nervous system is a nerve cell, or neurone. Irritated, inflamed or damaged nerves in the brain, spinal cord or body can cause headache and pain, which points to a causal connection to GBS. Severe headache in Guillain-Barré syndrome is rare; an initial episode of a headache, vomiting, fever and back and limb pain is usually followed by paralysis.
Many secondary complications may follow GBS that include dysautonomia, deep vein thrombosis, anemia, immobilization, and pain and sensory involvement (J. M., Meythaler, 1997; J. M. Meythaler, M. J. De Vivo, and W. C. Braswell, 1997). These medical complications have not been studied systematically, and the psychosocial complication of pain following GBS has certainly been overlooked in the literature. Adverse events may also accompany GBS which may include mild-to-moderate migraine attacks, chills, chest discomfort, fatigue, fever rarely, nausea, wheezing, dizziness, rashes, pains, and tenderness at injection site with increased risk of urticaria, pruritus, or petechiae and renal tubular necrosis in older volume-depleted diabetic patients.
Notably, Guillain-Barré syndrome (GBS) has become the major cause of acute neuromuscular paralysis in the Western world since the near eradication of polio. It is polio's closest mimic, manifesting as a cold or a mild flu, progressing to extreme fatigue, muscle weakness, debilitating joint pain, breathing difficulties and intolerance of cold. Feeling unusually cold is a common finding in many subjects with GBS. Conditions such as poliomyelitis and other neurological diseases such as Parkinson's disease that may mimic Guillain-Barré need to be ruled out before the diagnosis is made. Further, variants of GBS have emerged to make GBS a true syndrome rather than a specific disease. Certain viruses which exhibit strong neurotropism and generally thought to be associated with polio-like paralytic syndromes , also share a commonality with aseptic meningitis, including the Guillain Barré Syndrome. GBS can present with focal neurologic deficits or symptoms similar to Parkinsons disease, polio, and West Nile Virus. Several disorders have symptoms similar to those found in Guillain-Barré, so doctors must examine and question patients carefully before making a diagnosis.
GBS mimicking cerebral death has also been reported in the literature, illustrating an extreme polyneuropathy and there are also reports of other conditions that mimic brain death or that provide examples of the mistaken diagnosis of brain death. Fulminant cases of GBS have been reported in which a rapid deterioration evolves to a clinical state resembling “brain death”. Concerns have arisen with respect to the retrieval of organs for transplantation due to inconsistencies in the ethical declaration of brain death for confirming "complete cessation" of brain function and therefore brain death. Organ transplantation is premised on professional and public acceptance that the donor is dead and that the cessation of all brain function persists for an appropriate period of observation. There should be no central nervous system depressant or muscle relaxant drugs present and metabolic, endocrine or hypothermic causes of coma must first be excluded; physical signs may be obscured, or altered by the presence of sedative or muscle relaxant drugs, or due to anoxic ischemic insult. GBS is one of few neurological diseases whose clinical manifestations may be identical to those in brainstem death. Some similarities may also be found in other demyelinating syndromes.
Metabolic impairment causes demyelination or axonal degeneration. Axonal degeneration secondary to severe demyelination may mimic brain death.
Symptoms can range from mild muscle weakness that resolves quickly, to complete muscle paralysis. Subacute and chronic diffuse axonal types (diffuse axonal polyneuropathy) include most toxic and nutritional neuropathies, uremia, diabetes, hypothyroidism, human immunodeficiency virus (HIV) infection, Lyme disease, peroneoplastic disease, dysproteinemia, and amyloidosis. Metabolic processes may also cause diffuse weakness. Metabolic impairment causes demyelination or axonal degeneration.
Experimental parasitology, ie, testing parasite excretory-secretory products (ESP) as cures to treat Campylobacter jejuni infections is reported in the literature; may provide important clues to GBS triggers.
Acute inflammatory demyelinating polyradiculoneuropathy has a wide spectrum involving all the peripheral and cranial nerves; it can present with the same radiological features of brain tumors even on routine MR imaging., suggestive of growing large mass-like lesions.
Severe axonal GBS will show diffuse loss of sensory and motor responses with widespread active denervation. Axonal degeneration secondary to severe demyelination may mimic brain death. Glasgow Coma Scale should be cautiously applied as a prognostic measure in patients with metabolic or toxic CNS insults.
As a safeguard in determining brain death a number of tests need to be carried out every 6 hours and recorded, the physicians performing this determination must not be part of a transplantation team. In some cases, 48 to 72 hours is required to evaluate brain death and a repeat examinations are required to increase the diagnostic yield with observation up to 24 hours is sometime needed. The length of time between serial examinations to declare brain death varies marginally from 6 to 72 hours.
Although ascending paralysis is the most common form of spread in GBS, several variants of GBS are recognized. These disorders share similar patterns of evolution, recovery, symptom overlap, and probable immune-mediated pathogenesis.
- 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. In the initial stage of the disease the clinical features characteristic of the MFS may be observed, which is a special type of polyradicular and polyneural Guillaine-Barre syndrome involving mostly the cranial nerves. In its classic form the syndrome consists of external ophthalmoplegia, cerebellar ataxia, weakened reflexes with preserved consciousness and lack of damage to the pyramidal tracts. CT scans of some patients with this syndrome have shown structural damage to the brainstem in the form of decreased density; would show up this way whether the CT scan was done with contrast or not.
- Acute motor axonal neuropathy (AMAN) marked by diffuse weakness refers to the cases showing only motor symptoms; attacks motor nodes of Ranvier and is prevalent in China and Mexico. The disease may be seasonal and recovery is rapid. Anti-GD1a antibodies are present.
- Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but also affects sensory nerves with severe axonal damage. Other variants are notable for an axonal pattern of electrodiagnostic findings and axonal pathology with little inflammation. Recovery is slow and often incomplete.
- Also known as: Kussmaul-Landry syndrome, Landry's syndrome, Landry-Guillain-Barré syndrome, Landry-Kussmaul syndrome, Glanzmann-Saland syndrome (misnomer).
- Addendum Patients with the pharyngeal-cervical-brachial variant (PCB) of Guillain-Barre syndrome (GBS) have anti-GT1a IgG with or without GQ1b reactivity, whereas those with Miller Fisher syndrome (MFS) or Bickerstaff's brainstem encephalitis (BBE) have anti-GQ1b IgG antibodies which cross-react with GT1a. The presence of a common autoantibody (anti-GT1a IgG) and overlapping illnesses suggests that PCB is closely related not only to GBS but to MFS and BBE as well.
Rauschka, H., K. Jellinger, et al. (2003). "Guillain-Barre syndrome with marked pleocytosis or a significant proportion of polymorphonuclear granulocytes in the cerebrospinal fluid: neuropathological investigation of five cases and review of differential diagnoses." Eur J Neurol 10(5): 479-86 In summary "the presence of polymorphonuclear granulocytes does not rule out the diagnosis of GBS".
During the 1999 New York City West Nile virus (WNV) outbreak, 4 patients with profound muscle weakness, attributed to Guillain-Barré syndrome, were autopsied. These cases were the first deaths caused by WNV. Both Guillain-Barré disease and aseptic meningitis were diagnosed as polio during the US epidemics prior to 1957.
In September 2006 Guillian-Barré-type symptoms were observed on patients at Panama City's big public hospital. The patients, who showed signs of weakness or tingling sensation in the legs, did not develop the characteristic spread upwards of paralysis. The reason was found later in a poisoned medicine Lisinopril in which the glycerin excipient had been illegally replaced by diethylene glycol. (See Sources below)
The diagnosis is based on a clinical examination of the symptoms and their distribution and is dependent on the typical clinical findings, such as rapidly evolving flaccid paralysis, areflexia, absence of fever, and a likely inciting event. CSF and electrodiagnostics may be useful, but because of the acute nature of the disease, they may not become abnormal until the end of the first week. The diagnosis of Guillain-Barre syndrome is also based on clinical features and albumin cytological dissociation present in the spinal fluid. CBC shows leukocytosis and a shift to immature forms early in the illness. Cases of Guillain-Barre syndrome can have pleocytosis or CSF granulocytes.
Severe forms of GBS may result in urinary or fecal incontinence. A history of grossly bloody stools may be observed; fecal leukocytes are usually present. Toxic megacolon, pseudomembranous colitis, massive lower gastrointestinal hemorrhage, mesenteric adenitis, and appendicitis also have been described in immunocompetent patients with C jejuni infection.
GBS associated with marked migraine headache is a rare finding and one seldom reported in the literature, (see Brian Claman, Notable Patients below).
Review recent medical history; many cases are linked with recent bacterial or viral infections, vaccinations or surgeries. Infection with campylobacter, a bacteria found in undercooked food, especially poultry, may predispose Guillaine-Barre Syndrome.
A clinical feature of demyelination is muscle weakness without muscle atrophy.
Note any difficulty with facial muscles or movement, such as trouble moving your eyes, slow speech and problems chewing or swallowing.
Assess both sides of the body. Guillain-Barre Syndrome affects both sides, unlike a stroke. Strokes generally paralyze only one side.
Evaluate and report any changes in bladder or bowel function. Guillain-Barre Syndrome impacts the muscles that control bladder and intestinal function.
Pay attention to unusual or severe lower back pain, which can signal Guillain-Barre Syndrome.
Signs of hypotonia: Hypotonia is an abnormally severe loss of muscle tone. The muscles feel soft and doughy.
Weakening of diaphragm: Monitor for shallow or rapid breathing (breathing may be labored and difficult); patients with neuromuscular disorders have rapid shallow breathing secondary to severe muscle weakness or abnormal motor neuron function. Many people with the syndrome are temporarily placed on ventilators in order to breathe.
Gasping for breath - sleep apnea: GBS can freeze the breathing muscles with assault on muscle function resulting in episodic and paroxysmal disorders with progression. Snoring and sleep apnea are part of the same problem. Look at the shape of the neck. Adults and older adolescents having short thick necks are at greater risk of developing obstructive sleep apnoea. Aspiration and respiratory failure are major concerns.
GBS may induce a "locked-in" state of 'outer calm inner panic' due to a severely paralyzed motor function that patients are able to recall vividly and unpleasantly. The locked-in state involves damage to corticospinal and corticobulbar pathways in the basis pontis. GBS causes bilateral profound damage to these pathways with diffuse compromise to peripheral nerves.
Hypoventilation secondary to respiratory muscle weakness may be observed.
Dysautonomia, often causing profound swings in blood pressure (hypotension alternating with hypertension), are often conspicuous in patients with Guillain-Barré. There is even a panic factor marked by sudden onset of intense apprehension, fear, terror, or impending doom with awareness. In GBS, dysautonomia is usually acute and reversible.
Coagulopathy: blood clotting disorders, (blood thinners may be needed to prevent blood clots).
ECG may show heart problems in some cases.
NCV (nerve conduction velocity) shows nerve damage.
EMG tests the electrical activity in muscles. It may show that nerves do not react properly to stimulation.
Guillain-Barre Syndrome and its complications are unclear or arbitrary. Data contain little information on potential confounders, such as co-medication, including underlying diseases (eg, diabetes mellitus) and potentially confounding coincidental infections (eg, Campylobacter). Failure to recognize the separate variants may confound the clinical picture. Some of the same diseases that mimic radiographic findings also can confound the diagnostic interpretation of CT scans.
GBS may also develop secondary to untreated or inappropriately treated conditions (infections have severe systemic complications), drug side effect causes, "shotgun therapy" that inflicts collateral damage and drug interaction causes. CNS underlying disorders, poorly controlled insulin-dependent diabetes mellitus or cancer, encephalitis, meningitis, abscess , subdural hematoma, S.aureus endocarditis, spinal epidural haematoma, trauma, stroke, including acute intermittent porphyria in association with or which may mimic GBS suggest that infectious or metabolic triggers may also predispose and electrolyte imbalances may further confound the neurologic examination.
Transient diabetes has been observed in certain cases of Guillain-Barré syndrome. Hypokalemic periodic paralysis is one of the many disorders which, if left untreated may also mimic GBS. Several potential confounders, including diseases putatively associated with peripheral neuropathy; overlapping and/or underlying illness may confound the diagnosis of GBS. Early literature emphasised the high prevalence of gastrointestinal symptoms in patients with diabetes complicated by neuropathy. The reduction in the odds of neuropathy due to glycemic control is attenuated with increasing duration. Glycemic triggers may be prevented by maintaining appropriate blood glucose levels.
Drug intoxication is the most common cause of coma of rapid onset which may mimic brain death; examination can be marred by the effects of sedation. Metabolic derangement and endocrine crisis can also mimic brain death. More dramatic is the reversible Guillain–Barré syndrome involving all the peripheral and cranial nerves. The progression which can mimic brain death occurs over a period of days.
Obtundation in GBS: The term refers to a reduction in alertness and arousal in which these patients appear to be completely unresponsive. Although patients with GBS in the setting of preserved consciousness may be described as obtunded, patients may be fully lucid; ambiguities in deciding whether some individual patients are truly unconscious, in a vegitative state, or simply locked-in (implies fully preserved consciousness) cannot be diagnosed on the basis of a paltry CT.
Influence of tranquillisers (drugs that slow normal brain function), sedatives (drugs that have a depressant effect on the central nervous), narcotics (drugs that induce sleep), and neuroleptics (drugs that block dopamine receptors).
- Leukocytosis - early
- CSF - typical CSF findings include an elevated protein level (100 - 1000 mg/dL) without an accompanying pleocytosis (increased cell count).
- Coagulation - GBS presents with elevated fibrinogen and is typically elevated at presentation. CSF usually clots in the presence of increased fibrinogen.
- Acute phase reactant - marker of inflammation. Falls as syndrome resolves with treatment.
- pleocytosis - Sustained pleocytosis may indicate an alternative diagnosis such as infection. The diagnosis is confirmed by the presence of Albuminocytological dissociation in the CSF. CSF - (50 mg/dl - 100 mg/dl) - this reflects the widespread inflammation of the nerve roots; the protein increase usually begins after the 10th day of the neurologic illness and peaks at 4 wk-6wk; the increase parallels the clinical severity
- Complete blood count - Normal cell count (90% of patients); slightly increased mononuclear cells (10% of patients)
- Electrophysiological studies show demyelinating signs with decreased conduction velocity and normal amplitude of motor potentials in Guillain-Barré syndrome versus normal conduction velocity and reduced amplitude of motor potentials in axonal polyneuropathy.
- 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.
- Progressive weakness of 2 or more limbs due to neuropathy
- Disease course < 4 weeks
- Exclusion of other causes (see below)
- relatively symmetric weakness
- mild sensory involvement
- facial nerve or other cranial nerve involvement
- absence of fever
- typical CSF findings
- electrophysiologic evidence of demyelination
One of the clinical features of GBS is elevated protein levels in the CSF fluid evinced on the spinal tap.
- 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
The possibility of West Nile Virus (WNV) meningoencephalitis should be entertained in the setting of suspected Guillain-Barré syndrome, as management of the two entities is markedly divergent.
A noteworthy diagnostic dilemma which may mimic GBS is botulism, in which the differential diagnosis includes organophosphate ingestion, tick paralysis, brainstem tumor, poliomyelitis, and myasthenia gravis.
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 400 mg/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 (.4 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) 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. There would also be a doctor, nurse and perhaps a speech trainer involved, depending on the needs of the patient. This team contribute with their knowledge to guide the patient towards his goal, 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 own home and attend necessary training as needed.
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 worse 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).
About 70 to 80% of patients make a good recovery with little or no residual neurologic signs. Others have varying degrees of distal muscle wasting and weakness.
Demyelination as evidenced by slow condution velocity and conduction block are reversible features of the disease.
The disease was first described by the French physician Jean Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré and Andre Strohl discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.
Evidence of West Nile encephalitis virus (polio-like) infection has been documented in most states of the continental United States within a short period of its first introduction in 1999. Health care providers are mostly aware of the usual presentations of this disease, eg, aseptic meningitis, encephalitis and Guillain-Barré syndrome.
Central nervous system complications of many pharma-agents include aseptic meningitis and Guillain-Barré syndrome.
GBS is also known as acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio and Landry's ascending paralysis.
The antigenic similarity between specific regions (terminal tetrasaccharide) of lipopolysaccharide (LPS) of C jejuni and human gangliosides (GM1) led to the concept of "molecular mimicry."
- Joseph Heller, author of Catch-22. His experiences with the illness make up a large portion of his non-fiction book No Laughing Matter.
- Andy Griffith, actor on Andy Griffith Show, and Matlock. He contracted Guillain-Barré in 1983. 
- Rachel Chagall, actress, contracted GBS in 1982. In 1987 she portrayed Gabriela Brimmer, a notable disabilities activist.
- Franklin D. Roosevelt, U.S. president. In 2003, a peer-reviewed study found that it was more likely that Roosevelt's paralytic illness was actually Guillain-Barré syndrome, not poliomyelitis. See separate article on Franklin D. Roosevelt's paralytic illness.
- Scott Klopfenstein, backup vocals, trumpet player, 2nd guitar, and keyboard player for Reel Big Fish.
- Tony Benn Retired cabinet minister
- Morten Wieghorst, former footballer, most known for playing for Celtic FC
- Markus Babbel, ex-Bayern Munich & Liverpool footballer and German international.
- Michael P. Moran, an American actor.
- Jack Rohan, an American basketball player.
- Jamie Brooks, an English football player.
In Popular Culture
Guillain-Barré is recognized today in the media on the show House. When a patient on this popular American television program expresses certain symptoms in the lower legs, the characters—Dr. Chase, Cameron and Foreman—often diagnose him with Guillain-Barré. The brief statements add to the entertainment value of the show while also spreading awareness of the illness. A notable fictional patient is Michael Tolliver in the book Tales of the City, his disease and fear of the paralysis spreading to his lungs forming a major part of the plot.
Genova Diagnostics: May 12, 1999, Volume 4, Number 9 "We are too much accustomed to attribute to a single cause that which is the product of several, and the majority of our controversies come from that." Baron Justus von Liebig (1803-73).
- The New York Times May 6 2007, Article by Walt Bogdanich and Jake Hooker.
- Journal of Neurosurgical Anesthesiology - Fulltext: Volume 16(4 - Abstract: colon; Severe headache in Guillain-Barre syndrome...
- All About Guillain-Barré Syndrome, S. Marcussen. Symptoms & the way patients experience them.
- eMedicine - Serum Sickness : Article by Hassan M Alissa, MD  Neurologic complications.
- eMedicine - Guillain-Barre Syndrome : Article by Heather Rachel Davids, MD  Clinical: Section 3 of 10.
- Brain Death: C.J. Doig MD MSc, E. Burges, MD,  Inconsistencies in ethical declaration.
- Prodromal Malaise in GBS:  GP notebook tracker.
- Trip Database  Evidence Based Medicine (EBM) Search GBS
- PubMed  Fulminant Guillain-Barré syndrome mimicking cerebral death: case report and literature review.
- Dysautonomia in GBS:  When the Autonomic Nervous System or Body's Autopilot Fails
- Campylobacter jejuni  in Waterborne Protozoa.
- ^ Goldman, AS et al, What was the cause of Franklin Delano Roosevelt's paralytic illness?. J Med Biogr. 11: 232-240 (2003)
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