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Guillain-Barré syndrome
Classification and external resources
ICD-10G61.0
ICD-9357.0
DiseasesDB5465
eMedicineemerg/222 
MeSHD020275

Guillain-Barré syndrome (GBS) (in French pronounced [ɡilɛ̃ baˈʁe][1], in English pronounced /ˈɡiːlæn ˈbɑreɪ/[2], /ɡiːˈæn bəˈreɪ/,[3] etc. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings G00-G99 - Diseases of the Nervous system (G00-G09 Inflammatory diseases of the Central nervous system ( Bacterial meningitis The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books [4]) is an acute, autoimmune, polyradiculoneuropathy affecting the peripheral nervous system, usually triggered by an acute infectious process. Neuropathy is a medical term usually short for Peripheral neuropathy. The peripheral nervous system ( PNS) resides or extends outside the Central nervous system (CNS which consists of the Brain and Spinal cord. It is included in the wider group of peripheral neuropathies. Peripheral neuropathy is the term for damage to Nerves of the Peripheral nervous system, which may be caused either by diseases of the Nerve or from the 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. Plasmapheresis (from the Greek plasma, something molded and apheresis, taking away is the removal treatment and return of (components of Blood Antibodies (also known as immunoglobulins, abbreviated Ig) are Gamma globulin Proteins that are found in Blood or other Bodily However, death may occur if severe pulmonary complications and dysautonomia are present. Dysautonomia is any disease or malfunction of the Autonomic nervous system.

Contents

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). Molecular mimicry is defined as the theoretical possibility that sequence similarities between foreign and self-peptides are sufficient enough to result in the cross-activation of autoreactive 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. Ganglioside is a compound composed of a Glycosphingolipid ( Ceramide and Oligosaccharide) with one or more Sialic acids (AKA N-acetylneuraminic Nodes of Ranvier are known as the gaps (about 1 micrometer in diameter formed between myelin sheath cells along axons or nerve fibers 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. Campylobacter jejuni is a Species of curved Rod-shaped, non-spore forming Gram-negative Microaerophilic, Bacteria 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. Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues Myelin is an electrically-insulating Dielectric Phospholipid layer that surrounds only the Axons of many Neurons It is an outgrowth Paralysed redirects here For other uses see xx Paralysed (disambiguation Paralysis is the complete loss of Muscle function

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. Myelin is an electrically-insulating Dielectric Phospholipid layer that surrounds only the Axons of many Neurons It is an outgrowth An axon or nerve fiber is a long slender projectionof a nerve cell or Neuron, that conducts electrical impulses away from the neuron's Cell

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). Dysesthesia is defined as an unpleasant abnormal sensation It is caused by lesions of the nervous system peripheral or central and it involves abnormal sensations whether spontaneous 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. Cranial nerves are Nerves that emerge directly from the Brain stem in contrast to Spinal nerves which emerge from segments of the Spinal cord. 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. Proprioception (ˌproʊpriːəˈsɛpʃən PRO -pree-o-SEP-shun from Latin proprius, meaning "one's own" and perception is the Sense 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. Orthostatic hypotension (also known as postural hypotension, and colloquially as head rush or a dizzy spell) is a form of Hypotension in which

The symptoms are similar to those for progressive inflammatory neuropathy. Progressive inflammatory neuropathy ( PIN) is a Disease that was identified in a report released on January 31 2008 by the Centers for Disease Control and Prevention [5]

Clinical variants

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

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.


Diagnostic criteria

Required

Supportive

Differential diagnosis

Treatment

Supportive care with monitoring of all vital functions is the cornerstone of successful management in the acute patient. Botulism ( Latin, botulus, "sausage" is a rare but serious Paralytic illness caused by Botulin Toxin. Diphtheria ( Greek διφθερα ( diphthera)—“pair of leather scrolls" is an upper respiratory tract illness characterized by sore Lyme disease, or borreliosis, is an Emerging infectious disease caused by at least three Species of Bacteria belonging to the Genus Porphyrias are a group of inherited or acquired disorders of certain Enzymes in the Heme biosynthetic pathway (also called Porphyrin pathway Poliomyelitis, often called polio or infantile paralysis, is an acute viral Infectious disease spread from person to person primarily via Myasthenia gravis (literally "serious muscle-weakness" from Greek μύς "muscle" "weakness" and Latin gravis An organophosphate (sometimes abbreviated OP) is the general name for Esters of Phosphoric acid. Poison hemlock redirects here For other uses of "hemlock" see Hemlock. Thallium (ˈθæliəm is a Chemical element with the symbol Tl and Atomic number 81 Arsenic (ˈɑrsənɪk is a Chemical element that has the symbol As and Atomic number of 33 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. In Medicine, intubation refers to the placement of a tube into an external or internal orifice of the body Vital capacity is the maximum volume of air that a person can exhale after maximum Inhalation.

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. Antibodies (also known as immunoglobulins, abbreviated Ig) are Gamma globulin Proteins that are found in Blood or other Bodily Plasmapheresis (from the Greek plasma, something molded and apheresis, taking away is the removal treatment and return of (components of Blood 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. Glucocorticoids (GC are a class of Steroid hormones characterised by an ability to bind with the glucocorticoid receptor ( GR) and trigger similar effects 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. Activities of daily living ( ADLs) are "the things we normally do in daily living including any daily activity we perform for self-care (such as feeding ourselves bathing 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.


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). Chronic Inflammatory Demyelinating Polyneuropathy (CIDP is an acquired immune-mediated inflammatory disorder of the Peripheral nervous system but often can have The quickest known recovery from GBS was Robert Bernatz of Newport Beach, CA who in 1998 was released from a Hoag Memorial Hospital in just ten days from the onset of his symptoms and was running six miles at the end of one month. His primary treatment was the relatively new process of plasmapheresis. Plasmapheresis (from the Greek plasma, something molded and apheresis, taking away is the removal treatment and return of (components of Blood

History

The disease was first described by the French physician Jean Landry in 1859. This article is about the country For a topic outline on this subject see List of basic France topics. Jean Baptiste Octave Landry de Thézillat (b October 10, 1826 &ndash d Year 1859 ( MDCCCLIX) was a Common year starting on Saturday (link will display the full calendar of the Gregorian calendar (or a Common 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. Year 1916 ( MCMXVI) was a Leap year starting on Saturday (link will display the full calendar of the Gregorian calendar (or a Leap year Georges Charles Guillain ( March 3, 1876 - June 29, 1961) was a French Neurologist. Jean Alexandre Barré ( May 25, 1880 – April 26, 1967) French Neurologist, worked in 1916 on the identification of the André Strohl ( March 20, 1887 - March 10, 1977) was a French Physiologist who was a native of Poitiers. [9]

GBS is also known as acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio and Landry's ascending paralysis.

Notable sufferers

References

  1. ^ John Wells’s phonetic blog, Archive 15-28 February 2007[1]
  2. ^ Recommended by the GBS Support Group[2]
  3. ^ "Guillain-Barre Syndrome. " Dictionary. com Unabridged (v 1. 1). Random House, Inc. [3] (accessed: December 27, 2007).
  4. ^ 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]. A nasal vowel is a Vowel that is produced with a lowering of the velum so that air escapes both through Nose as well as the Mouth. A nasal vowel is a Vowel that is produced with a lowering of the velum so that air escapes both through Nose as well as the Mouth. A nasal consonant (also called nasal stop or nasal continuant) is produced with a lowered velum in the mouth allowing air to escape freely through the
  5. ^ David Brown (2008-02-04). 2008 ( MMVIII) is the current year in accordance with the Gregorian calendar, a Leap year that started on Tuesday of the Common Events 211 - Roman Emperor Septimius Severus dies leaving the Roman Empire in the hands of his two quarrelsome sons Inhaling Pig Brains May Be Cause of New Illness. The Washington Post. The Washington Post is the largest and most circulated Newspaper in Washington D Retrieved on 2008-02-04. 2008 ( MMVIII) is the current year in accordance with the Gregorian calendar, a Leap year that started on Tuesday of the Common Events 211 - Roman Emperor Septimius Severus dies leaving the Roman Empire in the hands of his two quarrelsome sons
  6. ^ McKhann GM, Cornblath DR, Ho T, Li CY, Bai AY, Wu HS, Yei QF, Zhang WC, Zhaori Z, Jiang Z, et al. Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China. Lancet 1991;338:593-7
  7. ^ Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW, Asbury AK, Blaser MJ, McKhann GM. Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain 1995;118:597-605.
  8. ^ Griffin JW, Li CY, Ho TW, Xue P, Macko C, Gao CY, Yang C, Tian M, Mishu B, Cornblath DR, et al. Guillain-Barré syndrome in northern China: The spectrum of neuropathological changes in clinically defined cases. Brain 1995;118:577-95
  9. ^ Guillain-Barré-Strohl syndrome and Miller Fisher's syndrome at Who Named It
  10. ^ Goldman, AS et al, What was the cause of Franklin Delano Roosevelt's paralytic illness?. Who Named It? is an English-language dictionary of medical eponyms and the People associated with their identification J Med Biogr. 11: 232-240 (2003)
  11. ^ Serge Payer Foundation, Serge Payer Foundation Mission.

External links


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