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Guillain Barre Syndrome(ghee-yan-bar syndrome) is a reactive self limited auto immune disease in which the body's immune system attacks part of the peripheral nervous system and which presents as acute generalized weakness.
It is referred to as syndrome because it represents a broad group of demyelinating inflammatory poly radiculo-neuropathies.
GBS can also be classified into following categories:
1. AIDP (Acute inflammatory demyelinating polyneuropathy)
2. Axonal can be further classified into: i. Acute motor axonal neuropathy (AMAN) ii. Acute motor sensory axonal neuropathy (AMSAN) 3. Miller Fisher Syndrome
GBS is a classic lower motor neuron disorder. It occurs as an auto immune response following vaccination, viral infection or respiratory infection.
Epidemiology affects all ages with a male preponderance. Children are less affected than adults.
The pathophysiology of GBS is complex the body's immune system begins to attack the body itself, as it involves autoimmune responses. The immune responses causes a cross reaction with the neural tissue. When myelin is destroyed, destruction is accompanied by inflammation. These acute inflammatory lesions are present within several days of the onset of symptoms.
Nerve conduction is slowed and may be blocked completely. Even though the Schwann cells that produce myelin in the peripheral nervous system are destroyed, the axons are left intact in all but the most severe cases. After 2-3 weeks of demyelination, the Schwann cells begin to proliferate, inflammation subsides, and re-myelination begins.
While GBS is the most common cause of acute paralysis, the exact pathogenesis is still unclear. The progression of demyelination appears different in AMAN type of GBS versus AIDP type. Nadir is the point of greatest severity and patients with AMAN type reach it earlier. =
Incidence -The annual incidence of GBS in the USA is 1.2-3 per 100,000 inhabitants; GBS has been reported throughout the world.[62, 63] Most studies show annual incidence figures similar to those in the United States.
Age: the annual mean rate of hospitalizations in the United States related to GBS increases with age, being 1.5 cases per 100,000 population in children under 15 years of age, and peaking at 8.6 cases per 100,000 population in 70-79 year olds.
Gender : GBS has a male-to-female ratio of 1.5:1.
The typical patient with GBS) presents 2-4 weeks following a relatively benign gastrointestinal or respiratory illness with complaints of finger dysesthesias and proximal muscle weakness of the lower limbs. The weakness may progress over hours to days to involve the arms, trunk, cranial nerves, and muscles of respiration. Variants of GBS may present as pure motor dysfunction or acute dysautonomia. a. “Typical” GBS is an acute, predominantly motor neuropathy involving distal limb paresthesias, relatively symmetric leg weakness, and frequent gait ataxia.'
i. Most cases will have subsequent arm weakness, and possibly weakness of facial, ocular, and oropharyngeal muscles.
b.'Weakness is always bilateral, although some asymmetry in onset and severity is common.
i. Proximal muscle weakness very frequent, especially initially, with subsequent distal arm and leg weakness.
ii. GBS with a descending pattern of weakness seen in 14% cases; onset initially with cranial nerve or arm muscle weakness,followed by leg weakness.
iii. In 1/3 of cases, the degree of weakness in the arms and legs is roughly equal.
c. Reduced or absent reflexes characterize GBS.
i. Early loss of reflexes may be due to desynchronization of afferent impulses in reflex arc due to non-uniform demyelination.
ii. About 70% of patients present with loss of reflexes; less than 5% retained all reflexes during the illness;
iii. The presence of intact reflexes should suggest an alternative diagnosis other than GBS.
d. Sensory disturbance
i. >50% will present with symmetric distal limb paresthesias, before clinically evident limb weakness. Early finger paresthesias suggest a patchy process, unlike the pattern seen with distal axonopathies.
ii. paresthesias of trunk or face unusual, but sensory loss over the trunk frequent and a psuedolevel may be evident
iii. beware if definite sensory level present as this may suggest structural cord disease
i. occurs in about 65% of cases
ii. more frequent in patients with severe paralysis and ventilator difficulties but may develop in mild cases.
iii. Most common manifestations include cardiac dysfunction such as sinus tachycardia, sinus bradycardia, sinus arrest and other supraventricular arrhythmias, paroxysmal hypertension, and hypotension (especially postural),
iv. ICU monitoring necessary because of possible cardiac complications.
v. Other features: ileus, urinary retention (1/4 cases), inappropriate ADH, altered sweating, mild orthostatic hypotension.
f.Cranial nerve involvement is observed in 45-75% of patients with GBS. Cranial nerves III-VII and IX-XII may be affected. Common complaints include:: Facial Palsy
Facial and oropharyngeal weakness usually appears after the trunk and limbs are affected. The Miller-Fisher variant of GBS is unique in that this subtype begins with cranial nerve deficits.
Antecedent Illness:Up to two thirds of patients with GBS report an antecedent illness or event 1-3 weeks prior to the onset of weakness. Upper respiratory and gastrointestinal illnesses are the most commonly reported conditions. Symptoms generally have resolved by the time the patient presents with the neurological condition.
GBS Symptom progression : The mean time to the peak of symptoms is 12 days (from 1st neurological symptoms), with 98% of patients reaching peak by 4 weeks. A plateau phase of persistent, unchanging symptoms then ensues, followed days later by gradual symptom improvement. Recovery usually begins 2-4 weeks after the progression ceases. The mean time to clinical recovery is 200 days.
a. Acute peripheral neuropathies
i. Toxic: thallium, arsenic, lead, n-hexane, organophosphate
ii. Drugs: amiodarone, perhexiline, gold
v. Systemic vasculitis
viii. Tick paralysis
ix. Critical illness polyneuropathy
b. Disorders of Neuromuscular Transmission
ii. Myasthenia gravis
c. Central Nervous System Disorders
i. Basilar artery occlusion
ii. Acute cervical transverse myelitis
a. Cerebrospinal fluid investigation : It will elevated at some stage of the illness but remains normal during the first 10 days. There may be lymphocytosis (> 50000000 cells/L). b. Electrophysiological studies : it includes nerve conduction studies and electromyography. They are normal in the early stages but show typical changes after a week or so with conduction block and multifocal motor slowing, sometimes most evident proximally as delayed F-waves.
The only way to classify a patient as having the axonal or nanaxonal type is electrodiagnostically. c. Further investigative procedures can be undertaken to identify an underlying cause
i. Chest X-ray , stool culture and appropriate immunological tests to rule out the presence of cytomegalovirus or mycoplasma
ii. Antibodies to the ganglioside GQ1b for miller fisher variant. d. MRI E. Lumbar Puncture: Most, but not all, patients with GBS have an elevated CSF protein level (>400 mg/L), with normal CSF cell counts. Elevated or rising protein levels on serial lumbar punctures and 10 or fewer mononuclear cells/mm3 strongly support the diagnosis.
Management / Interventions<span />
There is no known cure for Guillain-Barré syndrome. However, there are therapies that 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.
Medical Management :
The mainstay of medical management of patients with GBS is
b) Intravenous immunoglobulins ( I.V.I.G)
In plasmapheresis, blood is removed from the body, the red and white blood cells are separated from the plasma and only the blood cells are returned to the patient. It is thought that removing the plasma eliminates some of the immune factors that are responsible for the disease progression. Plasmapheresis helps in following ways:
a) Reducing the length of the illness
b) Shortened time on mechanical ventilation
c) Early ambulation
In I.V.I.G, immunoglobulins are given intravenously which shows a positive impact on the speed of recovery. But it has been shown to be less effective than plasmapheresis.
Further medical management can be done according to the symptoms and the complications :
a. Supportive Care
i. ICU monitoring
ii. Basic medical management often determines mortality and morbidity.
b. Ventilatory Support
i. Atelectasis leads to hypoxia.
ii. Hyper-carbia later finding; arterial blood cases may be misleading.
iii. Vidal capacity, tidal volume and negative inspiratory force are best indicators of diaphragmatic function.
iv. Progressive decline of these functions indicate an impending need or ventilatory assistance.
1. mechanical ventilation usually required if VC drops below about 14 ml/kg; ultimate risk depending on age, presence of accompanying lung disease, aspiration risk, and assessment of respiratory muscle fatigue.
v. Atelectasis treated initially by incentive spirometry, frequent suctioning, and chest physiotherapy to mobilize secretions.
vi. Intubation may be necessary in patients with substantial oro-pharyngeal dysfunction to prevent aspiration.
vii. Tracheostomy may be needed in patients intubated for 2 weeks who do not show improvement.
c. Autonomic dysfunction
i. Autonomic dysfunction may be self-limited; do not over-treat.
ii. Sustained hypertension managed by angiotensin-converting enzyme inhibitor or beta blocking agent. Use short acting intravenous medication for labile hypertension requiring immediate therapy.
iii. Postural hypotension treated with fluid bolus or positioning.
iv. Urinary difficulties may require intermittent catheterization.
d. Nosocomial infections usually involve pulmonary and urinary tracts.
i. Occasionally central venous catheters become infected.
ii. Antibiotic therapy should be reserved for those patients showing clinical infection rather than colonization of fluid or sputum specimens.
e. Venous thrombosis due to immobilization poses great risk of thromboembolism.
Aims of the treatment are to
1. Maintain clear airways
2. Prevent lung infection
3. Maintain anatomical joint range
4. Support joint in functional position to minimize damage or deformity
5. Prevention of pressure sores
6. Maintain peripheral circulation
7. Provide psychological support for the patient and relatives. 1. Maintenance of clear airway & prevention of lung infection :
a) The patients breathing will be assisted by intermittent positive pressure ventilation (IPPV) via a cuffed tracheostomy tube.
b) Posturally drain areas of lung tissues, 2-hourly turning into supine or side lying positions.
c) A suction catheter is used to remove secretions from respective passage until the cough reflex re-appears.
d) Manual techniques like vibration with/ without over pressure.
e) 2-4 litre anesthetic bag can be used to enhance chest expansion. Therefore , 2 people are necessary for this technique, one to squeeze the bag and another to apply chest manipulation.
f) Rib springing to stimulate cough.
g) After the removal of ventilator and adequate expansion, effective coughing must be taught to the patient
h) As neurons recover, active assisted or active breathing exercises may commence with good amount of relaxing time. 2. To maintain normal joint movement :
Gentle passive movements through full ROM at least three times a day especially at hip , shoulder, wrist, ankle, feet. 3. Support joints :
Use of light splints(eg. using PLASTAZOTE) may be required for the following purpose listed below:
a) Support the peripheral joints in comfortable and functional position during flaccid paralysis.
b) To prevent abnormal movements.
c) To stabilize patients using sandbags, pillows. 4. Prevention of pressure sores :
2- hourly change in patients position from supine to side lying. If the sores have developed then UVR or ice cube massage to enhance healing. 5. Maintenance of circulation:
a) Passive movements
b) Effleurage massage to lower limbs. 6. Relief of pain:
a) Transcutaneous electrical nerve stimulation
b) Massage with passive ROM
c) Patient can demonstrate increased sensitivity to light touch, a cradle can be used to keep the bed sheet away from the skin.
d) Low pressure wrapping or snug fitting garments can provide a way to avoid light touch.
e) Reassurance and explanation of what to expect can help in alleviation of anxiety that could compound the pain.
Exercises to be prescribed to the patient should be started with low repetitions and short, frequent bouts of exercises matched to the patients muscular strength. According to Bensman (1970), the following four guidelines are to be followed for prescription of exercises:
a) Use short periods of non-fatiguing exercises matched to the patients strength.
b) Progression of the exercise should be done only if the patient improves or if there is no deterioration in status after a week.
c) Return the patient to bed rest if a decrease in muscle strength or function occurs.
d) The objective should be directed towards not only at improving function but also in improving strength. ===== Long Term Effects ===== *A study of 35 patients (27 with classic GBS and 8 with acute motor axonal neuropathy [AMAN]), reported GBS-related deficits included: neuropathic pain requiring medication therapy (28 patients) *foot drop necessitating ankle-foot orthosis (AFO) use (21 patients) *locomotion difficulties requiring assistive devices (30 patients) *At 1-year follow-up, the authors found continued foot drop in 12 of the AFO patients. However, significant overall functional recovery had occurred within the general cohort.
Guillain-Barré syndrome can be a devastating disorder because of its sudden and unexpected onset. In addition, recovery is not necessarily quick. As noted above, patients usually reach the point of greatest weakness or paralysis days or weeks after the first symptoms occur. Symptoms then stabilize at this level for a period of days, weeks, or, sometimes, months. 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-Barré 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.
Guillain-Barré syndrome patients face not only physical difficulties, but emotionally painful periods as well. It is often extremely difficult for patients to adjust to sudden paralysis and dependence on others for help with routine daily activities. Patients sometimes need psychological counseling to help them adapt.
a) Pain and the Guillain-Barre ́ syndrome in children under 6 years old Dang Khoa Nguyen, MD Stacey Agenarioti-Bélanger, MD, Michel Vanasse, MD, FRCP(C) http://www.sciencedirect.com/science/article/pii/S0022347699702970 b) Guillain barre syndrome pathophysiology http://www.youtube.com/watch?v=hnvw2zFd-uM c) Hand therapy for guillain barre syndrome patients http://www.youtube.com/watch?v=u4NTqOPBkrU d) Campylobacter jejuni Infection and Guillain–Barré Syndrome Jeremy H. Rees, Ph.D., M.R.C.P., Sara E. Soudain, B.Sc., Norman A. Gregson, Ph.D., and Richard A.C. Hughes, M.D. N Engl J Med 1995; 333:1374-1379November 23, 1995DOI: 10.1056/NEJM199511233332102 http://www.nejm.org/doi/full/10.1056/NEJM199511233332102
e) Karni Y, Archdeacon L, Mills KR, et al. Clinical assessment and physiotherapy in Guillain-Barre syndrome . Physiotherapy 70(8): 288-292, 1984. f) Hahn AF. Guillain-Barre syndrome. Lancet 352(9128):635-641, 1998. g) Hiraga A, Mori M, Ogawara K, et al. Differences in patterns of progression in demyelinating and axonal Guillain-Barre syndromes. Neurology 61(4);471-474, 2003.
Recent Related Research (from Pubmed)
- Effect of rehabilitation on mortality of patients with Guillain-Barre Syndrome: a propensity-matched analysis using nationwide database.
- Clinico-electrophysiological profile and predictors of functional outcome in Guillain-Barre syndrome (GBS).
- ↑ Guillain-Barré Syndrome Fact Sheet http://www.ninds.nih.gov/disorders/gbs/detail_gbs.htm
- ↑ http://www.ninds.nih.gov/disorders/gbs/detail_gbs.htm
- ↑ Alshekhlee A, Hussain Z, Sultan B, Katirji B. Guillain-Barré syndrome: incidence and mortality rates in US hospitals. Neurology. Apr 29 2008;70(18):1608-13
- ↑ Kushnir M, Klein C, Pollak L, Rabey JM. Evolving pattern of Guillain-Barre syndrome in a community hospital in Israel. Acta Neurol Scand. May 2008;117(5):347-50
- ↑ Prevots DR, Sutter RW. Assessment of Guillain-Barré syndrome mortality and morbidity in the United States: implications for acute flaccid paralysis surveillance. J Infect Dis. Feb 1997;175 Suppl 1:S151-5
- ↑ Lo YL. Clinical and immunological spectrum of the Miller Fisher syndrome. Muscle Nerve. Nov 2007;36(5):615-27
- ↑ Nelson L, Gormley R, Riddle MS, Tribble DR, Porter CK. The epidemiology of Guillain-Barré Syndrome in U.S. military personnel: a case-control study. BMC Res Notes. Aug 26 2009;2:171
- ↑ Hughes RA, Rees JH. Clinical and epidemiologic features of Guillain-Barré syndrome. J Infect Dis. Dec 1997;176 Suppl 2:S92-8.
- ↑ El Mhandi L, Calmels P, Camdessanché JP, Gautheron V, Féasson L. Muscle strength recovery in treated Guillain-Barré syndrome: a prospective study for the first 18 months after onset. Am J Phys Med Rehabil. Sep 2007;86(9):716-24
- ↑ Guillain‐Barré syndrome Management of respiratory failure Allan H. Ropper, MD and Susan M. Kehne, MD http://www.neurology.org/content/35/11/1662
- ↑ Gupta A, Taly AB, Srivastava A, Murali T. Guillain-Barre Syndrome – rehabilitation outcome, residual deficits and requirement of lower limb orthosis for locomotion at 1 year follow-up. Disabil Rehabil. 2010;32(23):1897-902
- ↑ http://www.ninds.nih.gov/disorders/gbs/detail_gbs.htm