Neurology Assignment On

Guillain Barre Syndrome

Submitted By:
Amit Kochhar B.O.T.-4th year 18th Batch Roll No. 1


the patient is almost totally paralyzed. although some continue to have a certain degree of weakness. Such a patient is often put on a respirator to assist with breathing and is watched closely for problems such as an abnormal heart beat. or less frequently axonal degeneration affecting the spinal roots and peripheral nerves. and high or low blood pressure. The syndrome is rare. and occasionally the cranial nerves. These conditions produce acute and diffuse demyelination or conduction block. however. blood clots. It can strike at any age and both sexes are equally prone to the disorder. infections.potentially interfering with breathing and. They are usually post-infective and recover spontaneously. In many instances the weakness and abnormal sensations spread to the arms and upper body. These symptoms can increase in intensity until certain muscles cannot be used at all and. and post-infective polyneuritis. Guillain-Barré syndrome is a disorder in which the body's immune system attacks part of the peripheral nervous system. at times. . when severe. Landry Guillain Barré Strohl syndrome. In these cases the disorder is life threatening . with blood pressure or heart rate and is considered a medical emergency. Most patients. acute infectious polyneuritis.The term Guillain Barré syndrome (GBS) tends to be used interchangeably with acute idiopathic demyelinating polyneuropathy. recover from even the most severe cases of Guillain-Barré syndrome. Other names used for the condition have included: acute post-infective polyradiculoneuropathy. Guillain-Barré syndrome can affect anybody. The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs.

After the first clinical manifestations of the disease.however. Occasionally surgery or vaccinations will trigger the syndrome. afflicting only about one person in 100.000. 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. or weeks. the symptoms can progress over the course of hours. Usually GuillainBarré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection. days.

measurement of the p24 capsid antigen proving the underlying infection. and recovery can be markedly slow. Forms of Guillain Barré syndrome precipitated by both campylobacter and cytomegalovirus show delayed recovery compared to cases unassociated with these two infections. Campylobacter jejuni infection is associated with preceding diarrhoeal illness in 70 per cent. the electrophysiology often points to axonal dysfunction rather than demyelination. Preceding Campylobacter jejuni infections can evoke Guillain Barré syndrome even if there has been prompt treatment with antibiotics. By contrast. Unusual forms of acute polyneuritis may occur following campylobacter infection. Cytomegalovirus and campylobacter infections precipitate differing forms of Guillain Barré syndrome. including variants with ophthalmoplegia. . stool culture may be positive and serum IgM antibodies detected. When Campylobacter jejuni enteritis has precipitated Guillain Barré syndrome. and significant sensory involvement. The Guillain Barré syndrome may accompany primary infection with HIV at a stage before viral antibodies are detectable in the serum. Epstein Barr virus (10 per cent). That associated with cytomegalovirus tends to occur in younger patients. human immunodeficiency virus (HIV).Antecedent infections Over half of Guillain Barré syndrome patients experience symptoms of viral respiratory or gastrointestinal infections during the 1 3 weeks prior to the onset of neurological symptoms. Cytomegalovirus (13 per cent) and Campylobacter jejuni (in approximately 30 per cent) are the most common. a pure motor disorder (AMAN) is common. with a high occurrence of respiratory muscle weakness. Mycoplasma pneumoniae (5 per cent). Serological studies have implicated a wide range of infective agents. and childhood exanthems are also reported. cranial nerve involvement.

and is occasionally seen after renal transplantation from a cytomegaloviruspositive donor. usually Hodgkin's disease. It can appear in patients already being treated with substantial doses of steroids. and after bone-marrow or hepatic engraftment.Occasionally the Guillain Barré syndrome may be associated with underlying lymphoma. .

and the term is often used synonymously with GBS.Classification Six different subtypes of Guillain Barré syndrome exist:      Acute inflammatory demyelinating polyneuropathy (AIDP) is the most common form of GBS. proceeding in the reverse order of the more common form of GBS. it is probably due to an auto-immune response directed against the axoplasm of peripheral nerves. sometimes accompanied by encephalopathy. and associated dysrhythmias. also known as Chinese paralytic syndrome. It usually affects the eye muscles first and presents with the triad of ophthalmoplegia. Like AMAN. attacks motor nodes of Ranvier and is prevalent in China and Mexico. Acute panautonomic neuropathy is the most rare variant of GBS. itching and peeling of skin. owing to cardiovascular involvement.Anti-GQ1b antibodies are present in 90% of cases. and areflexia. Anti-GD3 antibodies are found more frequently in AMAN. It is caused by an auto-immune response directed against Schwann cell membranes. It is associated with a high mortality rate. ataxia. lack of tear formation. It is probably due to an auto-immune response directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete. Anti-GD1a antibodies are present. The disease may be seasonal and recovery can be rapid. dryness of nasal and oral mucosa. Impaired sweating.photophobia. Acute motor axonal neuropathy (AMAN). constipation unrelieved by laxatives or alternating with diarrhea occur frequently in this patient . dysphagia. Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but also affects sensory nerves with severe axonal damage. nausea. Miller Fisher syndrome (MFS) is a rare variant of GBS and manifests as a descending paralysis.

It is characterized by acute onset of ophthalmoplegia. dryness of eyes. obstipation. loss of accommodation) may also be observed. disturbance of consciousness. vomiting. hyperintense lesions located mainly in the brainstem specially in the Pons. Midbrain and Medulla. Parasympathetic impairment (abdominal pain. large. as well as gastrointestinal and sudomotor dysfunction (Suarez et al. A considerable number of BBE patients have associated axonal Guillain Barré syndrome. hyperreflexia or Babinski's sign. urinary retention. Bickerstaff's brainstem encephalitis (BBE). Initial nonspecific symptoms of lethargy. fatigue. ileus. BE despite severe initial presentation usually has a good prognosis. group. . diarrhea. dilated unreactive pupils. blurring of vision. is a further variant of Guillain Barré syndrome. indicative that the two disorders are closely related and form a continuous spectrum. headache. ataxia. Magnetic resonance imaging (MRI) plays a critical role in the diagnosis of BBE. irregular. and disturbed micturition. The most common symptoms at onset are related to orthostatic intolerance. abdominal pain. The course of the disease can be monophasic or remitting-relapsing. and decreased initiative are followed by autonomic symptoms including orthostatic lightheadedness. 1994).

Certainly no single antibody is ubiquitous for Guillain Barré syndrome. with associated areas of demyelination. Prominent neural inflammatory infiltrates can occur in both Guillain Barré syndrome and experimental allergic neuritis. Some Wallerian degeneration may occur. Electron microscopy shows that macrophages cause the myelin damage. but changes are found at all levels of the peripheral nervous system.Pathology An autoimmune basis for the Guillain Barré syndrome seems likely but remains unproven. particularly following campylobacter infection. and penetrate the basement membrane around nerve fibres before stripping myelin sheaths off axons. this may reflect the distal and purely sensory nature of the sural nerve.and cell-mediated immune mechanisms. Guillain Barré syndrome bears a strong histological resemblance to experimental allergic neuritis. including both antibody. resembling experimental allergic neuritis. Teased peripheral sensory nerve fibre preparations may show marked segmental demyelination. Although antibodies to various gangliosides are described in Guillain Barré syndrome. an acute monophasic disorder induced by immunization of experimental animals with peripheral-nerve myelin proteins. This inflammatory infiltrate is mainly perivascular and comprised of lymphocytes and macrophages. Spinal nerve roots may be particularly affected. particularly P2 and galactocerebroside. it is unclear whether these antibodies are pathogenic. . The peripheral nerves in acute Guillain Barré syndrome often show inflammatory cell infiltrate. It is likely that diverse immunopathogenic mechanisms occur. Biopsy of the sural nerve may show surprisingly few abnormalities in comparison to the marked clinical severity of the neuropathy.

Pathogen and host cell have homologous/identical amino acid sequences. rabies/influenza immunizations Poor hygiene Stress Diet Etiologic Agent Campylobacter Jejuni Enters the body by the use of multifenestrated cells or other mechanisms Innate immune response results in the uptake of the pathogens by immature antigen presenting cells. MOLECULAR MIMICRY -a mechanism wherein immune response is triggered against autoantigens Autoimmunity/auto immune disease is the consequence of an immune response against self antigens that results in the damage and eventual dysfunction of target organs Auto antigens attack and damages GBS target organ---the PNS (peripheral nervous system) DEMYELINATION Guillain Barré syndrome . differentiated antigen presenting cell can present in major histocompatibility complex molecules and activate CD4 T cells that recognize antigens from the infectious pathogen B cells can be activated as well by newly activated Th2 cells.Predisposing Factors Age (extreme ages) Sex (men) Precipitating Factors Post infection of Campylobacter Jejuni Herpes simplex AIDS/HIV Hodgkin s disease Surgery Epstein Barr Virus Mononucleosis Rarely. Migration to lymph nodes. a mature. This produces a cell mediated and humoral response against the pathogen. antigens in its capsule are shared with nerves.

As opposed to infectious causes. conduction block.  Electrodiagnostics Electromyography (EMG) and nerve conduction study (NCS) may show prolonged distal latencies. In primary axonal damage. this is an elevated protein level (100 1000 mg/dL). the findings include reduced amplitude of the action potentials without conduction slowing  Diagnostic criteria Required:  Progressive. absence of fever.Diagnosis The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis. 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. without an accompanying increased cell count pleocytosis. relatively symmetrical weakness of two or more limbs due to neuropathy  Areflexia . and temporal dispersion of compound action potential in demyelinating cases. A sustained increased white blood cell count may indicate an alternative diagnosis such as infection. areflexia. conduction slowing. and a likely inciting event.  Cerebrospinal fluid (CSF) Typical CSF findings include albumino-cytological dissociation.

and the cellular spinal fluid encountered in acute ascending or transverse myelitis. and psychologically determined weakness. sensory level. drug and chemical toxins. or poliomyelitis. vasculitic neuropathy. and spinal MRI needs to be undertaken in cases of doubt. critical illness polyneuropathy. borreliosis. Acute spinal-cord lesions pose the most common diagnostic difficulty. infective neuropathies such as diphtheria. malignant meningitis. prominent sphincter involvement. However. porphyria. Pointers to primary muscle disease include the absence of . metabolic abnormalities. the distinction is usually simple because of the extensor plantar responses. It is rare for acute inflammatory myopathies to be confused with the Guillain Barré syndrome. neuromuscular transmission disorders.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 EMG  Differential diagnosis: The Guillain Barré syndrome usually presents a distinctive clinical picture. myopathy. The potential range of differential diagnosis of acutely evolving paralysis is enormous: spinal cord disease. biological toxins such as tick paralysis or botulism.

sensory symptoms. . Porphyric polyneuropathy is associated with early neuropsychiatric abnormalities. abnormal electromyogram. preserved reflexes. Three rare acute neuropathies should be distinguished from the Guillain Barré syndrome because they require different approaches to therapy. and raised serum creatine kinase levels. and preservation of the ankle jerks despite loss of the knee jerks. normal spinal-fluid protein. radicular pain. a purely motor syndrome. and a cellular CSF. Borrelia infection causing Lyme disease or Bannwarth's syndrome is suggested by prominent unilateral or bilateral facial paralysis. abdominal pain.

 Reduced or absent reflexes characterize GBS. followed by leg weakness.  Weakness is always bilateral. y Most cases will have subsequent arm weakness. and frequent gait ataxia. y About 70% of patients present with loss of reflexes. y GBS with a descending pattern of weakness seen in 14% cases. the degree of weakness in the arms and legs is roughly equal. before clinically evident limb weakness. predominantly motor neuropathy involving distal limb paresthesias. although some asymmetry in onset and severity is common. unlike the pattern seen with distal axonopathies. y Proximal muscles weakness very frequent. with subsequent distal arm and leg weakness. Early finger paresthesias suggest a patchy process. but sensory loss over the trunk frequent and a psuedolevel may be evident . relatively symmetric leg weakness. especially initially. and possibly weakness of facial. less than 5% retained all reflexes during the illness. y The presence of intact reflexes should suggest an alternative diagnosis other than GBS. Paresthesias of trunk or face unusual.Clinical Features  Typical GBS is an acute.  Sensory disturbance y >50% will present with symmetric distal limb paresthesias. y Early loss of reflexes may be due to desynchronization of afferent impulses in reflex arc due to non-uniform demyelination. i. ocular. y In 1/3 of cases. and oropharyngeal muscles. onset initially with cranial nerve or arm muscle weakness.

only rarely truly unilateral. iii. deep muscle aching in back. y Ophthalmoparesis see in 10-20% of patients. abducens palsy most common. iii. y Radicular pain can occasionally be a presenting complaint obscuring the true diagnosis. Rarely. ii.  beware if definite sensory level present as this may suggest structural cord disease large diameter afferent modalities (JPS.  Respiratory dysfunction due to diaphragmatic weakness occurs in about 1/3 of patients.ii. vibration) are most severely affected.  Cranial nerve involvement y 1/2 of GBS patients have some degree of cranial nerve dysfunction during their illness. and flaccid paralysis. sharp radicular pain into the legs. usually bilateral. hips or proximal legs. i. y Facial weakness most common. an early sensory ataxia may not be obscured by concurrent limb weakness  Pain y Some discomfort reported in 2/3 of patients which may take one of the following forms: i. especially if substantial limb weakness present. . normal facial strength in the presence of marked quadriparesis very unusual in typical GBS. i. severe burning dyesthetic pain in feet or hands. ii. facial weakness usually bilateral but may be unequal in severity. patients with GBS may appear locked-in. due to paralysis of all cranial muscles. ventilatory failure. i. y Oropharyngeal weakness present in almost 1/2 of cases increasing the risk of aspiration.

inappropriate ADH. inspiratory force and tidal volume due to diaphragmatic weakness. y ICU monitoring necessary because of possible cardiac complications. y Other features: ileus. y Patients with weakness of neck muscles. mild orthostatic hypotension. y Most common manifestations include cardiac dysfunction such as sinus tachycardia. . have longer hospitalizations. and hypotension (especially postural). atelectasis results from reduced vital capacity. patients requiring ventilator support have less favorable prognosis for neurologic recovery. may also occur early on in patients with bibrachial weakness. sinus bradycardia. urinary retention (1/4 cases).  Dysautonomia y occurs in about 65% of cases y more frequent in patients with severe paralysis and ventilator difficulties but may develop in mild cases. and higher mortality. ii. paroxysmal hypertension. if diaphragmatic and respiratory muscle weakness has not occurred by 2 weeks into illness then assisted ventilation usually unnecessary. y Pathogenesis of respiratory failure: i.y Diaphragmatic weakness common in patients with severe quadriparesis. the resultant tachypnea and increased work of breathing iv. atelectasis worsened by impair cough. altered sweating. sinus arrest and other supraventricular arrhythmias. iii. v. result is arteriovenous shunting and hypoxia. tongue and palate often have concommitant diaphragmatic and respiratory muscle involvement.

Nasal endotracheal tubes are well tolerated by conscious patients and should be replaced by temporary tracheostomy if. and morphine. physiotherapy and careful limb positioning will prevent muscle contractions in patients with prolonged paralysis. frusemide (furosemide). the period of ventilation is likely to exceed 1 week. The gastrocnemius and soleus muscles are particularly prone to such contractures. Nursing care will prevent decubitus ulcers. Plasma exchange . Subcutaneous heparin (5000 units four times daily) and elastic stockings provide prophylaxis against deep venous thrombosis and pulmonary embolism. Feeding by nasogastric tube should be instituted in those with bulbar dysfunction. that is. Ventilation Endotracheal intubation and ventilation should be instituted without delay either if respiratory muscle failure is imminent or if paralysis of bulbar and laryngeal muscles places the patient at risk of choking. Assisted ventilation is usually required when the vital capacity has fallen to 15 ml/kg body weight. Vigilant electrocardiographic monitoring allows prompt recognition and treatment of cardiac arrhythmias which may be provoked by endotracheal suctioning or suxamethonium administration. which may lead to permanent walking disability even if muscle power returns.Management Survival in the Guillain Barré syndrome depends primarily upon meticulous attention to intensive care during the acute paralytic phase. a vital capacity of approximately 1 litre for a 65 kg adult. -Blockers may be required for those with hypertensive crises. Regular Occupational therapy. Pulmonary atelectasis and infection are common in intubated patients and should be treated promptly with antibiotics and Occupational therapy/physiotherapy. as is usually the case. Patients with Guillain Barré syndrome are particularly susceptible to hypotensive side-effects of drugs. including thiopentone (thiopental).

Plasma-exchange schedules vary. About 10 per cent of patients treated by plasma exchange will subsequently undergo a mild relapse between 5 and 42 days later. which may be treated by a further course of plasma exchange. to regain functional abilities such as walking. are recommended. is at least equally effective as plasma exchange. Intravenous immunoglobulin Intravenous immunoglobulin (IvIg). given at 0. given on sequential days. has become the treatment of choice because it is immediately available. Plasma exchange is recommended for those patients approaching inability to walk or with impairment of bulbar or respiratory function. Plasma exchange enabled the median patient to walk independently at 53 days compared to 85 days for controls. . It is unclear whether plasma exchange improves survival or reduces the number of patients unable to walk at 1 year. Also. but four or five 4-litre exchanges using a continuous-flow technique. IvIg may be more effective than plasma exchange for the motor axonal subgroup resulting from diarrhoeal campylobacter infections.4 g/kg body weight/day for 5 days. and allowed 82 per cent to walk independently at 6 months compared to 71 per cent of controls. To be maximally effective. and doesn't carry the same risks of exacerbating circulatory disturbances due to autonomic neuropathy. and reduces their requirement for assisted ventilation.Plasma exchange shortens the time taken for patients with Guillain Barré syndrome to start to improve. plasma exchange needs to be started within the first week of neurological symptoms. does not require cannulation of a major vessel. has fewer side-effects than plasma exchange.

and mean distal compound muscle action potentials of less than 20 per cent of normal. include age over 60 years. Even when general intensive care facilities are available. respiratory failure requiring ventilation. 16. These patients are usually elderly and generally succumb to cardiac disease. pulmonary embolism. Of the survivors. up to 10 per cent of patients may die in the acute phase of the disease. . absent ankle jerks. development of severe paralysis within 5 days of the onset.5 per cent are unable to walk at 48 weeks. nearly 60 per cent make a full recovery but the other 40 per cent show some permanent residual symptoms and signs. or chest infections. Even after IvIg or plasma exchange therapy. The factors predictive of poor outcome with slow recovery or permanent disability. a preceding diarrhoeal illness.Prognosis Most patients with the Guillain Barré syndrome will make a good spontaneous recovery if they receive competent supportive treatment. The mortality is 4 5 per cent even for patients treated in specialist neurological units with plasma exchange or IvIg. or distal sensory loss. usually weakness of distal leg muscles.

Bibliography: y NEUROLOGY AND NEUROSURGERY ILLUSTRATED.Angelika F Hahn .Alan R.Lindsay y Neurology-Brain's Diseases of the Nervous System y Wikipedia y Guillain Barre Syndrome and Its Variants.D.Jacqueline Lim RN y Guillain-Barré syndrome. M. y Guillain Barré Syndrome. Berger.

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