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Neurology Assignment GBS

Neurology Assignment GBS

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Neurology Assignment On

Guillain Barre Syndrome

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


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

Usually GuillainBarré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection. or weeks. After the first clinical manifestations of the disease. afflicting only about one person in 100. and by the third week of the illness 90 percent of all patients are at their weakest. . the symptoms can progress over the course of hours. Occasionally surgery or vaccinations will trigger the syndrome. days.however.000. Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear.

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

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

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

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

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

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. 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. This produces a cell mediated and humoral response against the pathogen. a mature. 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 . antigens in its capsule are shared with nerves. Pathogen and host cell have homologous/identical amino acid sequences. Migration to lymph nodes. 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.

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

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

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

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

deep muscle aching in back. hips or proximal legs. due to paralysis of all cranial muscles. i. i. iii. ii. severe burning dyesthetic pain in feet or hands.  beware if definite sensory level present as this may suggest structural cord disease large diameter afferent modalities (JPS. patients with GBS may appear locked-in. ventilatory failure.  Cranial nerve involvement y 1/2 of GBS patients have some degree of cranial nerve dysfunction during their illness. i. y Radicular pain can occasionally be a presenting complaint obscuring the true diagnosis. y Facial weakness most common.ii. facial weakness usually bilateral but may be unequal in severity. especially if substantial limb weakness present. abducens palsy most common. ii. Rarely. iii. and flaccid paralysis. normal facial strength in the presence of marked quadriparesis very unusual in typical GBS. vibration) are most severely affected. 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. y Ophthalmoparesis see in 10-20% of patients. y Oropharyngeal weakness present in almost 1/2 of cases increasing the risk of aspiration.  Respiratory dysfunction due to diaphragmatic weakness occurs in about 1/3 of patients. . sharp radicular pain into the legs. usually bilateral. only rarely truly unilateral.

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

the period of ventilation is likely to exceed 1 week. Subcutaneous heparin (5000 units four times daily) and elastic stockings provide prophylaxis against deep venous thrombosis and pulmonary embolism. Nursing care will prevent decubitus ulcers. as is usually the case. Pulmonary atelectasis and infection are common in intubated patients and should be treated promptly with antibiotics and Occupational therapy/physiotherapy.Management Survival in the Guillain Barré syndrome depends primarily upon meticulous attention to intensive care during the acute paralytic phase. -Blockers may be required for those with hypertensive crises. Feeding by nasogastric tube should be instituted in those with bulbar dysfunction. including thiopentone (thiopental). which may lead to permanent walking disability even if muscle power returns. The gastrocnemius and soleus muscles are particularly prone to such contractures. Assisted ventilation is usually required when the vital capacity has fallen to 15 ml/kg body weight. Patients with Guillain Barré syndrome are particularly susceptible to hypotensive side-effects of drugs. frusemide (furosemide). Vigilant electrocardiographic monitoring allows prompt recognition and treatment of cardiac arrhythmias which may be provoked by endotracheal suctioning or suxamethonium administration. physiotherapy and careful limb positioning will prevent muscle contractions in patients with prolonged paralysis. 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. Nasal endotracheal tubes are well tolerated by conscious patients and should be replaced by temporary tracheostomy if. a vital capacity of approximately 1 litre for a 65 kg adult. Plasma exchange . and morphine. Regular Occupational therapy.

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

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

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

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