Most individuals with Miller Fisher syndrome have a unique antibody that characterizes the disorder. Related peripheral nerve disorders with slow onset and persisting or recurrent symptoms include chronic inflammatory demyelinating polyneuropathy CIDP and multifocal motor neuropathy. CIDP features weakness that can recur, repeatedly, over the course of years. Multifocal motor neuropathy typically affects many different muscles in a small part of a limb or limbs. Usually the symptoms are more severe on one side of the body.
The initial signs and symptoms of GBS are varied and there are several disorders with similar symptoms. Therefore, doctors may find it difficult to diagnose GBS in its earliest stages. In GBS, deep tendon reflexes in the legs, such as knee jerks, are usually lost. Reflexes may also be absent in the arms. There is a change in the cerebrospinal fluid that bathes the spinal cord and brain in people with GBS.
Researchers have found the fluid contains more protein than usual but very few immune cells measured by white blood cells. Therefore, a physician may decide to perform a spinal tap or lumbar puncture to obtain a sample of spinal fluid to analyze. This procedure is usually safe, with rare complications.
However, some therapies can lessen the severity of the illness and shorten recovery time. There are also several ways to treat the complications of the disease. There are currently two treatments commonly used to interrupt immune-related nerve damage. One is plasma exchange PE, also called plasmapheresis ; the other is high-dose immunoglobulin therapy IVIg.
Both treatments are equally effective if started within two weeks of onset of GBS symptoms, but immunoglobulin is easier to administer. Using both treatments in the same person has no proven benefit. The blood cells from the liquid part of the blood plasma are extracted and returned to the person. Plasma contains antibodies and PE removes some plasma; PE may work by removing the bad antibodies that have been damaging the nerves.
Immunoglobulins are proteins that the immune system naturally makes to attack infecting organisms. IVIg therapy involves intravenous injections of these immunoglobulins. The immunoglobulins are developed from a pool of thousands of normal donors.
The IVIg can also shorten recovery time. However, controlled clinical trials have demonstrated that this treatment is not effective. Supportive care is very important to address the many complications of paralysis as the body recovers and damaged nerves begin to heal. Sometimes a mechanical ventilator is used to help support or control breathing.
The autonomic nervous system that regulates the functions of internal organs and some of the muscles in the body can also be disturbed, causing changes in heart rate, blood pressure, toileting, or sweating. Therefore, the person should be put on a heart monitor or equipment that measures and tracks body function.
Occasionally GBS-related nerve damage can lead to difficulty handling secretions in the mouth and throat. As individuals begin to improve, they are usually transferred from the acute care hospital to a rehabilitation setting. Scientists have multiple theories on why this increased risk may have occurred, but the exact reason for this association remains unknown. We expect the H1N1 vaccine to have a similar safety profile as seasonal flu vaccines, which have very good safety track records.
The seasonal influenza vaccine has not been consistently associated with GBS. Ensuring the safety of vaccines is a high priority for CDC. CDC and its partners are actively monitoring the H1N1 vaccine to ensure its safety.
Several systems are in place to monitor vaccine safety. CDC and FDA scientists review all VAERS reports and store the information in a computerized database that is monitored to detect new, unusual, or rare health events that could be possible side effects of vaccines. In addition to the normal vaccine safety monitoring systems, CDC has proactively put additional monitoring systems in place to ensure safety after licensing.
Some of these systems include: actively observing persons in defined geographic areas, collaborating with professional organizations for reports of any adverse events after vaccination, and conducting thorough investigations when severe adverse events occur to determine whether they may have been associated with the vaccine. Through these numerous approaches, we are able to detect any possible risk of GBS that might be associated with the H1N1 vaccine as early as possible and take appropriate action.
Most patients with GBS recover spontaneously. However, because of the unpredictable course and potential for death or significant disability, all patients with the disease should be hospitalized for multidisciplinary supportive care and disease-modifying therapy. Consensus guidelines recommend subcutaneous anticoagulation and graduated compression stockings for patients who are hospitalized to decrease the risk of venous thrombosis.
Predictive factors that indicate the need for mechanical ventilation are shown in Table 4. Patients with limited mobility should be closely monitored and prospectively treated to prevent skin breakdown.
Predicts the need for mechanical ventilation 24 — Vital capacity, maximum inspiratory pressure, or maximum expiratory pressure reduced by at least 30 percent.
Predicts long-term disability 19 , Information from references 19 , and 24 through Patients with GBS often experience both neuropathic and nociceptive pain, and one-half of patients rate the pain as severe. Small studies reported that, when added to other analgesic regimens, gabapentin Neurontin; 15 mg per kg daily 28 or carbamazepine Tegretol; mg daily for three days 29 was beneficial in patients with acute GBS in intensive care units.
Long-term management with tricyclic antidepressants, tramadol Ultram , gabapentin, or carbamazepine may be beneficial for chronic pain. Patients with GBS should do strengthening exercises during the acute phase, and rehabilitation should be considered to regain mobility and function as they improve. Up to 80 percent of patients experience persistent, severe fatigue after resolution of other symptoms.
The degree of fatigue does not appear to be related to severity of illness, duration of disability, or patient age. No pharmacologic treatments have proved beneficial for fatigue. Despite limited evidence, a supervised exercise program is recommended to improve fatigue and functional abilities.
Removing circulating immune complexes via plasma exchange has been shown to improve the time to recover the ability to walk, the need for artificial ventilation, the duration of ventilation, and measured muscle strength after one year compared with placebo. Intravenous immune globulin therapy has been shown to hasten recovery in adults and children compared with supportive therapy alone. The typical dosage is mg per kg per day for five days, although some evidence suggests that a total of 2 g per kg over two days is equally effective.
Intravenous immune globulin therapy should be started within two weeks of symptom onset, and should be considered for patients who are nonambulatory. The therapy may have a role two to four weeks after symptom onset as well, but the evidence of effectiveness is weaker.
The few studies of plasma exchange followed by intravenous immune globulin therapy have not shown benefit over monotherapy. Therefore, sequential therapy is not recommended. Corticosteroids are not recommended for the treatment of GBS. Even with treatment, about 3 percent of patients with GBS die. The median hospital stay is seven days, and up to 25 percent of patients require intubation and mechanical ventilation.
The prognosis is worse in older patients, those with severe symptoms, and those with rapid onset of symptoms.
Neurologic problems persist in up to 20 percent of patients, one-half of whom are severely disabled. The search included meta-analyses, randomized controlled trials, clinical trials, and reviews. We also searched the Agency for Healthcare Research and Quality evidence reports, Clinical Evidence, the Cochrane database, Essential Evidence Plus, the National Guideline Clearinghouse database, and the bibliographies of the initially identified papers.
Search date: November 15, Already a member or subscriber? Log in. Interested in AAFP membership? Learn more. Address correspondence to Anne D. Kansas, Wichita, KS Reprints are not available from the authors. Eur Neurol. Ann Neurol. Lancet Neurol. Ropper AH. N Engl J Med. J Neurol Neurosurg Psychiatry. Lancet Infect Dis. Acta Neurol Scand.
Zochodne DW. Muscle Nerve. Cerebrospinal fluid HIV infection and pleocytosis: relation to systemic infection and antiretroviral treatment. BMC Infect Dis. Neurol Clin. Arch Neurol. Crit Care Med. Crit Care. Anesth Analg. Tripathi M, Kaushik S. In children, autonomic dysfunction was present in half of the mildly affected patients and seemed not to be related to the severity of the disease, 81 which implies that autonomic disturbances are common and may occur throughout the spectrum of severity of GBS.
Specifically, the relationship between specific antiganglioside antibodies and autonomic dysfunction needs to be investigated in more detail. We did not observe a correlation between reduction of intraepidermal nerve fibre density and autonomic failure. Careful monitoring for the presence of features of autonomic dysfunction is indicated also after a patient is discharged from the ICU. As the clinical course and outcomes of GBS are highly variable, their accurate prediction is important to enable clinicians to tailor supportive care and treatment to the individual patient's needs and to inform patients and relatives about the expected clinical course.
Three large studies have been performed to predict the probability of respiratory insufficiency in patients with GBS. If a patient's predicted chance of developing respiratory insufficiency is high, it can be advisable to admit the patient to the ICU rather than to a general neurology ward. Validated prognostic models have been developed for use in clinical practice to predict long-term outcome in individual patients with GBS. In addition to their diagnostic role, electrophysiological findings might have prognostic relevance.
In one NCS study, patients with features of demyelination more often required mechanical ventilation than did patients without this type of damage. Further studies are required to precisely delineate the relationships between conduction block, the presence of antiganglioside antibodies, the effects of treatment, and outcome.
Further research is urgently required to improve this situation. From a clinical perspective, the following are the most challenging needs: to develop improved diagnostic criteria for use in daily clinical practice, trials and vaccine safety studies; to determine the burden of disease caused by GBS worldwide; to develop new and better GBS outcome measures; to establish the precipitating events and patient-related factors that lead to GBS; to define biological and clinical predictors of the clinical course and outcome in individual patients; and, most importantly, to develop more-effective and specific treatments, as well as protocols for supportive care.
These aims can probably only be achieved by large-scale international and multidisciplinary collaborations, such as the International GBS Outcome Study IGOS , which was launched in Tissue samples and detailed, standardized clinical data are being collected during a follow-up period of 1—3 years with the intention of including at least 1, patients with GBS from all over the world.
This trial is investigating the effect of a second course of IVIg given shortly after the first course in patients with GBS who have a poor prognosis defined as a mEGOS score of 6—12 at 1 week after initiation of a standard 5 day course of IVIg. In addition, a new randomized placebo-controlled trial of eculizumab in patients with early GBS who are unable to walk is about to start. GBS is a heterogeneous and often severe disorder. Although nonspecific immunotherapy is available and effective in most patients, a need remains for improved treatment and patient care throughout the disease course.
Optimal supportive medical care is also essential to prevent or treat disease-related complications. Novel prognostic models have been developed and might enable treatment to be tailored to each patient's requirements. Although a large amount of information on the immunopathogenesis of the various subtypes of GBS has been collected, further research is still needed.
In the past few years, new large-scale clinical studies based on worldwide collaborations have been started and will help us to further define this syndrome and to optimize the care of affected patients. PubMed was searched for articles published between January and March Only papers in English were reviewed. Articles were selected for their relevance, with a preference for new papers. Some other relevant papers known by the authors were also included.
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Biosynthesis of sialylated ganglioside mimics in the core oligosaccharide. A Campylobacter jejuni gene associated with immune-mediated neuropathy. Godschalk, P.
Kusunoki, S. Acta , — Drenthen, J. Psychiatry 82 , — Ogawara, K. Visser, L. A subgroup with specific clinical, electrodiagnostic and laboratory features. Chiba, A. Fisher syndrome and Bickerstaff brainstem encephalitis Fisher—Bickerstaff syndrome. Psychiatry 62 , — Rinaldi, S. Hakomori, S. The glycosynapse. Natl Acad. USA 99 , — Hafer-Macko, C.
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