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Introduction/aims: We aimed to determine whether specific severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) vaccines may be associated with acute-onset polyradiculoneuropathy and if they may result in particular clinical presentations., Methods: We retrospectively reviewed records of all persons presenting with acute-onset polyradiculoneuropathy from January 1, 2021, to June 30, 2021, admitted to two Neuroscience centers, of the West and North Midlands, United Kingdom. We compared subjects with previous SARS-CoV2 vaccine exposure with a local cohort of persons with acute-onset polyradiculoneuropathy admitted between 2005 and 2019 and compared admission numbers for the studied time frame with that of the previous 3 years., Results: Of 24 persons with acute-onset polyradiculoneuropathy, 16 (66.7%) presented within 4  weeks after first SARS-CoV2 vaccine. Fourteen had received the AstraZeneca vaccine and one each, the Pfizer and Moderna vaccines. The final diagnosis was Guillain-Barré syndrome (GBS) in 12 and acute-onset chronic inflammatory demyelinating polyneuropathy in 4. Among AstraZeneca vaccine recipients, facial weakness in nine persons (64.3%), bulbar weakness in seven (50%), and the bifacial weakness and distal paresthesias GBS variant in three (21.4%), were more common than in historical controls (P = .01; P = .004, and P = .002, respectively). A 2.6-fold (95% confidence interval: 1.98-3.51) increase in admissions for acute-onset polyradiculoneuropathy was noted during the studied time frame, compared to the same period in the previous 3 years., Discussion: Despite a low risk, smaller than that of SARS-CoV2 infection and its complications, exposure to the first dose of AstraZeneca SARS-CoV2 vaccine may be a risk factor for acute-onset polyradiculoneuropathy, characterized by more common cranial nerve involvement., (© 2021 Wiley Periodicals LLC.)

Objective: To define the clinicopathologic features of amphiphysin-immunoglobulin G (IgG)-mediated neuropathy., Methods: Patients examined at our institution from January 1, 1995, to September 30, 2018, with amphiphysin-IgG by indirect immunofluorescence and Western blot, were reviewed. Their phenotypes were compared to cases of coexisting collapsin response-mediator protein-5 (CRMP5)-IgG or anti-neuronal nuclear antibody type 1 (ANNA1-IgG) and CRMP5-IgG autoimmunity. Improvement in modified Rankin Scale (mRS) (≥1) on follow-up was considered a favorable outcome. Amphiphysin RNA expression was assessed in healthy nerves., Results: Fifty-three amphiphysin-IgG-positive cases were identified. Of 33 (60%) patients with neuropathy, 21 had amphiphysin-IgG alone, and 12 had coexisting autoantibodies (ANNA1-IgG, n = 8; CRMP5-IgG, n = 2; ANNA1-IgG and CRMP5-IgG, n = 2). The neuropathies in isolated amphiphysin-IgG autoimmunity included polyradiculoneuropathy (62%), diffuse sensory neuronopathy (35%), and facial neuropathy with gastroparesis (3%). Among these, pain (80%), breast cancer (63%), and CNS (57%) involvements commonly coexisted, and neuropathy frequently prompted breast cancer diagnosis (76%). Stiff-person spectrum disorder was the most common CNS accompaniment (45%). Nerve biopsies showed axonal loss (n = 6/6), subperineurial edema (n = 4/6), and CD4 predominant inflammation (n = 2/6). Median mRS score at last follow-up was 3.5; 58% of patients were immunotherapy-responsive. Patients with amphiphysin-IgG alone had more favorable immunotherapy response than patients with CRMP5-IgG polyneuropathy (n = 45) (44% vs 16%, p = 0.028, odds ratio 4.2, 95% confidence interval 1.1 to 15.5). Only 1/9 (11%) patients with amphiphysin-IgG with coexisting CRMP5-IgG or ANNA1-IgG had immunotherapy response. RNA amphiphysin expression occurred at low levels in nerve., Conclusion: Amphiphysin-IgG autoimmune neuropathy has a recognizable phenotype, is frequently immune responsive, and can prompt early diagnosis of breast cancer., (© 2019 American Academy of Neurology.)

Objective: To determine the previously unknown incidence of lumbosacral radiculoplexus neuropathy (LRPN) and its association with diabetes mellitus (DM)., Methods: LRPN defined by clinical and electrophysiologic criteria was identified among Olmsted County, Minnesota, residents during a 16-year period (2000-2015) using the unique facilities of the Rochester Epidemiology Project. DM was ascertained using American Diabetes Association criteria., Results: Of 1,892 medical records reviewed, 59 patients (33 men, 26 women) were identified as having LRPN. The median age was 70 years (range 24-88 years) and the median time of onset of symptoms to diagnosis was 2 months (range 1-72 months). DM was more frequent in patients with LRPN than in controls (39/59 vs 35/177, p < 0.001) but not in those with pre-DM (10/20 vs 55/142, p = 0.336). LRPN recurred in 3 patients with DM resulting in 62 LRPN episodes during the study period. The overall incidence of LRPN was 4.16/100,000/y (95% confidence interval [CI] 3.13-5.18). The incidences of LRPN among DM and non-DM groups were 2.79/100,000/y (95% CI 1.94-3.64) and 1.27/100,000/y (95% CI 0.71-1.83), respectively. The odds of LRPN among patients with DM and pre-DM was 7.91 (95% CI 4.11-15.21) and 1.006 (95% CI 1.004-1.012), respectively., Conclusions: LRPN incidence in Olmsted County of 4.16/100,000/y makes LRPN a common inflammatory neuropathy and is higher than that of other immune-mediated neuropathies (acute or chronic inflammatory demyelinating polyradiculoneuropathy, brachial plexus neuropathy) assessed within the same population. DM is a major risk factor for LRPN and thus justifies the continued classification of LRPN into diabetic and nondiabetic forms., (© 2019 American Academy of Neurology.)

Objectives: To investigate whether the development of acute canine polyradiculoneuritis is associated with various putative risk factors., Materials and Methods: Retrospective case-control study with conditional logistic regression analysis from a referral hospital population in the UK where controls were matched for year of presentation., Results: Forty-three cases were identified with acute canine polyradiculoneuritis and 86 controls were selected. Jack Russell terriers and West Highland white terriers were found to have a significantly greater odds of developing acute canine polyradiculoneuritis compared to a mixed baseline group of dogs. The odds of developing acute canine polyradiculoneuritis were greater in the autumn and winter compared to spring. Vaccination, rural/urban habitation, sex, neuter status and age were not associated with development of acute canine polyradiculoneuritis in our population of dogs., Clinical Significance: Breed and season were associated with development of acute canine polyradiculoneuritis. However, this is a small sample and so this observation needs confirmation in further studies and other factors may also be involved. Nevertheless, these findings may be important in further understanding the aetiopathogenesis of this condition., (© 2017 British Small Animal Veterinary Association.)

Background: Tremor is known to occur in patients with neuropathies although its reported prevalence varies widely. Tremor has been shown to cause disability in children with Charcot-Marie-Tooth disease but no data exit about the disability caused by tremor in inflammatory neuropathies. Little is known about the response of neuropathic tremor to treatment and why it selectively occurs in some people and not others., Methods: This case control study investigates the presence and severity of tremor in 43 consecutively recruited patients with inflammatory neuropathies at the National Hospital for Neurology and Neurosurgery, London. Clinical assessment, including Fahn-Tolosa-Marin Scale for tremor, sensory scores, power scores and Overall Neuropathy Limitations Scale, were recorded. Results of nerve conduction studies were retrieved and assessed. Nine patients' tremors were recorded with accelerometry., Results: Tremor was most common in IgM paraproteinaemic neuropathies, as previously reported, but also occurred in 58% of those with chronic inflammatory demyelinating polyradiculoneuropathy and 56% of those with multifocal motor neuropathy with conduction block. We describe, for the first time, tremor in the majority of patients with multifocal motor neuropathy with conduction block. Tremor in all of these patients seems generally refractory to treatment except in a small number of cases where tremor improves with treatment of the underlying neuropathy. We provide evidence that tremor may add to disability in patients with inflammatory neuropathy. Mean tremor frequency was 6 Hz and did not vary with weight loading. We demonstrate for the first time that although tremor severity correlates with F wave latency, it is not sufficient to distinguish those with, from those without, tremor., Conclusion: Tremor in inflammatory neuropathies is common, adds to disability and yet does not often respond to treatment of the underlying neuropathy. When present, tremor severity is associated with F wave latency.

This article reviews the main diagnostic, epidemiological and therapeutic issues relating to the three main inflammatory neuropathies: Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy. The current knowledge base and recent developments are described.

We report meningitis with diffuse neuralgic pain or polyradiculoneuropathy associated with PCR-documented acute hepatitis E in 2 adults. These observations suggest that diagnostic testing for hepatitis E virus should be conducted for patients who have neurologic symptoms and liver cytolysis.

Twenty-four patients, all of whom were exposed to aerosolized porcine brain tissue through work-place environment (abattoir), developed a syndrome of immune-mediated polyradiculoneuropathy; three also had central nervous system manifestations (transverse myelitis, meningoencephalitis, and aseptic meningitis). Patients had characteristic electrophysiological findings of very distal and proximal conduction slowing (prolonged distal and F-wave latencies, regions where the blood-nerve barrier is the most permeable) and all patients' serum contained a novel IgG immunofluorescence pattern. Nerve pathology, when available, showed mild changes of segmental demyelination, axonal degeneration, and inflammatory changes. Patients had meaningful improvement of symptoms and electrophysiologic findings with immune therapy and with removal of exposure to aerosolized brain tissue. We postulate that this outbreak is an auto-immune polyradiculoneuropathy triggered by occupational exposure to multiple aerosolized porcine neural tissue antigens that result in neural damage where the blood-nerve barrier is the least robust., (© 2011 Peripheral Nerve Society.)

Background: Acute canine polyradiculoneuritis (ACP) is considered to be an animal model of the acute axonal form of Guillain-Barré syndrome (GBS) in humans. Various antecedent events have been associated with GBS, including bacterial or viral infection. The relationship between ACP and previous infection requires additional attention., Hypothesis: We hypothesized a relationship between ACP and serological evidence of exposure to Ehrlichia canis, Borrelia burgdorferi, Toxoplasma gondii, Neospora caninum, Campylobacter jejuni, and canine distemper virus (CDV)., Animals: Eighty-eight client-owned dogs, 44 with ACP, 44 age-matched controls., Methods: Retrospective study with stored serum samples. Serum antibodies against the target organisms were measured with commercially available assays. Sera from dogs with and without ACP that were positive for T. gondii IgG by ELISA were assayed by an IgG heavy chain-specific, Western blot immunoassay., Results: Dogs with ACP (55.8%) were more likely to have T. gondii IgG serum antibody titers than dogs without ACP (11.4%). Serum antibodies from 8 affected dogs and 11 control dogs bound to T. gondii antigens with apparent molecular masses of 67, 61, 58, 45, 33, 24, 9, and 6 kDa. An antigen with an apparent molecular mass of 36 kDa was recognized by 2 dogs with ACP but none of the control dogs., Conclusions: Results of this study suggest that ACP in some dogs, like GBS in some humans, may be triggered by T. gondii and a prospective study should be performed to further evaluate this potential association., (Copyright © 2011 by the American College of Veterinary Internal Medicine.)

Background: In October 2007, a cluster of patients experiencing a novel polyradiculoneuropathy was identified at a pork abattoir (Plant A). Patients worked in the primary carcass processing area (warm room); the majority processed severed heads (head-table). An investigation was initiated to determine risk factors for illness., Methods and Results: Symptoms of the reported patients were unlike previously described occupational associated illnesses. A case-control study was conducted at Plant A. A case was defined as evidence of symptoms of peripheral neuropathy and compatible electrodiagnostic testing in a pork abattoir worker. Two control groups were used - randomly selected non-ill warm-room workers (n = 49), and all non-ill head-table workers (n = 56). Consenting cases and controls were interviewed and blood and throat swabs were collected. The 26 largest U.S. pork abattoirs were surveyed to identify additional cases. Fifteen cases were identified at Plant A; illness onsets occurred during May 2004-November 2007. Median age was 32 years (range, 21-55 years). Cases were more likely than warm-room controls to have ever worked at the head-table (adjusted odds ratio [AOR], 6.6; 95% confidence interval [CI], 1.6-26.7), removed brains or removed muscle from the backs of heads (AOR, 10.3; 95% CI, 1.5-68.5), and worked within 0-10 feet of the brain removal operation (AOR, 9.9; 95% CI, 1.2-80.0). Associations remained when comparing head-table cases and head-table controls. Workers removed brains by using compressed air that liquefied brain and generated aerosolized droplets, exposing themselves and nearby workers. Eight additional cases were identified in the only two other abattoirs using this technique. The three abattoirs that used this technique have stopped brain removal, and no new cases have been reported after 24 months of follow up. Cases compared to controls had higher median interferon-gamma (IFNgamma) levels (21.7 pg/ml; vs 14.8 pg/ml, P<0.001)., Discussion: This novel polyradiculoneuropathy was associated with removing porcine brains with compressed air. An autoimmune mechanism is supported by higher levels of IFNgamma in cases than in controls consistent with other immune mediated illnesses occurring in association with neural tissue exposure. Abattoirs should not use compressed air to remove brains and should avoid procedures that aerosolize CNS tissue. This outbreak highlights the potential for respiratory or mucosal exposure to cause an immune-mediated illness in an occupational setting.

Background: Between November, 2006, and May, 2008, a subacute neurological syndrome affected workers from two swine abattoirs in Minnesota and Indiana who had occupational exposure to aerosolised porcine brain. We aimed to describe the pathogenic and immunological characteristics of this illness., Methods: All patients from two abattoirs who presented or were referred to the Mayo Clinic (Rochester, MN, USA) with neurological symptoms were included. We recorded details of exposure to aerosolised brain tissue and did comprehensive neurological, laboratory, neuroimaging, electrophysiological, pathological, and autoimmune serological assessments. Healthy controls were recruited from the community and from workers at the plant in Minnesota., Findings: 24 patients were identified (21 from Minnesota, three from Indiana). The shortest duration from first exposure to symptom onset was 4 weeks. No infectious agent that could trigger disease was identified. All patients developed polyradiculoneuropathy, which was usually sensory predominant and painful. Two patients had initial CNS manifestations: transverse myelitis and meningoencephalitis. Nerve conduction studies localised abnormalities to the most proximal and distal nerve segments. Quantitative sensory and autonomic testing revealed involvement of large and small sensory fibres and sweat fibres. MRI showed prominent abnormalities of roots and ganglia. Nerve biopsies identified mild demyelination, axonal degeneration, and perivascular inflammation. Protein concentrations were high in the CSF of 18 (86%) of 21 patients. Sera from all patients and 29 (34%) of 85 unaffected workplace controls (but none of 178 community controls) had a distinctive neural-reactive IgG; 75% of patients' sera contained an IgG specific to myelin basic protein. Seropositivity correlated directly with exposure risk in patients and controls. 17 patients required immunomodulatory therapies, six improved spontaneously, and one was lost to follow-up after exposure stopped., Interpretation: The neurological disorder described is autoimmune in origin and is related to occupational exposure to multiple aerosolised porcine brain tissue antigens. The pattern of nerve involvement suggests vulnerability of nerve roots and terminals where the blood-nerve barrier is most permeable., Funding: Mayo Clinic Foundation; Minnesota Department of Health; Centers for Disease Control and Prevention., (Copyright 2010 Elsevier Ltd. All rights reserved.)

We have examined whether antibodies to myelin-associated glycoprotein (anti-MAG) influence neuropathy occurrence and phenotype in primary (AL IgM) amyloidosis. Anti-MAG and the cross-reacted sulfoglucuronyl paragloboside antibodies (SGPG) were studied in 46 patients with IgM amyloidosis (21 with polyneuropathy), and 21 matched IgM MGUS (monoclonal gammopathies of undetermined significance) controls without neuropathy. We assessed the occurrence, phenotype of neuropathy, and attributes of nerve conduction and their relation to antibody activity. Twenty of 46 patients with IgM amyloidosis (7 with and 13 without polyneuropathy) had elevation of anti-MAG or SGPG by enzyme-linked immunosorbent assay (ELISA). Two of the polyneuropathy patients with IgM amyloidosis had antibodies to MAG based on Western blot (WB) positivity. One of these patients, with the highest anti-MAG titer, had a painful sensory ataxia, with prominent demyelination, and amyloid deposition in sural nerve. The other anti-MAG WB-positive amyloid patient had an axonal neuropathy and dysautonomia. Low levels of anti-MAG antibodies were found in 12 of 21 IgM MGUS controls without neuropathy (mean follow-up, 11 years). We conclude that finding serum anti-MAG antibodies does not exclude the diagnosis of primary amyloidosis. They do not appear to affect the occurrence or expression of polyneuropathy, except possibly in occasional cases with WB positivity.

Systemic inflammatory response syndrome (SIRS) is a medical condition in which the all-organ microcirculation is affected including nervous system. We describe neurological findings in 64 patients with SIRS at Hospital das Clínicas of Sao Paulo University School of Medicine; 45.3% were male and 54.7% female; their age ranged from 16 to 95 years old. SIRS was caused by infection in 68.8% of patients, trauma in 10.9%, burns in 7.8%, and elective surgery in 4.7%. The central nervous system involvement occurred in 56.3% of patients and was characterized as encephalopathy in 75%, seizures in 13.9%, non-epileptic myoclonus in 2.8%, and ischemic stroke in 8.3%. The magnetic resonance imaging, cerebrospinal fluid and electroencephalographic changes were unremarkable in encephalopathic patients. Neuromuscular disorders were diagnosed in 43.7%. Critical ill polyneuropathy was characterized in 57.1%, critical ill myopathy in 32.1%, demyelinating neuropathy in 7.2%, and pure motor neuropathy in 3.6%. Nerve and muscle pathological studies dismissed inflammatory abnormalities. The identification of these conditions has important economic implications and may change the critically ill patients' prognosis.

Although there are now widely accepted diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) there are few epidemiological data. A prevalence study was performed in the four Thames health regions, population 14 049 850. The prevalence date was 1 January 1995. Data were from a national consultant neurologist surveillance programme and the personal case series of two investigators. A diagnosis of CIDP was made according to definite, probable, possible, or suggestive diagnostic criteria. A wide difference in prevalence rates between the four health regions was noted, probably due to reporting bias. In the South East Thames Region, from which the data were most comprehensive the prevalence for definite and probable cases was 1.00/100 000; the highest total prevalence (if possible and suggestive cases were included) would have been 1.24/100 000. On the prevalence date 13% of patients required aid to walk and 54% were still receiving treatment.

The Guillain-Barré's syndrome, or acute polyradiculoneuropathy, is a monophasic neurological disease affecting 50-100 persons a year in Norway. In addition to peripheral paresis, respiratory and autonomic disturbances may occur. We present 22 patients, mean age 34.8 years, including four children between four and six years of age, who all received plasma exchange treatment. All our patients reported symptoms of a modest infection average 19 days before the neurological symptoms appeared. All patients had walking difficulties, half of them were unable to walk without assistance. There were cranial nerve findings in 18 patients, and nine had autonomic disturbances when admitted. All except one had increased protein contents in the spinal fluid as well as pathological findings in electrophysiological investigations. They received on average 8.6 plasma exchanges. In spite of such treatment, the total mortality rate has not decreased substantially. Two of our patients died, and three developed severe permanent paresis. Further studies on pathogenesis will be required to increase treatment success.

To estimate the incidence of Guillain-Barré syndrome (GBS) following Campylobacter jejuni infection (CI) we studied three populations where outbreaks of CI had occurred involving an estimated 8000 cases. No case of GBS was detected in the 6 months following the outbreaks in the local populations. The point estimate for the risk of GBS following CI estimated in this study was 0 in 8000 (95% confidence interval 0-3).

Background: The number of reports of influenza-vaccine-associated Guillain-Barré syndrome to the national Vaccine Adverse Event Reporting System increased from 37 in 1992-1993 to 74 in 1993-1994, arousing concern about a possible increase in vaccine-associated risk., Methods: Patients given a diagnosis of the Guillain-Barré syndrome in the 1992-1993 and 1993-1994 influenza-vaccination seasons were identified in the hospital-discharge data bases of four states. Vaccination histories were obtained by telephone interviews during 1995-1996 and were confirmed by the vaccine providers. Disease with an onset within six weeks after vaccination was defined as vaccine-associated. Vaccine coverage in the population was measured through a random-digit-dialing telephone survey., Results: We interviewed 180 of 273 adults with the Guillain-Barré syndrome; 15 declined to participate, and the remaining 78 could not be contacted. The vaccine providers confirmed influenza vaccination in the six weeks before the onset of Guillain-Barré syndrome for 19 patients. The relative risk of the Guillain-Barré syndrome associated with vaccination, adjusted for age, sex, and vaccine season, was 1.7 (95 percent confidence interval, 1.0 to 2.8; P=0.04). The adjusted relative risks were 2.0 for the 1992-1993 season (95 percent confidence interval, 1.0 to 4.3) and 1.5 for the 1993-1994 season (95 percent confidence interval, 0.8 to 2.9). In 9 of the 19 vaccine-associated cases, the onset was in the second week after vaccination, all between day 9 and day 12., Conclusions: There was no increase in the risk of vaccine-associated Guillain-Barré syndrome from 1992-1993 to 1993-1994. For the two seasons combined, the adjusted relative risk of 1.7 suggests slightly more than one additional case of Guillain-Barré syndrome per million persons vaccinated against influenza.

We studied the relation between pregnancy and Guillain-Barré syndrome in the Swedish female population ages 15-49 years during the period 1978-1993. Person-based information from the national Hospital In-patient Registry on patients discharged with a diagnosis of Guillain-Barré syndrome was linked to data on pregnancy and delivery from the Swedish Medical Birth Registry. We validated coded Guillain-Barré syndrome diagnoses and the time periods of clinical onset for patients hospitalized with Guillain-Barré syndrome during pregnancy or during the first 90-day postpartum period. We compared the incidence of Guillain-Barré syndrome in women in different exposure categories related to pregnancy with that in women neither pregnant nor in the 90-day postpartum period. Poisson regression analysis yielded age-adjusted rate ratios of 0.89 [95% confidence interval (CI) = 0.52-1.53] for pregnant women, 1.37 (95% CI = 0.64-2.91) for women during the first 90 days, and 2.93 (95% CI = 1.20-7.11) during the first 30 days after delivery. Our results indicate that the risk of Guillain-Barré syndrome increases after delivery, particularly during the first 2 weeks postpartum.

The authors retrospectively reviewed Guillain-Barre syndrome (GBS) in 48 Thai children over a period of 20 years from 1970 to 1989. The clinical presentations of this syndrome were compared to those reported in previous studies of children in Western and Asian countries. Antecedent infection, including respiratory tract infection and nonspecific viral infection, in this group of patients is similar to previous studies in children. Cranial nerve involvement found in this study (45.8%) was higher than that in other studies. The outcome of GBS in Thai children in this study was not different from other reports even without IVIG administration. The authors emphasize that respiratory and supportive care are important in managing patients suffering from this syndrome.

We studied Guillain-Barré syndrome, affecting children 12 years old or less, throughout Kuwait, in the period between January 1, 1992, and March 31, 1997. Nineteen children had the diagnostic criteria of Guillain-Barré syndrome, with an overall annual incidence rate of 0.95/100,000 population at risk. Female patients outnumbered male patients with a sex ratio of 1.4:1. There was a clustering of cases in winter and spring and in the year 1996. The disease symptoms were relatively severe in our patients because only 16% (3 of 19) of them were able to walk at the height of their illness, whereas the rest were bed or chair bound or needed assisted ventilation. Two patients had the electrodiagnostic features of axonal neuropathy and both had residual deficits on follow-up, whereas the rest recovered fully. All the patients received intravenous immunoglobulin. The mean time to walk unaided was 23.5 days (range, 2-84 days) after intravenous immunoglobulin and excluding the two patients with axonal neuropathy, and full recovery was achieved in a mean time of 103 days (range, 30-300 days). Contrary to previous studies, we found no correlation between oral polio vaccine administration and Guillain-Barré syndrome in 2 successive years (1995 and 1996) during a nationwide campaign targeting children less than 5 years old.

Group A beta-hemolytic streptococcus causes immune-mediated disorders such as acute rheumatic fever and acute glomerulonephritis. We describe a second patient with Fisher's syndrome (FS) from whom beta-hemolytic streptococcus was isolated. We performed a study of the antecedent pharyngeal symptoms in FS and Guillain-Barré syndrome. Sore throat was statistically more frequent in FS (18/24 cases, 75%) than in Guillain-Barré syndrome (29/58 cases, 50%). In a series, however, the association of FS with group A streptococcal infection was not shown. Some patients may develop FS after group A streptococcal infection, but the bacterium is not a major antecedent agent in FS.

Guillain-Barré syndrome (GBS) is viewed as a reactive, self-limited, autoimmune disease triggered by a preceding bacterial or viral infection. Campylobacter jejuni, a major cause of bacterial gastroenteritis worldwide, is the most frequent antecedent pathogen. It is likely that immune responses directed towards the infecting organisms are involved in the pathogenesis of GBS by cross-reaction with neural tissues. The infecting organism induces humoral and cellular immune responses that, because of the sharing of homologous epitopes (molecular mimicry), cross-react with ganglioside surface components of peripheral nerves. Immune reactions against target epitopes in Schwann-cell surface membrane or myelin result in acute inflammatory demyelinating neuropathy (85% of cases); reactions against epitopes contained in the axonal membrane cause the acute axonal forms of GBS (15% of cases). Care for such patients may be challenging, yet the prognosis overall is favourable. Optimal supportive care and anticipation and prevention of complications are the mainstay of therapy. Admission to the intensive-care unit is necessary in 33% of patients who require intubation and assisted ventilation. Immunomodulation with infusions of IgG or plasma exchange treatments foreshorten the disease course.

Objectives: To estimate the incidence rate of Guillain-Barré syndrome variants in an unselected population and to describe their clinical features and prognosis., Methods: A two year prospective multicentre study on the incidence and prognosis of Guillain-Barré syndrome was performed in Emilia-Romagna, northern Italy (3,909,512 inhabitants). A surveillance system was instituted within the study area, which comprised all the neurological departments, private and public general hospitals, and practising neurologists. The international classification of diseases (ICD) codes 357.XX (any peripheral neuropathy) of hospital discharges were also reviewed., Results: Data were separately analysed for Miller Fisher syndrome and other Guillain-Barré syndrome variants. During the study period 18 patients with Guillain-Barré syndrome variants including seven with Miller Fisher syndrome were recruited; the incidence rates were 0.14/100000/year (95% confidence interval (95% CI) 0.07-0.25) for Guillain-Barré syndrome variants (excluding Miller Fisher syndrome) and 0.09/100000/year (95% CI 0.04-0.18) for Miller Fisher syndrome. Guillain-Barré syndrome variants alone (excluding Miller Fisher syndrome) accounted for 10.5% of total cases. Death and relapses were not found. Details of clinical, electrophysiological, and CSF findings of Guillain-Barré syndrome variants are provided., Conclusions: Guillain-Barré syndrome variants other than Miller Fisher syndrome, as obtained through a population based study, account for about 10% of total cases of Guillain-Barré syndrome and, as a whole, have a good prognosis. Their clinical features are heterogeneous; bifacial weakness (associated with other signs, mainly sensory disturbances) represents the most frequent finding.

Guillain-Barré syndrome (GBS) is the commonest cause of acute flaccid paralysis worldwide. Recent pathological and electrodiagnostic studies indicated that there are different patterns within this syndrome. The demyelinating pattern predominates in North America and Europe, whereas axonal variants of GBS occur more frequently in Northern China. Infection with Campylobacter jejuni is one of the most frequently recognized antecedent events in all variants of GBS. The lipopolysaccharides of these organisms share ganglioside-like epitopes with peripheral nerves, and patients with GBS have antiganglioside antibodies. These observations have given rise to the hypothesis that "molecular mimicry" is the immunopathogenic mechanism of injury to peripheral nerve fibers. With this hypothesis in view, we summarize our experience of GBS as it occurs in Northern China. To explore the role of molecular mimicry in this cohort we sought evidence of preceding Campylobacter infection and correlated this with clinical characteristics and antiganglioside serology. Based on our results we propose a sequence of pathogenic events leading to peripheral nerve injury in GBS.

The numbers of small rodents in northern Sweden fluctuate heavily, peaking every 3 or 4 years. We found that the incidence of Guillain-Barré syndrome and insulin-dependent diabetes mellitus, as well as the number of deaths caused by myocarditis, followed the fluctuations in numbers of bank voles, although with different time lags. An environmental factor, such as an infectious agent, has been suggested for all three diseases. We hypothesize that Guillain-Barré syndrome, myocarditis, and insulin-dependent diabetes mellitus in humans in Sweden are caused by one or more infectious agents carried by small rodents. Also, a group of novel picornaviruses recently isolated from these small rodents is being investigated as the possible etiologic agent(s).

A previously unrecognized sequelum of infection with Campylobacter jejuni, a common cause of gastroenteritis, is Guillain-Barré syndrome (GBS). GBS is the most common cause of acute neuromuscular paralysis; 30% to 40% of cases are preceded by C. jejuni infection. Both patient and bacterial characteristics likely play a role in the pathogenesis of C. jejuni-induced GBS. Molecular mimicry between the LPS of some campylobacters and structures present on the gangliosides of the peripheral nerve may explain how this acute infectious diarrheal illness triggers GBS.

A retrospective analysis of the incidence of Guillain-Barré syndrome (GBS) in Finland in 1981-1986 was carried out by careful examination of medical records identified from nationwide Hospital Discharge Register data based on a mean total population of 5 million people. Records from 247 patients fulfilled the accepted criteria of GBS corresponding to a mean annual incidence of 0.82 per 100,000 population. Monthly rates showed an increased incidence of GBS in March 1985, following by a few weeks the onset of the nationwide oral poliovirus vaccine campaign and partly overlapping it. Analysis of the time series in depth suggested, however, that a change point in the occurrence of GBS had already taken place before the oral poliovirus vaccine campaign. Widespread circulation of wild-type 3 poliovirus in the population immediately preceded the oral poliovirus vaccine campaign and the peak occurrence of GBS. These results demonstrate a temporal association between poliovirus infection, caused by either wild virus or live attenuated vaccine, and an episode of increased occurrence of GBS, but they cannot prove the suspected cause-effect relation between GBS and oral poliovirus vaccine administration.

The relationship between preceding infections and antibodies to glycolipids was investigated in 205 Japanese patients with Guillain-Barré syndrome (GBS). Serological evidence of recent Campylobacter jejuni (C. jejuni) infection was found in 45% of the patients, compared with 1% in healthy controls. In contrast, recent infection of cytomegalovirus (CMV), Mycoplasma pneumoniae (M. pneumoniae) and Epstein-Barr virus (EBV) was detected in only 5%, 2% and none of the patients, respectively. C. jejuni-associated GBS was more frequent in early spring than in other seasons. All stool specimens positive for C. jejuni isolation were obtained within 10 days after the onset of GBS symptoms. Of 13 C. jejuni isolates from GBS patients, 10 (77%) belonged to Penner serotype 19 (heat-stable, HS-19). Elevated titers of anti-GM1 antibody were found in 8 (80%) of 10 GBS patients whose C. jejuni isolates belonged to HS-19 and in none of those infected with non-HS-19 C. jejuni (P = 0.04), and in 49% of 92 patients with C. jejuni infection and 25% of patients without infection of C. jejuni, CMV, EBV, or M. pneumoniae (P = 0.0007). The frequencies of elevated antibody titers to GD1a, GD1b and GQ1b were also significantly higher in GBS patients associated with C. jejuni than those not associated with C. jejuni, CMV, EBV, and M. pneumoniae. GBS in Japan seems to be associated more frequently with C. jejuni and less frequently with CMV than in Europe and North America.

We performed a case-control study to investigate the association between Campylobacter jejuni (CJ) infection and Guillain-Barr| syndrome (GBS) or Miller-Fisher syndrome. We compared 60 cases with 109 hospital controls matched for age, gender, hospital and geographical location. To diagnose the CJ infection, we considered the association between serologic positivity for CJ and a previous diarrheal illness within 3 months of inclusion in the study. Fifteen percent of cases versus 5% of hospital controls had CJ infection (p < 0.003, OR = 3.96, 95% CI: 1.0817.85). However, CJ infection was related to GBS only if it occurred during the previous month (p< 0.001, OR = 7.29, 95% CI: 1.4371.28). No statistical differences were found between the cases who were positive for CJ infection and those who were negative for CJ infection when studied by stepwise multivariate logistic regression for age, gender, clinical and electrophysiological features and outcome. Recent CJ infection may be a risk factor for GBS.

In 1992, the USA embargo on Cuba was tightened through the passage of the Cuban Democracy Act (CDA) that explicitly restricts food and medical supplies. The embargo has contributed to cause a number of public health problems in Cuba including: (1) an epidemic of more than 50,000 cases of optic and peripheral neuropathies in 1992-1993, resulting from dietary deficiency; (2) an epidemic of esophageal stenoses in toddlers who inadvertently drank liquid lye as a result of a soap shortage for which liquid lye was substituted; (3) an outbreak of Guillain-Barré syndrome in Havana, in June and July 1994, resulting from water contamination due to lack of chemicals for water treatment to eliminate Campylobacter sp.; (4) outbreaks of self-inflicted disease and injuries caused by rioting among Cubans detained at the US Naval base at Guantánamo Bay, and (5) a decline in medical practice standards and public health indicators in Cuba resulting from the enactment of the CDA, documented by the American Public Health Association in 1993 and confirmed in March 1997 by the American Association for World Health. Despite this evidence, the Cuban embargo remains a politically sensitive subject in the USA, resistant to public health concerns, as evidenced by the recent passage of the Helms-Burton Act. The public health effects of the CDA need to be reviewed with possible revocation or at least modification.

Objectives: To determine the incidence, treatment, and outcome of Guillain-Barré syndrome in south east England., Methods: Patients presenting with confirmed Guillain-Barré syndrome between 1 July 1993 and 30 June 1994 were recruited via a voluntary reporting scheme coordinated by the British Neurological Surveillance Unit, hospital activity data collected from acute admitting hospitals within the South East and South West Thames Regional Health Authorities, death certificates, and a contemporary research study of Guillain-Barré syndrome and Campylobacter jejuni infection. All patients were followed up for one year to determine outcome., Results: Seventy nine patients were recruited, 35 (44%) male, 44 (56%) female, including three children (two boys, one girl). The crude (95% confidence interval (95% CI)) annual incidence was 1.2 (0.9-1.4) cases/100000 population and 1.5 (1.3-1.8)/100000 when adjusted for undetected cases. Twenty (25%) patients required ventilation for an average (SD) of 42 (64) days. Thirty six (46%) patients received intravenous human immunoglobulin, five (6%) received plasma exchange, 11 (14%) both treatments, three (4%) steroids, and 25 (32%) no immunomodulatory treatment. One year later, six patients (8%) had died, all of whom were older than 60, three (4%) remained bedbound or ventilator dependent, seven (9%) were unable to walk unaided, 14 (17%) were unable to run, and 49 (62%) had made a complete or almost complete recovery. Increasing age was significantly associated with a poorer outcome at one year., Conclusions: Despite the frequent use of modern immunomodulatory treatments Guillain-Barré syndrome still carries considerable morbidity and mortality.

Guillain-Barré syndrome (GBS) is an autoimmune disease characterized by acute neuromuscular paralysis. Of an estimated annual number of 2628-9575 US cases, 526-3830 are triggered by Campylobacter infection. Research objectives were to identify the lifetime consequences of GBS and, when possible, to quantify their economic burden. The cost-of-illness method was used to calculate annual societal resources spent on medical care and lost productivity due to illness or premature death from Campylobacter-associated GBS. Estimated total costs (in US$) of Campylobacter-associated GBS ($0.2-$1.8 billion) were added to previously estimated costs of campylobacteriosis ($1.3-$6.2 billion) for a total annual cost from Campylobacter of $1.5-$8.0 billion (1995 dollars). It is concluded that up to $8.0 billion in US human illness costs are spent annually because of Campylobacter infection. Economic evaluation of the other costs associated with GBS, such as physical and psychological costs, would increase these estimates.

Objectives: This study examined whether there is a risk that tetanus-toxoid-containing vaccines could cause Guillain-Barré syndrome and, if so, how large the risk is., Methods: This study was based on previous active surveillance epidemiological studies of Guillain-Barré syndrome and vaccination history., Results: A background rate of 0.3 cases of Guillain-Barré syndrome per million person-weeks has been estimated. By chance, 2.2 people with the syndrome would have received tetanus-toxoid-containing vaccine within the 6 weeks before onset, yet only 1 person had done so. Data on children show similar results., Conclusions: If an association exists, it must be extremely rare and not of public health significance.

Guillain-Barré syndrome (GBS) is defined clinically as a peripheral neuropathy causing limb weakness that progresses for up to 4 weeks before reaching a plateau. The symptoms may be caused by inflammatory demyelination, axonal degeneration, or both. GBS occurs throughout the world, with a median incidence of 1.3 cases/100,000 population (range, 0.4-4.0). Males are more commonly affected than females, and there are peaks in young adults and the elderly. There is no clear seasonal association in Western countries, although this may be because the most frequent antecedent events, respiratory and enteric infections, have opposite seasonality. The most frequently identified cause of GBS is Campylobacter jejuni infection, which has been identified in up to 41% of patients and is associated with more severe disease and prolonged disability. Summer epidemics of GBS occur among children and young adults in Northern China and are particularly likely to be associated with C. jejuni infection.

Over a 20-month period, 3 adult and 6 pediatric patients were diagnosed with Guillain-Barré syndrome (GBS) at Groote Schuur and Red Cross Hospitals in Cape Town. All 9 GBS patients had Campylobacter jejuni biotype 2, serotype O:41 in their stools. C. jejuni infection was confirmed by ELISA testing of patient sera. Strains of this sero-biotype are rare: Only 12 such strains, including the GBS-associated strains, were recognized among 776 Campylobacter strains isolated and identified at Red Cross Hospital from March 1994 to October 1995. This is the first known association of C. jejuni biotype 2, serotype O:41 with GBS. Patients infected with this Campylobacter strain had a particularly severe form of the infection, requiring hospitalization and ventilation much longer than GBS patients infected with other Campylobacter species and patients with Campylobacter-negative stools. The O:41 Campylobacter isolates from the GBS patients are identical by phenotypic, serologic, and molecular criteria, and they are clonal.

Guillain-Barré syndrome (GBS), a neurologic disease that produces ascending paralysis, affects people all over the world. Acute infectious illnesses precede 50%-75% of the GBS cases. Although many infectious agents have been associated with GBS, the strongest documented association is with Campylobacter infection. The first line of evidence supporting Campylobacter infection as a trigger of GBS is anecdotal reports. The second line of evidence is serologic surveys, which have demonstrated that sera from GBS patients contain anti-Campylobacter jejuni antibodies, consistent with recent infection. Finally, culture studies have proven that a high proportion of GBS patients have C. jejuni in their stools at the time of onset of neurologic symptoms. Neurologic symptoms are more severe and more likely to be irreversible when GBS is preceded by C. jejuni infection. One of every 1058 Campylobacter infections results in GBS, and 1 of 158 Campylobacter type O:19 infections results in GBS.

Serologic evidence of recent Campylobacter jejuni infection was found in 92 (45%) of 205 Japanese patients with Guillain-Barré syndrome (GBS), and 49% of those 92 patients also had antibodies to GM1. Sixteen independent clinical isolates from GBS patients were serotyped: 12 belonged to Penner's heat-stable (HS) O serotype HS-19, 3 to HS-2, and 1 to HS-4. Of the patients whose C. jejuni isolates belonged to HS-19, 80% had elevated anti-GM1 antibodies. Although the correlation was significant between C. jejuni and GM1 antibody, anti-GM1 also was detected in 25% of patients without C. jejuni infection. Polymerase chain reaction-based restriction fragment length polymorphism analysis of an flaA gene showed that all HS-19 isolates, regardless of a GBS association, had an identical and distinguishable pattern, Cj-1, suggesting that HS-19:Cj-1 isolates are distinctive among C. jejuni isolates. Lectin typing showed that all GBS-associated HS-19 isolates contained terminal beta-N-acetylglucosamine residues on their cell surface, but HS-19 isolates from patients with enteritis did not.

Antecedent Campylobacter jejuni infection, detected by serologic tests, has been implicated in some acute immune polyneuropathies (AIP). Antibodies to Helicobacter pylori, C. jejuni, and GM1 ganglioside were measured in sera from 35 Chinese patients with AIP. Anti-GM1 antibodies were found in 54% of C. jejuni-seropositive, H. pylori-seronegative patients. In contrast, anti-GM1 antibodies were rare in sera that were either seropositive for both C. jejuni and H. pylori (P = .04) or seronegative for C. jejuni (P = .01). Motor axonal AIP was more common in the C. jejuni-seropositive, H. pylori-seronegative patients (82%) than in the bacterial antibody-negative group (38%). It was concluded that in AIP patients, C. jejuni-positive sera may be polyreactive, in that it may also react with H. pylori. In this situation, the specificity for either infection requires further validation. In contrast, sera with specific C. jejuni seropositivity are associated with both motor axonal AIP and selective serum IgG anti-GM1 antibodies.

Objective: To identify clinical characteristics of various forms of Guillain-Barré syndrome in Taiwan., Methods: The clinical and electrophysiological data of 167 consecutive patients with Guillain-Barré syndrome admitted to Chang Gung Memorial Hospital, a general paediatric and adult hospital in Taiwan, were reviewed., Results: Analysis of age distribution disclosed a high incidence (21%) among patients under the age of 10 years. Seasonal preponderance in Spring (March to May) was found. Utilizing clinical and electrophysiological data, these 167 patients with Guillain-Barré syndrome were subclassified; 82 (49%) had acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 32 (19%) had Fisher syndrome (FS), and six (4%) had axonal forms of Guillain-Barré syndrome. The remaining 47 (28%) patients were unclassified. Patients with AIDP and FS had many common clinical features, including seasonal distribution, history of preceding illness, sensory abnormalities, cranial nerve involvement except for extraocular motor nerves, and albuminocytological dissociation on examination of CSF. Follow up study on 145 patients disclosed that 127 (87%) recovered satisfactorily, 14 (10%) were persistently disabled, and four (3%) died during admission to hospital. Clinical features associated with poor outcome (persistent disability or death) were requirement for mechanical ventilation, a low mean compound muscle action potential amplitude (< or = 10% of the lower limit of normal), and age greater than 40 years., Conclusion: Guillain-Barré syndrome in Taiwan showed a peculiar age and seasonal distribution and a high frequency of FS not seen in other series. Given that patients with AIDP and FS had many common clinical features, AIDP and FS may have similar underlying pathological mechanisms.

Peripheral neuropathy is associated with HIV infection. The prevalence and types of peripheral neuropathy encountered in a randomly-selected HIV infected African population at different stages of disease were investigated. HIV positive individuals were categorized into 1 of 3 groups: asymptomatic, symptomatic and AIDS. HIV negative individuals formed the control group. Nerve conduction data were obtained using standard electrophysiological procedures and CD4+ levels were measured. The type of neuropathy was determined from the history, clinical presentation and electrophysiological abnormalities. The prevalence of peripheral neuropathy was 44%: subclinical neuropathy (SCN) accounted for 56%, acute inflammatory demyelinating polyneuropathy (AIDP) for 15% and distal symmetrical polyneuropathy (DSPN) for 22% of cases of neuropathy. SCN was found in all categories whereas AIDP predominated in the symptomatic category and DSPN in individuals with AIDS. The pattern and frequency of neuropathies seen in our African population is similar to that reported from other continents.

A retrospective study was carried out in the Ferrara Local Health District, Italy, for the period 1981-1993 (average resident population: 177,235 inhabitants) to establish whether people exposed to exogenous gangliosides had a higher risk of Guillain-Barré syndrome. The incidence of Guillain-Barré syndrome of 1.9/100,000 population/year [95% confidence interval (CI): 1.3-2.5] reported in Ferrara Local Health District in the same period was used as a reference for comparison. The data bank of Ferrara Local Health District made it possible, first to estimate the number of individuals exposed to gangliosides in the resident population of Ferrara Local Health District (3.7%), the number of ganglioside prescriptions and the number of cases of Guillain-Barré syndrome who had treatment with gangliosides (nine patients, 20.9%), and, secondly, to verify the sequence of events between the ganglioside injection and the onset of the disease. Seven of the nine patients (77.8%) received gangliosides as treatment for peripheral neuropathy (Guillain-Barré syndrome onset before gangliosides were prescribed). For the other two patients (22.2%) a possible appropriate temporal sequence between ganglioside injection and onset of Guillain-Barré syndrome was found. Based on two possible ganglioside-related cases, the risk of Guillain-Barré syndrome was higher in the exposed (0.53/100,000 population/month following ganglioside injection; 95% CI: 0.06-1.91) compared with the unexposed population, but the difference was not significant. When only individuals prescribed with mixed gangliosides were considered (both possible ganglioside-related Guillain-Barré syndrome cases received mixed gangliosides), the risk of Guillain-Barré syndrome was higher (0.64/100,000 population/month following ganglioside injection; 95% CI: 0.08-2.31) but the difference from the risk in unexposed individuals was not statistically significant. The relative risk for the exposure to mixed gangliosides was borderline (relative risk = 4.3; 95% CI: 1.0-17.8). The wide 95% confidence intervals were a consequence of sample size limitations. Considering also that the exposed and unexposed groups differed in age (those exposed were older than those unexposed and the age-specific incidence of Guillain-Barré syndrome in the study population increased with increasing age), the present findings question either a strong increased risk of Guillain-Barré syndrome in people exposed to exogenous gangliosides or an immunogenic role of these agents in humans. However, because of the limited sample size, the results are not conclusive.

Aim: To estimate the financial impact of campylobacter infection in Canterbury and New Zealand in 1995., Methods: The records of Canterbury Health Laboratories and hospital notes were searched to locate all inpatients admitted with campylobacteriosis in Christchurch in 1995 and to determine the costs involved. Further estimates of costs of outpatient care and costs due to lost productivity were made using local notification data. The computer database was searched for all cases of Guillain-Barré syndrome and reactive arthritis in 1995, and an estimate made of the cost attributable to campylobacter., Results: There were 65 admissions for campylobacteriosis at a cost of $92,262. Notified cases in general practice were estimated to cost $61,911 and loss of work an additional $338,499. Of the eight admissions with Guillain-Barré syndrome in 1995, the estimated cost of cases due to campylobacter infection was $89,522. Inpatient costs of reactive arthritis were negligible. Overall costs in Canterbury in 1995 amounted to $582,194, with a corresponding national figure of $4.48 million. The true cost of campylobacter infection may be up to ten times higher., Conclusions: The current high incidence of campylobacter infection has considerable financial impact on the community. Further public health and research initiatives to decrease incidence and associated costs are encouraged.