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Biomedical subjects

F G van der Meché

Publications and source records attributed to F G van der Meché.

At least 19 recordsLinked to original sources

Differential immune response to gangliosides in Guillain-Barré syndrome patients from Japan and The Netherlands.

Anti-ganglioside antibodies are consistently found in Guillain-Barré syndrome (GBS) patients from different geographical parts of the world. Several studies indicated differences in relative frequencies of anti-ganglioside reactivity and isotype distribution between GBS patients from Asia and from Europe. We investigated antibody reactivity against the gangliosides GM1, GM1b and GalNAc-GD1a in GBS patients from Japan and The Netherlands in two different laboratories. The proportion of GBS patients with anti-ganglioside antibodies did not differ between the two countries. GBS patients from The Netherlands more frequently had cross-reacting anti-GalNAc-GD1a/anti-GM1b antibodies and a stronger IgM anti-ganglioside response. Our findings indicate that geographical determined factors, dependent on either the host or the triggering infectious agent, determine the isotype distribution and fine specificity of anti-ganglioside antibodies in GBS patients.

Adolescent↗

Gastroenteritis-associated Guillain-Barré syndrome on the Caribbean island Curaçao.

BACKGROUND: The number of patients with Guillain-Barré syndrome (GBS) who have been observed in Curaçao, the Netherlands Antilles, may be increasing. METHODS: Clinical and serologic data were obtained from records of patients admitted between 1987 and 1999 and fulfilling National Institute of Neurological and Communicative Disorders and Stroke criteria for GBS. When possible, serum and stool samples were collected. The results were compared with a large Dutch epidemiologic study. RESULTS: The authors identified 49 patients, an overall crude incidence rate (IR) in Curaçao of 2.53/100,000 inhabitants (95% CI 1.87 to 3.35) (Dutch study 1.18, rate ratio (RR) of 2.14, p < 0.001). The IR in Curaçao increased from 1.62 in 1987 to 1991 to 3.10 in 1992 to 1999, RR 5.22 (95% CI 2.48 to 10.2, p = 0.02). The IR showed a curvilinear shape within a year. In comparison with the Dutch group, patients from Curaçao had a more severe course of the disease, with a mortality rate of 23% (3.4% in the Dutch group, p < 0.001), a higher percentage of preceding gastroenteritis (p < 0.001), and less sensory involvement (p < 0.001). In 8 of 10 serum samples, evidence was found for a recent infection with Campylobacter jejuni. CONCLUSIONS: The authors found a steady increase in incidence of GBS over the years in association with a more pronounced seasonal preponderance and a more severe course. The clinical characteristics suggest a role for C jejuni.

Campylobacter Infections↗

Long-term sensory deficit after Guillain-Barré syndrome.

In order to document the sensory deficit still present several years after onset of Guillain-Barré syndrome (GBS) and to determine if the sensory residua have a disrupting effect on daily life, 122 patients were asked to cooperate in a neurological examination and to complete a questionnaire three to six years after onset. On functional assessment 84 patients had no or only minor neurological symptoms or signs, 24 patients showed moderate recovery and 14 patients were left with severe residual signs. On neurological examination, residual sensory deficit was found in the arms of 38 % of the patients and in the legs of 66 % of the patients. Sensory disturbance was experienced as moderate to severe in the arms of 27 % of the patients and in the legs of 40 % of the patients. Muscle aches and cramps were still present in 48 %. There was a statistically significant relation between muscle aches and cramps and objective residual sensory deficit but not with residual weakness. Furthermore, in the group of patients with a pure motor GBS, significantly fewer people suffered from muscle aches and cramps than in the remaining patients (p=0.04). Twenty-five percent of patients changed jobs after their illness, and 44% gave up some leisure activities. It can be concluded that many patients still suffer from sensory deficit, and a considerable number experience these as moderately to seriously disruptive, especially in the legs. Muscle aches and cramps seems to be related to sensory rather than motor dysfunction.

Activities of Daily Living↗

Risk factors for the development of polyneuropathy and myopathy in critically ill patients.

BACKGROUND: Previously, mainly retrospective and a few important prospective studies postulated the role of sepsis or systemic inflammatory response syndrome (SIRS), multiple organ failure, and the use of medication as causative factors for the development of critical illness polyneuropathy and myopathy (CIPNM). This study aimed to identify the risk factors in the development of CIPNM. METHODS: Prospectively, we studied 98 patients who were on artificial respirators for the development of CIPNM. The Acute Physiology and Chronic Health Evaluation (APACHE) III score, presence of SIRS, and sepsis severity score at entry; the dosage of midazolam, vecuronium, and steroids at entry and day 7 of artificial respiration; and the use of aminoglycosides at entry were related with time to CIPNM or time of last follow-up. The Kaplan-Meier method and log-rank test were used. RESULTS: Thirty-two patients (33%) developed CIPNM. After multivariate analysis, it was found that the APACHE III score and the presence of SIRS were significantly related with risk for the development of CIPNM. No significant relation was found for the use of midazolam, vecuronium, or steroids. Based on a risk index from a Cox regression model with APACHE III score and presence of SIRS as outcomes, three groups could be constructed with low-, medium-, and high-risk patients for the development of CIPNM. CONCLUSIONS: The APACHE III score, a quantitative index of disease severity based on clinical and laboratory physiologic data, is a valuable predictor for the development of CIPNM in patients in the intensive care unit. Together with the presence of SIRS, it can be used to estimate the risk of developing CIPNM for patients on artificial respirators.

APACHE↗

Guillain-Barré syndrome- and Miller Fisher syndrome-associated Campylobacter jejuni lipopolysaccharides induce anti-GM1 and anti-GQ1b Antibodies in rabbits.

Campylobacter jejuni infections are thought to induce antiganglioside antibodies in patients with Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) by molecular mimicry between C. jejuni lipopolysaccharides (LPS) and gangliosides. We used purified LPS fractions from five Campylobacter strains to induce antiganglioside responses in rabbits. The animals that received injections with LPS from GBS-associated strains developed anti-GM1 and anti-GA1 antibodies. Animals injected with LPS from one MFS-related C. jejuni strain produced anti-GQ1b antibodies. Rabbits that were injected with Penner O:3 LPS had a strong anti-LPS response, but no antiganglioside reactivity was observed. The antiganglioside specificity in the rabbits reflected the specificity in the patients from whom the strains were isolated. In conclusion, our results indicate that an immune response against GBS- and MFS-associated C. jejuni LPS results in antiganglioside antibodies. These results provide strong support for molecular mimicry as a mechanism in the induction of antiganglioside antibodies following infections.

Adult↗

Comparative study of preceding Campylobacter jejuni infection in Guillain-Barré syndrome in Japan and The Netherlands.

A comparative study was made in Japan and The Netherlands of the presence of preceding Campylobacter jejuni infections in Guillain-Barré syndrome (GBS). It was conducted in two laboratories using different serological criteria. The Japanese results showed no significant difference in the frequency of C jejuni infection between the Japanese (17/88, 19%) and Dutch (21/132, 16%) patients with GBS. The Dutch investigation showed a higher frequency in Dutch patients (45/132; 34%) than in Japanese patients(20/88; 23%), but the difference did not reach significance. Although the frequencies of preceding C jejuni infection have been reported to be higher in Asian countries than in western countries, the findings of this collaborative study show that the incidence of antecedent C jejuni infection in GBS in Japan is not higher than in The Netherlands and that serological assays vary considerably between laboratories.

Campylobacter Infections↗

A case of Guillain-Barré syndrome following a family outbreak of Campylobacter jejuni enteritis.

We describe an outbreak of Campylobacter jejuni enteritis involving three family members of whom one developed Guillain-Barré syndrome (GBS). The patients' serum reacted strongly with several gangliosides and with the lipopolysaccharide (LPS) fractions from the C. jejuni strains isolated from his family members. Only low titer anti-ganglioside antibodies were found in his siblings. HLA-typing did not indicate a locus associated with auto-antibody production. Comparing the immune response in GBS patients and C. jejuni enteritis patients can be of great value in determining the additional factors that lead to post-Campylobacter GBS. Ganglioside mimicry alone is necessary but not sufficient for the induction of anti-ganglioside antibodies. Other susceptibility factors are required to induce an anti-neural immune response.

Adult↗

Critical illness polyneuropathy and myopathy (CIPNM): evidence for local immune activation by cytokine-expression in the muscle tissue.

In a longitudinal prospective study a muscle biopsy was taken from 30/32 (33%) of the 98 patients who developed critical illness polyneuropathy and myopathy (CIPNM). Neuropathic changes were found in 37%, myopathic in 40%, and a combination in 23% of the biopsies. The immunohistopathology showed macrophages and Th-cells in 40% and 60% of the muscle biopsies respectively. Small mainly perivascular infiltrates contained macrophages and Th-cells. ICAM-1, VCAM and MAC were found on the vascular endothelium in 58%, 53% and 79% respectively. In all biopsies there was an upregulation of both HLA-I and HLA-DR. Proinflammatory cytokines and TNFalphaR75 were also produced locally (IL-1beta in 71%, IFN-gamma in 40%, IL-12 in 73%, TNFalphaR75 in 90%). The anti-inflammatory cytokine IL-10 was simultaneously expressed in 96% of the biopsies. HLA-DR, TNFalphaR75 and IL-10 differed significantly when compared with control muscle biopsies. Our data provide evidence that small numbers of activated leukocytes producing both pro- and anti-inflammatory cytokines infiltrate skeletal muscle of CIPNM patients. We propose that the local balance of leukocyte activities is of importance in the pathophysiology of muscle weakness in CIPNM.

Antigens, CD↗

Campylobacter jejuni lipopolysaccharides from Guillain-Barré syndrome patients induce IgG anti-GM1 antibodies in rabbits.

Lipopolysaccharides (LPS) from Campylobacter jejuni strains isolated from patients with Guillain-Barré syndrome (GBS) display molecular mimicry with GM1. We immunized rabbits with C. jejuni LPS from GBS-associated strains containing a GM1-like epitope. All animals produced high titre anti-LPS antibodies that were cross-reactive with GM1. We conclude that C. jejuni strains from GBS patients are able to induce antibodies that cross-react with gangliosides and LPS. This study further confirms the role of molecular mimicry in the induction of anti-ganglioside antibodies in GBS patients.

Animals↗

Cross-reactive antibodies against GM2 and CMV-infected fibroblasts in Guillain-Barré syndrome.

OBJECTIVE: To investigate whether anti-GM2 antibodies in patients with Guillain-Barré syndrome (GBS) are induced by molecular mimicry with cytomegalovirus (CMV). BACKGROUND: Antibodies against ganglioside GM2 are frequently present in the serum from GBS patients with an antecedent infection with CMV. METHODS: The authors detected inhibition of anti-GM2 reactivity after incubation of GM2-reactive serum samples with fibroblasts infected with a GBS-associated CMV strain. Control sera consisted of GQ1b-reactive samples, and control antigens included uninfected fibroblasts and fibroblasts that were infected with other herpes viruses. RESULTS: Serum immunoglobulin M reactivity with GM2 was decreased in a dose-dependent manner after incubation with CMV-infected fibroblasts. Incubation of anti-GM2-positive serum samples with uninfected fibroblasts and fibroblasts infected with varicella zoster virus did not inhibit anti-GM2 reactivity, whereas this reactivity was slightly decreased after incubation with herpes simplex virus type 1 in one patient. Antibodies against ganglioside GQ1b did not react with CMV-infected fibroblasts. CONCLUSIONS: CMV-infected fibroblasts express gangliosidelike epitopes that recognize specifically anti-GM2 antibodies. These results support the hypothesis that antiganglioside antibodies in CMV-infected GBS patients are induced by molecular mimicry between GM2 and antigens that are induced by a CMV infection.

Adult↗

Psychometric evaluation of a new sensory scale in immune-mediated polyneuropathies. Inflammatory Neuropathy Cause and Treatment (INCAT) Group.

OBJECTIVE: To perform a psychometric evaluation of the inflammatory neuropathy cause and treatment (INCAT) sensory sumscore (ISS) in sensory-motor immune-mediated polyneuropathies. This new sensory scale was evaluated to strive for uniformity in assessing sensory deficit in these disorders. METHODS: The ISS comprises vibration and pinprick sense plus a two-point discrimination value and ranges from 0 (normal sensation) to 20 (maximum sensory deficit). Before its clinical use, a panel of expert neurologists concluded that the ISS has face and content validity. The construct validity of the ISS was investigated by correlation and regression studies with additional scales (Nine-Hole Peg Test, 10-Meter Walking Test, a disability sumscore). All scales were applied in 113 patients with a stable neurologic condition (83 patients who experienced Guillain-Barre syndrome [GBS] in the past, 22 with chronic inflammatory demyelinating polyneuropathy [CIDP], 8 patients with a monoclonal gammopathy associated polyneuropathy), and 10 patients with recently diagnosed GBS or CIDP with changing clinical conditions. Reliability of the ISS was evaluated in the stable patients. Its responsiveness was investigated in the patients examined longitudinally. RESULTS: A moderate to good validity was obtained for the ISS (stable group: r = 0.38 to 0.56, p < or = 0.006; longitudinal group: R = 0.60 to 0.82, p < or = 0.007, except for the association with the 10-Meter Walking Test [p = 0.08]). Acceptable internal consistency, and inter- and intraobserver reliability were demonstrated for the ISS (alpha = 0.68 to 0.87; R = 0.85 to 0.89, p < 0.0001). Standardized response mean scores for the ISS were high (> or =0.8), indicating good responsiveness. CONCLUSIONS: All psychometric requirements are provided for the the inflammatory neuropathy cause and treatment sensory sumscore. The use of this scale is therefore suggested for bedside evaluation of sensory deficit in the individual patient with a sensory-motor immune-mediated polyneuropathy as well as in clinical trials.

Adolescent↗

Corticosteroids for treating Guillain-Barré syndrome.

BACKGROUND: The cause of Guillain-Barré syndrome (GBS) is inflammation of the peripheral nerves which corticosteroids would be expected to benefit. OBJECTIVES: To examine the efficacy of corticosteroids in hastening recovery and reducing the long term morbidity from Guillain-Barré syndrome (GBS). SEARCH STRATEGY: Search of the Cochrane Neuromuscular Disease Group register for randomised trials and enquiry from authors of trials and other experts in the field. SELECTION CRITERIA: Types of studies: quasi-randomised or randomised controlled trials TYPES OF PARTICIPANTS: patients with GBS of all ages and all degrees of severity Types of interventions: any form of corticosteroid or adrenocorticotrophic hormone Types of outcome measures: Primary: improvement in disability grade on a commonly used seven point scale four weeks after randomisation Secondary: time from randomisation until recovery of unaided walking, time from randomisation until discontinuation of ventilation (for those ventilated), mortality, proportion of patients dead or disabled (unable to walk without aid) after 12 months, improvement in disability grade after six months, improvement in disability grade after 12 months, proportion of patients who relapse, and proportion of patients with adverse events related to corticosteroid treatment DATA COLLECTION AND ANALYSIS: We identified six randomised trials. One author extracted the data and the other checked them. We obtained some missing data from investigators. MAIN RESULTS: The six eligible trials included a total of 195 corticosteroid treated patients and 187 control subjects. One trial of intravenous methylprednisolone accounted for 243 of the total 382 subjects studied (63%). This trial did not show a significant difference in any disability related outcome between the corticosteroid and placebo groups. There was no significant difference between the corticosteroid and control groups for the primary outcome measure, improvement in disability grade four weeks after randomisation. The weighted mean difference of the three trials for which this outcome was available showed no difference. The actual figure was 0.01 (95% CI -0.27 to 0.29) grade in favour of the corticosteroid group. There was also no significant difference between the groups for most of the secondary outcome measures. However in the largest trial hypertension developed less often in the intravenous methylprednisolone group (2/124, 1.6%) than in the control group (12/118, 10.2%), a significant difference in favour of corticosteroid treatment (relative risk 0.20, 95% CI 0.04 to 0.66). REVIEWER'S CONCLUSIONS: Corticosteroids should not be used in the treatment of Guillain-Barré syndrome. If a patient with Guillain-Barré syndrome needs corticosteroid treatment for some other reason its use will probably not do harm. The effect of intravenous methylprednisolone combined with intravenous immunoglobulin in Guillain-Barré syndrome is being tested with a randomised trial.

Anti-Inflammatory Agents↗

Guidelines for autologous blood and marrow stem cell transplantation in multiple sclerosis: a consensus report written on behalf of the European Group for Blood and Marrow Transplantation and the European Charcot Foundation. BMT-MS Study Group.

Recent reports suggest the possible beneficial effects of haemopoietic stem cell transplantation (HSCT) in autoimmune diseases such as multiple sclerosis (MS). The definition of the risk/benefit ratio for such a treatment is perceived as a major issue for the neurological community worldwide. The First Consensus Conference on Bone Marrow Transplantation in Patients with Multiple Sclerosis was held in Milan, Italy on 21 February 1998. Participants from 16 European, North American, and South American countries discussed the guidelines for performing HSCT in MS. This conference was organized in order to: (a) define criteria for patient selection; (b) define transplantation procedures to maximize efficacy of the treatment and minimize its toxicity; (c) standardize patient outcome evaluation; and (d) establish an international working group to evaluate the efficacy and safety of HSCT in MS and to study the immunological changes related to HSCT in MS patients. During the meeting in Milan agreement was reached on: (a) the preparation and distribution of a consensus report on HSCT in MS and (b) the design of an open trial for an initial assessment of the safety and efficacy of HSCT in MS. The consensus reached during the meeting and the design of the clinical trial are summarized in this contribution.

Blood Transfusion, Autologous↗

Long-term results of uvulopalatopharyngoplasty for obstructive sleep apnea syndrome.

OBJECTIVES: Assessment of the long-term effect of uvulopalatopharyngoplasty (UPPP) on snoring, excessive daytime sleepiness, and nocturnal oxygen desaturation index (ODI) in patients with obstructive sleep apnea syndrome. STUDY DESIGN: Evaluation of snoring, excessive daytime sleepiness, and ODI in patients treated by UPPP earlier. MATERIALS AND METHODS: Patients (n = 58) with a follow-up period of 11 to 74 months (median, 34 mo) were included in this study. Snoring and excessive daytime sleepiness were scored on specially designed semiquantitative scales. In all patients ODI was calculated from pulse-oximetry combined with polysomnography at base line and by polygraphy (MESAM 4) during follow-up in 38 patients. Long-term response was compared with 6-month response in the same cohort. RESULTS: There was a long-term improvement of snoring in 63% of patients, no change in 23%, and a deterioration in 14% (P < .00001). Overall snoring increased slightly between 6 months and long-term follow-up. There was an improvement of excessive daytime sleepiness in 38%, no change in 27%, and a deterioration in 35% (P = .80). Excessive daytime sleepiness showed a relapse to preoperative levels between 6 months and long-term follow-up. The median improvement of ODI was -1 (95% interpercentile range, 73-51) and was not significant (P = .35). In 5 of 13 patients in whom ODI at baseline exceeded 20, ODI was reduced to less than 20. In 4 of the 38 patients ODI was reduced to less than 5. The improvement of ODI decreased significantly between 6 months and long-term follow-up (P = .03). No relation was found between body mass index, Mueller maneuver, X-cephalometry, and long-term outcome. An additional finding was that the ODI decreased after UPPP in combination with tonsillectomy, compared with a slight increase after UPPP alone; the difference was significant (P = .008). CONCLUSION: The response to UPPP for obstructive sleep apnea syndrome decreases progressively over the years after surgery. UPPP in combination with tonsillectomy was more effective than UPPP alone.

Adult↗

Reliability and responsiveness of a graduated tuning fork in immune mediated polyneuropathies. The Inflammatory Neuropathy Cause and Treatment (INCAT) Group.

The interobserver and intraobserver reliability of the Rydel-Seiffer (RS) graduated tuning fork was evaluated in 113 patients with a clinically stable immune mediated polyneuropathy (83 patients who had had Guillain-Barré syndrome (GBS) in the past, 22 with a chronic inflammatory demyelinating polyneuropathy (CIDP), and eight with a polyneuropathy associated with a gammopathy of undetermined significance). Additionally, the responsiveness of this instrument was serially investigated in 20 patients with recently diagnosed GBS or CIDP and changing clinical conditions. The measures were done in triplicate at eight different locations in the limbs and the values were compared with the recently published vibration threshold reference values. Good interobserver and intraobserver agreements (quadratic weighted kappa=0.67-0.98) and high responsiveness values (standardised response mean scores>0.8) were demonstrated for the RS tuning fork. These results provide, in addition to literature findings, further evidence for incorporation of this easily applicable instrument in routine neurological examination.

Adolescent↗

Clinical features and response to treatment in Guillain-Barré syndrome associated with antibodies to GM1b ganglioside.

GM1b is a minor ganglioside in human peripheral nerves. Serum anti-GM1b antibodies frequently are present in patients with Guillain-Barré syndrome (GBS). In this collaborative study, we investigated the antecedent infections, clinical features, and response to treatment of GBS patients with anti-GM1b antibodies. Of 132 GBS patients who participated in the Dutch GBS trial that compared the effect of intravenous immunoglobulins and plasma exchange, 25 (19%) patients had anti-GM1b antibodies. IgM antibodies were present in 14, IgG antibodies in 15, and both isotypes in 4 patients. The 25 patients with anti-GM1b antibodies had a clinical pattern distinct from that of the other 107 GBS patients. They more often had an episode of gastrointestinal illness and frequently showed serological evidence of recent infection by Campylobacter jejuni. The anti-GM1b-positive subgroup was marked by more rapidly progressive, more severe, and predominantly distal weakness. Cranial nerve involvement and sensory deficits were less common in the patients with anti-GM1b antibodies. The presence of anti-GM1b antibodies was associated with slower recovery. The clinical manifestations predominantly were associated with anti-GM1b antibodies of the IgG isotype. Fourteen (56%) of the 25 patients with anti-GM1b antibodies also had anti-GM1 antibodies. The group of patients with both antibodies was clinically more homogeneous and had a more rapidly progressive, pure motor neuropathy. The subgroup of anti-GM1b-positive GBS patients responded well to treatment with immunoglobulins but not to plasmapheresis. The distinctive clinical features of the patients with anti-GM1b antibodies show that acute motor neuropathy represents a specific subgroup within GBS and that recognizing these patients may have consequences as to the choice of therapy.

Adolescent↗