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Biomedical subjects

J Meulstee

Publications and source records attributed to J Meulstee.

At least 19 recordsLinked to original sources
PubMed2005

HLA class II alleles are not a general susceptibility factor in Guillain-Barré syndrome.

OBJECTIVE: To assess whether human leukocyte antigen (HLA)-DRB1 and HLA-DQB1 alleles confer susceptibility to Guillain-Barre syndrome (GBS) or are related to specific clinical or serologic subgroups of GBS. METHODS: The HLA-DRB1 and HLA-DQB1 loci were genotyped by PCR amplification with sequence-specific primers in 164 well-documented Dutch patients with GBS and 207 healthy Dutch control subjects. Patients with GBS were divided into subgroups based on clinical features, severity of disease, antecedent infection, and anti-ganglioside antibodies. Data were compared with those of all case-control HLA studies in GBS performed previously. RESULTS: In this case-control study, HLA-DRB1 and HLA-DQB1 alleles did not differ between GBS patients and control subjects. The frequency of HLA-DRB1*01 was increased in patients who needed mechanical ventilation (odds ratio 4.2; 95% CI 1.9 to 9.6; p(c) = 0.02). Multivariate logistic regression analysis showed that this association was independent of the severity of paresis and the presence of cranial nerve involvement (all p < 0.05). There was a tendency toward an association between certain HLA alleles and several anti-ganglioside antibodies. CONCLUSIONS: Human leukocyte antigen (HLA) class II antigens are not a general susceptibility factor in Guillain-Barre syndrome (GBS). However, HLA class II alleles may be a determinant in distinct subgroups of GBS, indicating the need for further exploration in large-scale studies.

Adolescent↗
PubMed2004

Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial.

BACKGROUND: Despite available treatment with intravenous immunoglobulin (IVIg), morbidity and mortality are considerable in patients with Guillain-Barré syndrome (GBS). Our aim was to assess whether methylprednisolone, when taken with IVIg, improves outcome when compared with IVIg alone. METHODS: We did a double-blind, placebo-controlled, multicentre, randomised study, to which we enrolled patients who were unable to walk independently and who had been treated within 14 days after onset of weakness with IVIg (0.4 g/kg bodyweight per day) for 5 days. We assigned 233 individuals to receive either intravenous methylprednisolone (500 mg per day; n=116) or placebo (n=117) for 5 days within 48 h of administration of first dose of IVIg. Because age is an important prognostic factor, we split treatment groups into two age-groups-ie, younger than age 50 years, or 50 years and older. Our primary outcome was an improvement from baseline in GBS disability score of one or more grades 4 weeks after randomisation. Analysis was by intention to treat. FINDINGS: We analysed 225 patients. GBS disability scores increased by one grade or more in 68% (76 of 112) of patients in the methylprednisolone group and in 56% (63 of 113) of controls (odds ratio [OR] 1.68, 95% CI 0.97-2.88; p=0.06). After adjustment for age and degree of disability at entry, treatment OR was 1.89 (95% CI 1.07-3.35; p=0.03). Side-effects did not differ greatly between groups. INTERPRETATION: We noted no significant difference between treatment with methylprednisolone and IVIg and IVIg alone. Because of the relevance of prognostic factors and the limited side-effects of methylprednisolone, the potential importance of combination treatment with the drug and IVIg, however, warrants further investigation.

Adolescent↗
PubMed2002

Clinical value of electrodiagnostic testing following repair of peripheral nerve lesions: a prospective study.

This study prospectively assessed the level of correlation between functional and electrophysiological recovery after median and ulnar nerve lacerations. Motor and sensory recovery were recorded clinically and electrophysiologically every 3 months in 24 patients with 29 complete median or ulnar nerve lacerations. The strength of agreement between the clinical motor score and the electrophysiological score was "fair", but in 41% a discrepancy was identified (kappa factor 0.39). Regeneration was not detected earlier by electrophysiology than by a thoroughly performed clinical evaluation. This suggests that electrophysiologic testing is of value as an adjunct to clinical assessment for evaluating cases in which there is clinical doubt as to the progress of regeneration and secondary surgery is contemplated.

Adolescent↗
PubMed2001

What is the best way to assess focal slowing of the ulnar nerve?

OBJECTIVE: The study assessed the influence of the length of the across elbow (AE) segment of the ulnar nerve on the true and false positive rates of velocity measurements of the AE segment. Using a short AE length will increase effects of the measurement error (ME), and using a long distance will 'dilute' the slowing due to the focal lesion; it is not known which length is optimal to detect focal slowing. METHODS: A simulation was performed to assess diagnostic yield for AE lengths of 50, 100 and 150 mm, taking into account ME, variation in true velocity, and severity of the lesion. ME of latencies and distances were first determined in a healthy subject. RESULTS: ME proved lower than in a published study. Diagnostic yield was consistently better for an AE length of 50 mm than for 100 or 150 mm. The optimum length is therefore near 50 mm. Yield increased with severity of the lesion, smaller ME, and when interindividual variation in true velocity was small. Judging AE on its own had a slightly better yield than comparing AE velocity to forearm velocity, except for populations with a larger than normal spread in true conduction variability. CONCLUSIONS: The best balance between effects of ME and 'dilution' to detect focal nerve slowing is obtained at nerve lengths of about 50 mm. The need to incorporate all possible compression sites necessitates the use of a suboptimal length of about 80 mm.

Computer Simulation↗
PubMed2001

Diagnostic and classification criteria for the Guillain-Barré syndrome.

BACKGROUND: Diagnostic criteria for the Guillain-Barré syndrome (GBS) have been available since 1978. Since then, several variants have been described. More recently, a distinction has been made between pure motor forms, severe sensory forms, primary axonal and primary demyelinating varieties. Associations of clinical characteristics, and specific infections and the presence of antiganglioside antibodies have been found. For further studies on GBS, it is therefore necessary to reconsider the available diagnostic criteria and add additional criteria for subclassification. METHODS: A panel of (20) experts was formed. The literature representing the recent developments in GBS subclassification was reviewed. Following a consensus protocol, diagnostic and classification criteria were formulated. RESULTS: The diagnosis of GBS can usually be made on clinical characteristics. A schedule for subclassification has been made to cover also the clinical variants in a systematic manner.

Autoantibodies↗
PubMed2000

Changes in functional coupling between neural networks in the brain during maturation revealed by omega complexity.

To study age-dependent changes in coupling between cortical neural networks we applied a new method (omega complexity) to determine overall coherence of EEGs of 34 subjects ranging in age from 3 months to 16 years. We found that the functional coupling between different brain regions is low at birth and increases significantly in the first two decades of life. We suggest that this coupling depends critically upon the system of associational and callosal fibers which is unmyelinated at birth, and which only finishes myelinization in the second or third decade. Thus age-dependant changes in omega complexity may reflect maturation of brain structures underlying higher cerebral functions. If these results can be replicated, preferably in prospective, cohort rather than transectional type studies, omega complexity might prove to be clinically useful as an objective, quantitative measure of brain maturation.

Adolescent↗
PubMed1999

Prognostic factors of Guillain-Barré syndrome after intravenous immunoglobulin or plasma exchange. Dutch Guillain-Barré Study Group.

OBJECTIVE: To determine the influence of clinical, laboratory, and electrodiagnostic factors on the prognosis of Guillain-Barré syndrome (GBS). BACKGROUND: Identification of prognostic factors may lead to better selection of patients with a poor prognosis for new therapeutic trials. METHODS: The authors studied 147 patients with GBS who participated in the Dutch GBS trial comparing the effect of IV immunoglobulins with plasma exchange (PE). Outcome was measured at 8 weeks because half of the patients had recovered independent locomotion by then and at 6 months, the endpoint of the study. RESULTS: Multivariate logistic regression revealed the following factors predicting outcome (inability to walk independently) at 8 weeks: a preceding gastrointestinal illness (yes, no), age (> or =50, <50 years), Medical Research Council sum score (<40, > or =40) at the start of treatment, and-described for the first time-a recent cytomegalovirus (CMV) infection (yes, no). At 6 months, the same clinical factors were found, but an initial rapid progression of weakness also appeared to be a prognostic factor. Analysis of treatment interactions revealed that the effect of diarrhea was more pronounced in the PE-treated group. CONCLUSIONS: The main predictors of outcome in GBS are clinical factors. Diarrhea is an important poor predictor of outcome, especially for the PE-treated group, and a recent CMV infection predicts delayed early recovery.

Electromyography↗
PubMed1999

Chronic motor neuropathies: response to interferon-beta1a after failure of conventional therapies.

OBJECTIVES: The effect of interferon-beta1a (INF-beta1a; Rebif) was studied in patients with chronic motor neuropathies not improving after conventional treatments such as immunoglobulins, steroids, cyclophosphamide or plasma exchange. METHODS: A prospective open study was performed with a duration of 6-12 months. Three patients with a multifocal motor neuropathy and one patient with a pure motor form of chronic inflammatory demyelinating polyneuropathy were enrolled. Three patients had anti-GM1 antibodies. Treatment consisted of subcutaneous injections of IBF-beta1a (6 MIU), three times a week. Primary outcome was assessed at the level of disability using the nine hole peg test, the 10 metres walking test, and the modified Rankin scale. Secondary outcome was measured at the impairment level using a slightly modified MRC sumscore. RESULTS: All patients showed a significant improvement on the modified MRC sumscore. The time required to walk 10 metres and to fulfil the nine hole peg test was also significantly reduced in the first 3 months in most patients. However, the translation of these results to functional improvement on the modified Rankin was only seen in two patients. There were no severe adverse events. Motor conduction blocks were partially restored in one patient only. Anti-GM1 antibody titres did not change. CONCLUSION: These findings indicate that severely affected patients with chronic motor neuropathies not responding to conventional therapies may improve when treated with INF-beta1a. From this study it is suggested that INF-beta1a should be administered in patients with chronic motor neuropathies for a period of up to 3 months before deciding to cease treatment. A controlled trial is necessary to confirm these findings.

Adult↗
PubMed1998

Risk factors for treatment related clinical fluctuations in Guillain-Barré syndrome. Dutch Guillain-Barré study group.

The risk factors for treatment related clinical fluctuations, relapses occurring after initial therapeutic induced stabilisation or improvement, were evaluated in a group of 172 patients with Guillain-Barré syndrome. Clinical, laboratory, and electrodiagnostic features of all 16 patients with Guillain-Barré syndrome with treatment related fluctuations, of whom 13 were retreated, were compared with those who did not have fluctuations. No significant differences were found between patients with Guillain-Barré syndrome treated with plasma exchange and patients treated with intravenous immune globulins either alone or in combination with high dose methylprednisolone. None of the patients with Guillain-Barré syndrome with preceding gastrointestinal illness, initial predominant distal weakness, acute motor neuropathy, or anti-GM1 antibodies showed treatment related fluctuations. On the other hand patients with fluctuations showed a trend to have the fluctuations after a protracted disease course. It is therefore suggested that treatment related clinical fluctuations are due to a more prolonged immune attack. There is no indication that the fluctuations are related to treatment modality. The results of this study may help the neurologist to identify patients with Guillain-Barré syndrome who are at risk for treatment related fluctuations.

Disease Progression↗
PubMed1997

[A special case of 'deafness'; Landau-Kleffner syndrome].

A boy aged 5 had displayed from the age of 3 a fluctuating deficit in understanding spoken language. Audiometric testing proved his hearing to be normal. After 18 months, the parents noticed minor attacks of 'absence'. An EEG showed a normal background pattern and frequent spikes and spike waves complexes; CT-scanning of the brain revealed no abnormalities. On the basis of the acquired language disorder, the seizures and the features of the nocturnal EEG, the diagnosis the syndrome of Landau-Kleffner was made. After treatment with ethosuximide a temporary improvement of the aphasia occurred. A permanent improvement however was realized by treatment with prednisone. The boy could attend a primary school. The syndrome runs a fluctuating course; the patient may recover, but he may also undergo general mental deterioration. Antiepileptic agents only may have a brief favorable effect on the aphasia. In case of failure corticosteroids or even subpial cortical transsection are indicated.

Child, Preschool↗
PubMed1997

Electrodiagnostic findings related to anti-GM1 and anti-GQ1b antibodies in Guillain-Barré syndrome.

Antibodies against the gangliosides GM1 and GQ1b may induce conduction failure in mice. To investigate their possible site of action in the Guillain-Barré syndrome (GBS), we studied the relation between serum anti-GM1 and anti-GQ1b antibodies and electromyography in 124 GBS patients. Anti-GM1 antibodies were found in 22 (18%) and anti-GQ1b antibodies in 5 (4%) patients. Anti-GM1 antibodies were associated with low distal compound muscle action potential amplitudes and relatively high compound sensory nerve action potential (CSNAP) amplitudes. In none of the patients with anti-GQ1b antibodies could CSNAPs be detected. Patients with anti-GM1 and anti-GQ1b antibodies were heterogenous with respect to electrodiagnostic features exclusive fordemyelination oraxonal degeneration, although the anti-GM1 positive patients tended to have more axonal degeneration. In conclusion, electromyographic studies indicate selective and more severe damage of motor nerves in patients with anti-GM1 antibodies, while patients with anti-GQ1b antibodies have more severe damage of sensory nerves. These antibodies may interfere with the electrophysiologic properties of different nerve fibers and thereby contribute to the clinical heterogeneity in GBS.

Animals↗
PubMed1997

Guillain-Barré syndrome: multifactorial mechanisms versus defined subgroups.

The clinical spectrum of Guillain-Barré syndrome (GBS) is summarized in relation to antecedent infections and anti-ganglioside antibodies. Associations exist between a pure motor form of GBS, diarrhea, Campylobacter jejuni infection, and anti-GM1 antibodies; between cranial nerve involvement and Miller Fisher syndrome, C. jejuni infection, and anti-GQ1b antibodies; and between variants, such as severe sensory involvement and cytomegalovirus infection. These three clinical variants are suggested to form the extremes of a continuous spectrum; they are discussed in relation to the more pathologically defined patterns of acute motor axonal neuropathy and acute motor-sensory axonal neuropathy. In particular, patients with a clinically pure motor variant of GBS, diarrhea, anti-GM1 antibodies, or C. jejuni infection seem to respond better to early treatment with high-dose immunoglobulins than to plasma exchange.

Autoantibodies↗
PubMed1997

Decreased electrical excitability of peripheral nerves in demyelinating polyneuropathies.

Not recognising the presence of decreased excitability may give rise to a seemingly low compound muscle action potential, which may lead erroneously to the conclusion of conduction block. To quantify decreased electrical excitability, stimulation-response curves and the current needed to achieve 90% of the maximal compound muscle action potential amplitude, i90, were obtained in 17 healthy controls, eight patients with Guillain-Barre syndrome, 14 with chronic inflammatory demyelinating polyneuropathy, and 10 with hereditary motor sensory neuropathy type I. Decreased electrical excitability was found in patients with chronic inflammatory demyelinating polyneuropathy and hereditary motor sensory neuropathy type I, by contrast with patients with Guillain-Barré syndrome. Recognising decreased excitability prevents the false assertion of conduction block and has electrodiagnostic importance for the differential diagnosis of demyelinating polyneuropathies.

Action Potentials↗
PubMed1996

The diagnosis of subclinical hepatic encephalopathy in patients with cirrhosis using neuropsychological tests and automated electroencephalogram analysis.

Neuropsychological tests used for the assessment of subclinical hepatic encephalopathy (SHE) may overdiagnose SHE because scores are usually not corrected for age. The aim of this study was to estimate the prevalence of SHE using two easy administrable psychometric tests (Number Connection Test part A [NCT-A], Symbol Digit Test [SDT]) with age-related normal values. In addition, spectral electroencephalogram (EEG) was used, which is the in-house electrophysiological method for quantifying encephalopathy. One hundred and thirty-seven consecutive patients (mean age 49 years, range 17-77) with cirrhosis without any clinical signs of encephalopathy, were screened for SHE. In addition, the Child-Pugh score and the arterial blood ammonia were determined. Patients with concurrent use of alcohol, benzodiazepines or anti-epileptics were excluded. Fifty percent of the patients had an abnormal NCT according to the standard recommended procedure, in contrast only 7% of the patients had an abnormal NCT when scores corrected for age were used. Combining the results of the spectral EEG and the psychometric tests corrected for age yielded a higher prevalence of SHE (23%) than when each test method was used alone (17% vs. 10% abnormal, respectively). Severity of liver disease correlated with the presence of SHE, because the prevalence of abnormal tests increased from 14% in Child-Pugh grade A to 45% in Child-Pugh grade B or C. Age above 40 years and an elevated blood ammonia level were significant determinants related to an abnormal EEG. We conclude that the NCT uncorrected for age markedly overdiagnoses SHE and, therefore, should not be used as a test for the screening of SHE. Using a combination of spectral EEG and two psychometric tests with age-corrected normal values a low prevalence of SHE in patients with Child A liver cirrhosis is found. Older patients with an elevated arterial ammonia are more prone to develop SHE than younger patients with an equal arterial ammonia concentration.

Adolescent↗
PubMed1996

Cytomegalovirus infection and Guillain-Barré syndrome: the clinical, electrophysiologic, and prognostic features. Dutch Guillain-Barré Study Group.

Guillain-Barré syndrome (GBS) is usually preceded by infections, in particular cytomegalovirus (CMV) and Campylobacter jejuni infection. We studied the clinical and electrophysiologic features of 20 CMV-associated GBS patients and compared the findings with earlier established data of C. jejuni-related GBS patients (n = 43) and of GBS patients without these infections (n = 71). The patients all participated in the Dutch GBS trial in which we compared the effect of intravenous immune globulins and plasma exchange. We demonstrate that CMV-related GBS patients have a different clinical pattern in comparison with the other two GBS groups. They are significantly younger, initially have a severe course indicated by a high frequency of respiratory insufficiency, and often develop cranial nerve involvement and severe sensory loss. This is in contrast to C. jejuni infection, which is associated with motor GBS. Both infections are associated with delayed recovery compared with the GBS patients without these infections.

Cytomegalovirus Infections↗
PubMed1995

Guillain-Barré syndrome without sensory loss (acute motor neuropathy). A subgroup with specific clinical, electrodiagnostic and laboratory features. Dutch Guillain-Barré Study Group.

We analysed data obtained from 27 out of a group of 147 patients with Guillain-Barré syndrome, who did not have sensory loss during a follow-up period of 6 months (motor Guillain-Barré syndrome). These patients had a distinctive clinical pattern compared with the other 120 Guillain-Barré syndrome patients. The clinical course was marked by a more rapid onset of weakness (3.9 versus 6.1 days, P = 0.002), an earlier nadir (6.3 versus 9.1 days, P < 0.001), an initially predominant distal weakness (67% versus 27%, P < 0.001), sparing of the cranial nerves (26% versus 68%, P < 0.001) and the disease was more often preceded by a gastro-intestinal illness (41% versus 13%, P = 0.001) often caused by a Campylobacter jejuni infection (67% versus 28% in the other Guillain-Barré syndrome patients, P < 0.001). High titres of anti-GM1 antibodies were also significantly more common in motor Guillain-Barré syndrome patients (42% versus 5%, P < 0.001). Electromyographic data of the motor Guillain-Barré syndrome patients at nadir revealed little or no evidence for demyelination. Abundant denervation activity was present in half of the patients. The response to immune globulin treatment was good but with plasma exchange significantly fewer motor Guillain-Barré syndrome patients reached the stage of independent locomotion after a follow-up period of 6 months especially if the acute motor neuropathy occurred after a C.jejuni infection.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗