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N C Notermans

Publications and source records attributed to N C Notermans.

At least 19 recordsLinked to original sources
PubMed2006

Neurologic and hematologic response to fludarabine treatment in IgM MGUS polyneuropathy.

We studied the efficacy of fludarabine in 16 patients with immunoglobulin M monoclonal gammopathy of unknown significance polyneuropathy in a prospective uncontrolled trial. The modified Rankin scale improved in 5/16 patients, all of whom had a demyelinating polyneuropathy. The motor conduction velocity improved by more than 10% in two or more nerves for four of five of these patients. Hematologic response in bone marrow occurred in three of five of these patients, whereas two of five already had small polyclonal B cell populations. There were no serious side effects.

Aged↗
PubMed2006

The Dutch neuromuscular database CRAMP (Computer Registry of All Myopathies and Polyneuropathies): development and preliminary data.

Each of the various neuromuscular diseases is rare. Consequently, solid epidemiological data are not available and it is often difficult to find sufficient patients for studies. For this reason, the Dutch neuromuscular database, CRAMP (Computer Registry of All Myopathies and Polyneuropathies), was developed in 2004 by the Dutch Neuromuscular Research Support Centre, to store information on patient characteristics and diagnoses (based on Rowland and McLeod's classification) in a uniform and easily retrievable manner. Care was taken to preserve data confidentiality. It is envisaged that CRAMP will prove particularly useful for studies in which multicentre collaboration is needed to recruit a sufficiently large number of patients. More than 10,000 patients with neuromuscular diseases (4,837 female, 5,476 male) have been registered since 2004, half of whom (n=5059) have peripheral nerve disorders.

Computers↗
PubMed2006

Feasibility and cost efficiency of a diagnostic guideline for chronic polyneuropathy: a prospective implementation study.

BACKGROUND: Extensive investigations are often performed to reveal the cause of chronic polyneuropathy. It is not known whether a restrictive diagnostic guideline improves cost efficiency without loss of diagnostic reliability. METHODS: In a prospective multicentre study, a comparison was made between the workup in patients with chronic polyneuropathy before and after guideline implementation. RESULTS: Three hundred and ten patients were included: 173 before and 137 after guideline implementation. In all patients, the diagnosis would remain the same if the workup was limited to the investigations in the guideline. After guideline implementation, the time to reach a diagnosis decreased by two weeks. There was a reduction of 33% in the number and costs of routine laboratory investigations/patient, and a reduction of 27% in the total number of laboratory tests/patient, despite low guideline adherence. CONCLUSION: The implementation of a diagnostic guideline for chronic polyneuropathy can reduce diagnostic delay and the number and costs of investigations for each patient without loss of diagnostic reliability. Continuous evaluation strategies after guideline implementation may improve guideline adherence and cost efficiency.

Chronic Disease↗
PubMed2005

[Sensory disturbances caused by multivitamin preparations].

2 patients, men aged 60 and 65 years, presented with symptoms of chronic sensory polyneuropathy. Symptoms in the first patient were bilateral numbness in the hands and leg pain and, in the second patient, painful tingling in the legs. Pyridoxine (vitamin B6) toxicity due to daily use of multivitamin supplements was diagnosed. The patients were taking 24 and 40 mg per day, respectively. Neurotoxic syndromes due to pyridoxine overdose have been described before in patients taking high-dose vitamin B. These patients mostly developed progressive sersory neuronopathy with sensory ataxia. Chronic sensory polyneuropathy has not been associated with the use of vitamin supplements, which have previously been considered harmless. Both patients recovered after discontinuation of supplement intake.

Aged↗
PubMed2005

Malignant transformation in polyneuropathy associated with monoclonal gammopathy.

OBJECTIVE: To assess the frequency of hematologic malignancies at diagnosis and to determine the incidence and predictors of malignant transformation during follow-up in patients with polyneuropathy associated with monoclonal gammopathy. METHODS: Potential predictors of malignant transformation from medical history, hematologic, neurologic, and laboratory examination performed each 6 months were evaluated by univariable and multivariable Cox proportional hazard analysis. RESULTS: Of 193 patients with polyneuropathy associated with monoclonal gammopathy, 17 patients had a hematologic malignancy at diagnosis. The incidence rate of malignant transformation in 176 patients without a malignancy at diagnosis was 2.7/100 patient years. Weight loss, progression of the polyneuropathy, unexplained fever or night sweats, and M-protein level were independent predictors. CONCLUSIONS: Since hematologic malignancies occur frequently in polyneuropathy associated with monoclonal gammopathy, the authors suggest that all patients should be screened at diagnosis and subsequently during follow-up if malignant transformation is suspected.

Aged↗
PubMed2004

Drug therapy for chronic idiopathic axonal polyneuropathy.

BACKGROUND: Chronic idiopathic axonal polyneuropathy is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, it reduces quality of life. OBJECTIVES: To assess whether drug therapy for chronic idiopathic axonal polyneuropathy reduces disability, ameliorates neurological symptoms and associated impairments, and whether treatment is safe. SEARCH STRATEGY: We searched Cochrane Library (Cochrane Neuromuscular Disease Review Group Register, Cochrane Database of Systematic Reviews, Cochrane Database of Abstracts of Reviews of Effectiveness, and the Cochrane Central Register of Controlled Trials), MEDLINE, EMBASE, ISI, and ACP Journal Club's Best Evidence, from 1981 until December 2002. We also hand searched the reference lists of relevant articles, reviews and textbooks identified electronically, and contacted authors and other experts in the field to identify additional studies. SELECTION CRITERIA: We sought all randomised or quasi-randomised (alternate or other systematic treatment allocation), unconfounded trials that examined the effects of any drug therapy in patients with chronic idiopathic axonal polyneuropathy at least one year after the onset of treatment. Patients with chronic idiopathic axonal polyneuropathy had to fulfil the following criteria: age 40 years or older, distal sensory or sensorimotor polyneuropathy, absence of systemic or other neurological disease, chronic clinical course not reaching a nadir in less than two months, exclusion of any recognised cause of the polyneuropathy by medical history taking, clinical or laboratory investigations, electrophysiological studies in agreement with axonal polyneuropathy without evidence of demyelinating features. The primary outcome was the proportion of patients with a significant improvement in disability. Secondary outcomes were change in the mean disability score, change in the proportion of patients who make use of walking aids, change in the mean Medical Research Council sum score, degree of pain relief and/or reduction of other positive sensory symptoms, change in the proportion of patients with pain or other positive sensory symptoms, and frequency of adverse effects. DATA COLLECTION AND ANALYSIS: Two reviewers independently reviewed and extracted details of trial methodology and outcome data of all potentially relevant trials. MAIN RESULTS: Eighteen studies were identified and assessed for possible inclusion in the review, but all were excluded because of insufficient quality or lack of relevance. REVIEWERS' CONCLUSIONS: Even though chronic idiopathic axonal polyneuropathy has been clearly described and delineated, no adequate randomised or quasi-randomised controlled clinical treatment trials have been performed. In their absence there is no proven efficacious drug therapy.

Aged↗
PubMed2003

Increase of sural nerve T cells in progressive axonal polyneuropathy and monoclonal gammopathy.

The authors investigated whether T cells have a role in the pathogenesis of axonal polyneuropathy and monoclonal gammopathy by comparing the presence of T cells in sural nerves of 23 patients with axonal polyneuropathy and monoclonal gammopathy (12 IgM, 11 IgG), of 15 patients with chronic idiopathic axonal polyneuropathy, and of 10 autopsy cases. Seven patients with an increased T-cell density had a progressive disease course, and four of these patients were treated with prednisone with a good response, suggesting that vasculitis plays a role in the pathogenesis.

Axons↗
PubMed2002

Spinocerebellar ataxias in the Netherlands: prevalence and age at onset variance analysis.

BACKGROUND: International prevalence estimates of autosomal dominant cerebellar ataxias (ADCA) vary from 0.3 to 2.0 per 100,000. The prevalence of ADCA in the Netherlands is unknown. Fifteen genetic loci have been identified (SCA-1-8, SCA-10-14, SCA-16, and SCA-17) and nine of the corresponding genes have been cloned. In SCA-1, SCA2, SCA3, SCA6, SCA7, SCA-12 and SCA-17 the mutation has been shown to be an expanded CAG repeat. Previously, the length of the CAG repeat was found to account for 50 to 80% of variance in age at onset. Because of heterogeneity in encoded proteins, different pathophysiologic mechanisms leading to neurodegeneration could be involved. The relationship between CAG repeat length and age at onset would then differ accordingly. METHOD: Based on the results of SCA mutation analysis in the three DNA diagnostic laboratories that serve the entire Dutch population, the authors surveyed the number of families and affected individuals per SCA gene, as well as individual repeat length and age at onset. Regression analysis was applied to study the relationship between CAG repeat length and age at onset per SCA gene. The slopes of the different regression curves were compared. RESULTS: On November 1, 2000, mutations were found in 145 ADCA families and 391 affected individuals were identified. The authors extrapolated a minimal prevalence of 3.0 per 100,000 (range 2.8 to 3.8/100,000). SCA3 was the most frequent mutation. CAG repeat length contributed to 52 to 76% of age at onset variance. Regression curve slopes for SCA-1, SCA2, SCA3, and SCA7 did not differ significantly. CONCLUSIONS: The estimated minimal prevalence of ADCA in the Netherlands is 3.0 per 100,000 inhabitants. Except for SCA6, the relationship between age at onset and CAG repeat expansion does not differ significantly between SCA-1, SCA2, SCA3, and SCA7 patient groups in our population, indicating that these SCA subtypes share similar mechanisms of polyglutamine-induced neurotoxicity, despite heterogeneity in gene products.

Adult↗
PubMed2002

Quantitative assessment of the innervation of epineurial arteries in the peripheral nerve by immunofluorescence: differences between controls and patients with peripheral arterial disease.

The peripheral nerve is supplied by the vasa nervorum. The epi- and perineurial vessels are innervated by an autonomic plexus, which plays a role in regulation of the endoneurial blood flow. This innervation is decreased in diabetes and alcohol polyneuropathy and seems to precede the development of diabetic polyneuropathy. A decreased innervation may therefore play a role in the development of polyneuropathy. In peripheral arterial disease (PAD) clinical and morphological features are present, related to severity of ischemia. To investigate the innervation of the vasa nervorum in severe ischemia, we performed immunofluorescence staining with the general neural marker protein gene product (PGP) 9.5 in whole mount preparations of epineurial vessels of nine sural nerves taken from patients with legs amputated because of severe PAD (59+/-15 years, mean +/- SD) and ten age-matched controls (61+/-24 years). In patients with PAD the nerve density of the perivascular plexus was decreased in comparison with controls (mean intercept density/mm +/- SD) 26.0+/-6.9 in PAD and 39.9+/-10.7 in controls, area% 6.0+/-1.6 in PAD and 9.9+/-2.6 in controls, both P<0.01, t-test). A decreased perivascular plexus may result in a diminished regulation of the endoneurial blood flow in patients with severe PAD.

Adult↗
PubMed2002

Response to exercise of patients with idiopathic hyper-CK-emia.

Patients with an idiopathic increase in serum creatine kinase (CK) levels (hyper-CK-emia) have a benign prognosis, but symptoms may be disabling in daily life. Previous studies have suggested that physical exercise increases the severity of complaints in these patients. We studied whether maximal and submaximal bouts of exercise on a cycle ergometer are harmful for patients with idiopathic hyper-CK-emia. Such dynamic exercise did not lead to larger increases in serum CK activity or more complaints in 11 patients with idiopathic hyper-CK-emia, compared with 11 age-matched healthy controls. Our data suggest that exercise does not result in more extensive muscle damage in patients with idiopathic hyper-CK-emia than in healthy subjects.

Adolescent↗
PubMed2002

Is cardiovascular disease a risk factor in the development of axonal polyneuropathy?

OBJECTIVES: To determine if cardiovascular disease may be a risk factor in the development of chronic idiopathic axonal polyneuropathy (CIAP). METHODS: In this incidence case-control study, the prevalence of cardiovascular disease and risk factors in 97 patients with CIAP (mean age 67.5 (SD 7.9) years) and the prevalence of neuropathic features in 97 patients with peripheral arterial disease (PAD) (mean age 67.1 (SD 7.3) years) were investigated. The results were compared with those for 96 age and sex matched controls without diagnosed PAD or polyneuropathy (mean age 67.5 (SD 9.1) years). In a randomly chosen subgroup of 23 patients with CIAP, 42 patients with PAD, and 48 controls, an electrodiagnostic investigation was performed. RESULTS: Patients with CIAP more often had manifest cardiovascular disease and cardiovascular risk factors than controls (stroke 18% v 6% of patients, odds ratio (OR) 3.2 (95% confidence interval (CI) 1.8 to 5.9); heart disease 29% v 15%, OR 2.4 (95% CI 1.2 to 4.9); family history of cardiovascular disease 42% v 21%, OR 2.8 (95% CI (1.5 to 5.2); hypertension 56% v 39%, OR 2.0 (95% CI 1.1 to 3.6); hypercholesterolaemia 46% v 21%, OR 3.3 (95% CI 1.5 to 7.3); current smoking 38% v 23%, OR 2.1 (95% CI 1.1 to 3.9)). The prevalence of cardiovascular disease and cardiovascular risk factors was lower than in patients with PAD. Patients with PAD more often had polyneuropathy than controls (15% v 5%, OR 3.3 (95% CI 1.1 to 10.0)). There was a trend towards lower nerve conduction velocities and lower amplitudes on electrodiagnostic investigation compared with controls. CONCLUSION: This study shows that cardiovascular disease and CIAP often coexist, and therefore cardiovascular disease may be a cofactor in the development of CIAP.

Aged↗
PubMed2001

Antiganglioside antibodies in polyneuropathy associated with monoclonal gammopathy.

Antibody reactivity to GA1, GM1, GM2, GD1a, GD1b, and GQ1b gangliosides was measured in 87 patients with polyneuropathy associated with monoclonal gammopathy (60 IgM, 25 IgG, 2 IgA) and 42 control patients with monoclonal gammopathy without polyneuropathy (21 IgM, 21 IgG). Of these 87 patients, 30% had anti-myelin-associated glycoprotein antibodies and 15% had antiganglioside antibodies. Antiganglioside antibodies were significantly associated with demyelinating neuropathy and with IgM monoclonal gammopathy. Anti-GD1b and anti-GQ1b antibodies were significantly associated with predominantly sensory ataxic neuropathy.

Adult↗
PubMed2001

Sural nerve T cells in demyelinating polyneuropathy associated with monoclonal gammopathy.

The clinical presentation of polyneuropathy associated with monoclonal gammopathy is heterogeneous. As T cells in sural nerve biopsy specimens may represent a marker of inflammation, we analyzed whether the presence of sural nerve T cells in patients with demyelinating polyneuropathy associated with monoclonal gammopathy may help to define a specific clinical entity. Using immunohistochemical analysis we investigated the number and distribution of sural nerve T cells in 18 patients with polyneuropathy associated with IgM monoclonal gammopathy (including 14 with antibodies to the myelin-associated glycoprotein) and 7 with IgG monoclonal gammopathy, and compared them with sural nerves of 23 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 15 patients with chronic idiopathic axonal polyneuropathy (CIAP), and 10 normal controls. Six patients with polyneuropathy associated with monoclonal gammopathy had increased T cell densities compared with CIAP patients and normal controls. No differences were found in distribution or phenotype of the T cells. T cell densities in patients with IgM monoclonal gammopathy were significantly lower than in patients with IgG monoclonal gammopathy or with CIDP. Increased sural nerve T cells were significantly associated with a subset of patients who had a more progressive disease course and more pronounced weakness. Increased sural nerve T cells were found significantly more often in patients with a monoclonal gammopathy of the IgG isotype, which was frequently associated with hematological malignancy.

Adult↗
PubMed2001

Neuropathy and IgM M-proteins: prognostic value of antibodies to MAG, SGPG, and sulfatide.

BACKGROUND: In polyneuropathy associated with immunoglobulin M (IgM) monoclonal gammopathy, antibodies to myelin-associated glycoprotein (MAG), sulfoglucuronyl paragloboside (SGPG), and sulfatide have been associated with specific clinical and electrophysiologic features. However, it is not known whether the results of antibody tests provide additional information for the individual patient (and the neurologist) in terms of future neurologic deficit or outcome. OBJECTIVE: To study the independent contribution of potential prognostic factors to the prediction of outcome of neuropathy associated with IgM monoclonal gammopathy. METHODS: In accordance with the chronology in which prognostic factors are available in clinical practice, the association between prognostic factors and outcome was evaluated by univariate and multivariate logistic regression analysis in 65 patients with polyneuropathy and IgM monoclonal gammopathy. RESULTS: In univariate analysis, the initial symptoms, the IgM light chain type, electrophysiologic and pathologic studies, the presence of sural nerve IgM deposition, and anti-MAG or anti-SGPG antibodies were significantly associated with outcome. However, multivariate analysis showed that only initial symptoms and electrophysiologic studies are independent prognostic factors: initial sensory symptoms of the feet are prognostic for a slowly progressive disease course and less disability at 4 years, and evidence for demyelination on electrophysiologic examination is prognostic for development of weakness and symptoms of the upper extremities at 4 years. Addition of anti-MAG or anti-SGPG antibody tests did not yield any additional prediction of outcome. CONCLUSION: These results indicate that in clinical practice, antibody tests in polyneuropathy associated with IgM monoclonal gammopathy do not have a prognostic value in terms of future neurologic deficit or outcome.

Globosides↗
PubMed2001

Risk factors for hematological malignancy in polyneuropathy associated with monoclonal gammopathy.

Polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS) is a well-known disease entity. Of the patients with monoclonal gammopathy without neuropathy, 25% develop a hematological malignancy during long-term follow-up. Whether the frequency of hematological malignancy is similar in patients with polyneuropathy associated with monoclonal gammopathy and whether hematological screening is necessary in these patients is unknown. To determine the frequency of and risk factors for a hematological malignancy, we investigated 104 patients with polyneuropathy and monoclonal gammopathy. Potential diagnostic variables were obtained from medical history, physical and neurological examination, and laboratory analysis. The associations between potential diagnostic variables and outcome, hematological malignancy, were evaluated by univariable and multivariable logistic-regression analysis. Among our patients, 23 had a hematological malignancy (8 multiple myeloma, 10 low-grade lymphoma, 3 plasmacytoma, 1 Castleman's disease and 1 POEMS syndrome [polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes]). Weight loss, progression of the neuropathy, and an M-protein level > 1 g/L were independent risk factors for malignancy. Extensive screening is indicated in patients with these features.

Adult↗
PubMed2001

MRI of the brachial plexus in polyneuropathy associated with monoclonal gammopathy.

On magnetic resonance (MR) imaging of the brachial plexus increased signal intensity and swelling of the brachial plexus has been found in chronic inflammatory demyelinating polyneuropathy (CIDP). Whether these proximal abnormalities are also present in the distal polyneuropathy associated with monoclonal gammopathy is unknown. Therefore, we performed MR imaging of the brachial plexus in 21 patients with polyneuropathy associated with IgM monoclonal gammopathy (11 IgM with anti-MAG antibodies, 10 IgM without anti-MAG antibodies). For comparison we studied 9 patients with polyneuropathy associated with IgG monoclonal gammopathy and 8 patients with CIDP. Among the 30 patients with monoclonal gammopathy, 24 patients had demyelinating polyneuropathy. Among these 24 patients, there was increased signal intensity of the brachial plexus on the T2-weighted images regardless of whether clinical deficits were generalized or purely distal in location. No association was found with the isotype of the monoclonal gammopathy. Of the 8 patients with CIDP, 5 had brachial plexus abnormalities. None of the 6 patients with axonal polyneuropathy associated with monoclonal gammopathy had such abnormalities. Thus, MR imaging of the brachial plexus shows that the distal demyelinating polyneuropathy associated with monoclonal gammopathy is more generalized than presumed.

Adult↗
PubMed2001

Medullary cone movement in subjects with a normal spinal cord and in patients with a tethered spinal cord.

PURPOSE: To compare movement of the normal medullary cone when the patient has changed from a supine to prone position with that in patients with known or suspected tethered spinal cord syndrome. MATERIALS AND METHODS: Fifty-six individuals divided into three groups were examined with lumbar spine magnetic resonance (MR) imaging performed with the patient in the prone and supine positions. Group 1 consisted of 15 healthy volunteers and six patients with a herniated disk; group 2, 25 patients clinically suspected of having a tethered cord; and group 3, 10 patients who previously had undergone tethered cord surgery. RESULTS: All group 1 subjects showed distinct and statistically significant medullary cone movement (range, 21%--41%); no patient in group 3 showed movement (Wilcoxon rank sum test, P <.001). In group 2, the 20 patients in whom a definite diagnosis of tethered cord syndrome was made on the basis of initial supine MR image findings showed no movement, whereas two of five patients with normal supine MR images had abnormal and decreased cone movement at prone imaging. CONCLUSION: Prone MR imaging has no additional value when the supine MR image has clearly shown the cause of tethering or in patients who have undergone tethered cord surgery, but it can provide additional information in patients clinically suspected of having a tethered cord and in whom supine MR imaging depicted no abnormalities.

Adolescent↗