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2. Longitudinal analysis of serum neurofilament light chain levels as marker for neuronal damage in hereditary transthyretin amyloidosis

Author

Neurologen, Brain, Berends, Milou, Brunger, Anne F., Bijzet, Johan, Kroesen, Bart Jan, Drost, Gea, Lange, Fiete, Teunissen, Charlotte E., in ‘t Veld, Sjors, Vrancken, Alexander F.J.E., Gans, Reinold O.B., Hazenberg, Bouke P.C., van der Zwaag, Paul A., Nienhuis, Hans L.A., Neurologen, Brain, Berends, Milou, Brunger, Anne F., Bijzet, Johan, Kroesen, Bart Jan, Drost, Gea, Lange, Fiete, Teunissen, Charlotte E., in ‘t Veld, Sjors, Vrancken, Alexander F.J.E., Gans, Reinold O.B., Hazenberg, Bouke P.C., van der Zwaag, Paul A., and Nienhuis, Hans L.A.

Published
2024

5. IgM anti-MAG± peripheral neuropathy (IMAGiNe) study protocol: An international, observational, prospective registry of patients with IgM M-protein peripheral neuropathies

Author

Neurologen, Brain, Neurologie, Cancer, Infection & Immunity, Hamadeh, Tatiana, van Doormaal, Perry T.C., Pruppers, Mariëlle H.J., van de Mortel, Johannes P.M., Hoeijmakers, Janneke G.J., Cornblath, David R., Vrancken, Alexander F.J.E., Faber, Catharina G., Notermans, Nicolette C., Merkies, Ingemar S.J., the IMAGiNe Consortium, Neurologen, Brain, Neurologie, Cancer, Infection & Immunity, Hamadeh, Tatiana, van Doormaal, Perry T.C., Pruppers, Mariëlle H.J., van de Mortel, Johannes P.M., Hoeijmakers, Janneke G.J., Cornblath, David R., Vrancken, Alexander F.J.E., Faber, Catharina G., Notermans, Nicolette C., Merkies, Ingemar S.J., and the IMAGiNe Consortium

Published
2023

6. Thoracic outlet syndrome (TROTS) registry:A study protocol for the primary upper extremity deep venous thrombosis section

Author

Schropp, Ludo, de Kleijn, Robert J.C.M.F., Westerink, Jan, Nijkeuter, Mathilde, Vonken, Evert Jan, van der Schaaf, Irene C., Goedee, H. Stephan, Vrancken, Alexander F.J.E., van Hattum, Eline S., Petri, Bart Jeroen, de Borst, Gert J., ten Cate, Arina, van Weel, Vincent, Mol, Gerben C., Schropp, Ludo, de Kleijn, Robert J.C.M.F., Westerink, Jan, Nijkeuter, Mathilde, Vonken, Evert Jan, van der Schaaf, Irene C., Goedee, H. Stephan, Vrancken, Alexander F.J.E., van Hattum, Eline S., Petri, Bart Jeroen, de Borst, Gert J., ten Cate, Arina, van Weel, Vincent, and Mol, Gerben C.

Abstract

Introduction There is a lack of comprehensive and uniform data on primary upper extremity deep venous thrombosis (pUEDVT). pUEDVT includes venous thoracic outlet syndrome related upper extremity deep venous thrombosis (UEDVT) and idiopathic UEDVT. Research on these conditions has been hampered by their rarity, lack of uniform diagnostic criteria, and heterogeneity in therapeutic strategies. To improve current research data collection using input of all various pUEDVT treating medical specialists, we initiated the ThoRacic OuTlet Syndrome (TROTS) registry. The aim of the TROTS registry is to a) collect extensive data on all pUEDVT patients through a predefined protocol, b) give insight in the long term outcome using patient reported outcome measures, c) create guidance in the diagnostic and clinical management of these conditions, and thereby d) help provide content for future research. Methods and analysis The TROTS registry was designed as an international prospective longitudinal observational registry for data collection on pUEDVT patients. All pUEDVT patients, regardless of treatment received, can be included in the registry after informed consent is obtained. All relevant data regarding the initial presentation, diagnostics, treatment, and follow-up will be collected prospectively in an electronic case report form. In addition, a survey containing general questions, a Health-related Quality of Life questionnaire (EQ-5D-5L), and Functional Disability questionnaire (Quick-DASH) will be sent periodically (at the time of inclusion, one and two years after inclusion, and every five years after inclusion) to the participant. The registry protocol was approved by the Medical Ethical Review Board and registered in the Netherlands Trial Register under Trial-ID NL9680. The data generated by the registry will be used for future research on pUEDVT and published in peer reviewed journals. Conclusion TROTS registry data will be used to further establish the optimal manage

Published
2023

7. Thoracic outlet syndrome (TROTS) registry: A study protocol for the primary upper extremity deep venous thrombosis section

Author

Schropp, Ludo, de Kleijn, Robert J.C.M.F., Westerink, Jan, Nijkeuter, Mathilde, Vonken, Evert Jan, van der Schaaf, Irene C., Goedee, H. Stephan, Vrancken, Alexander F.J.E., van Hattum, Eline S., Petri, Bart Jeroen, de Borst, Gert J., ten Cate, Arina, van Weel, Vincent, Mol, Gerben C., Surgery, ACS - Atherosclerosis & ischemic syndromes, ACS - Microcirculation, APH - Digital Health, Vascular Medicine, ACS - Pulmonary hypertension & thrombosis, Amsterdam Cardiovascular Sciences, Hematology, Radiology & Nuclear Medicine, and Molecular Genetics

Subjects
Multidisciplinary
Abstract

Introduction There is a lack of comprehensive and uniform data on primary upper extremity deep venous thrombosis (pUEDVT). pUEDVT includes venous thoracic outlet syndrome related upper extremity deep venous thrombosis (UEDVT) and idiopathic UEDVT. Research on these conditions has been hampered by their rarity, lack of uniform diagnostic criteria, and heterogeneity in therapeutic strategies. To improve current research data collection using input of all various pUEDVT treating medical specialists, we initiated the ThoRacic OuTlet Syndrome (TROTS) registry. The aim of the TROTS registry is to a) collect extensive data on all pUEDVT patients through a predefined protocol, b) give insight in the long term outcome using patient reported outcome measures, c) create guidance in the diagnostic and clinical management of these conditions, and thereby d) help provide content for future research. Methods and analysis The TROTS registry was designed as an international prospective longitudinal observational registry for data collection on pUEDVT patients. All pUEDVT patients, regardless of treatment received, can be included in the registry after informed consent is obtained. All relevant data regarding the initial presentation, diagnostics, treatment, and follow-up will be collected prospectively in an electronic case report form. In addition, a survey containing general questions, a Health-related Quality of Life questionnaire (EQ-5D-5L), and Functional Disability questionnaire (Quick-DASH) will be sent periodically (at the time of inclusion, one and two years after inclusion, and every five years after inclusion) to the participant. The registry protocol was approved by the Medical Ethical Review Board and registered in the Netherlands Trial Register under Trial-ID NL9680. The data generated by the registry will be used for future research on pUEDVT and published in peer reviewed journals. Conclusion TROTS registry data will be used to further establish the optimal management of pUEDVT and lay the foundation for future research and guidelines.

Published
2023
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8. Withdrawal of intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy

Author

Adrichem, Max E., Lucke, Ilse M., Vrancken, Alexander F.J.E., Goedee, H. Stephan, Wieske, Luuk, Dijkgraaf, Marcel G.W., Voermans, Nicol C., Notermans, Nicolette C., Faber, Catharina G., Visser, Leo H., Kuitwaard, Krista, van Doorn, Pieter A., Merkies, Ingemar S.J., de Haan, Rob J., van Schaik, Ivo N., Eftimov, Filip, Adrichem, Max E., Lucke, Ilse M., Vrancken, Alexander F.J.E., Goedee, H. Stephan, Wieske, Luuk, Dijkgraaf, Marcel G.W., Voermans, Nicol C., Notermans, Nicolette C., Faber, Catharina G., Visser, Leo H., Kuitwaard, Krista, van Doorn, Pieter A., Merkies, Ingemar S.J., de Haan, Rob J., van Schaik, Ivo N., and Eftimov, Filip

Abstract

Intravenous immunoglobulins are an efficacious treatment for chronic inflammatory demyelinating polyradiculoneuropathy. Biomarkers for disease activity are lacking, making the need for ongoing treatment difficult to assess, leading to potential overtreatment and high health-care costs. Our objective was to determine whether intravenous immunoglobulin withdrawal is non-inferior to continuing intravenous immunoglobulin treatment and to determine how often patients are overtreated. We performed a randomized, double-blind, intravenous immunoglobulin-controlled non-inferiority trial in seven centres in the Netherlands (Trial registration: ISRCTN 13637698; www.isrctn.com/ISRCTN13637698). Adults with clinically stable chronic inflammatory demyelinating polyradiculoneuropathy using intravenous immunoglobulin maintenance treatment for at least 6 months were included. Patients received either intravenous immunoglobulin withdrawal (placebo) as investigational treatment or continuation of intravenous immunoglobulin treatment (control). The primary outcome was the mean change in logit scores from baseline to 24-week follow-up on the patient-reported Inflammatory Rasch-Overall Disability Scale. The non-inferiority margin was predefined as between-group difference in mean change scores of -0.65. Patients who deteriorated could reach a relapse end point according to predefined criteria. Patients with a relapse end point after intravenous immunoglobulin withdrawal entered a restabilization phase. All patients from the withdrawal group who remained stable were included in an open-label extension phase of 52 weeks. We included 60 patients, of whom 29 were randomized to intravenous immunoglobulin withdrawal and 31 to continuation of treatment. The mean age was 58 years (SD 14.7) and 67% was male. The between-group difference in mean change Inflammatory Rasch-Overall Disability Scale scores was -0.47 (95% CI -1.24 to 0.31), indicating that non-inferiority of intravenous immunoglobuli

Published
2022

9. Vegetarians, Pescatarians and Flexitarians with Adequate Vitamin B12 Levels Have No Increased Risk of Polyneuropathy

Author

Van Doormaal, Perry T.C., Warendorf, Janna K., Visser, Nora A., De Vries, Jeanne H.M., Notermans, Nicolette C., Vrancken, Alexander F.J.E., Van Doormaal, Perry T.C., Warendorf, Janna K., Visser, Nora A., De Vries, Jeanne H.M., Notermans, Nicolette C., and Vrancken, Alexander F.J.E.

Abstract

Background: In recent years, an increasing number of people adapt to a vegetarian, pescatarian or flexitarian dietary pattern that reduces the consumption of meat and fish. Although these dietary patterns have a risk for developing vitamin B12 deficiency associated polyneuropathy, it is unknown whether this risk is still increased when vitamin B12 levels are adequate. Objective: To examine whether a vegetarian, pescatarian or flexitarian dietary pattern is associated with an increased risk for idiopathic axonal polyneuropathy. Methods: We conducted a case-control study that included 256 idiopathic axonal polyneuropathy patients with adequate vitamin B12 blood levels and 630 controls. We used questionnaire data to determine the frequency of meat and fish consumption and defined dietary patterns. Results: The vegetarian (no meat or fish consumption) and the pescatarian (fish consumption, no meat consumption) dietary patterns showed no increased risk of axonal polyneuropathy. Frequency-effect analysis and quantity-effect analysis also did not show that a reduction of meat or fish consumption (flexitarian dietary pattern), either small or large, changed the risk of axonal polyneuropathy. Conclusions: We did not find an increased risk for axonal polyneuropathy among people with a vegetarian, pescatarian or flexitarian diet and an adequate vitamin B12 level.

Published
2022

10. Enriched enrollment randomized double-blind placebo-controlled cross-over trial with phenytoin cream in painful chronic idiopathic axonal polyneuropathy (EPHENE): a study protocol

Author

Onderzoek NEU, Data Science & Biostatistiek, Projectafdeling ALS, Brain, Opleiding Neurologie, Neurologie, Neurologen, Kopsky, David J., van Eijk, Ruben P.A., Warendorf, Janna K., Keppel Hesselink, Jan M., Notermans, Nicolette C., Vrancken, Alexander F.J.E., Onderzoek NEU, Data Science & Biostatistiek, Projectafdeling ALS, Brain, Opleiding Neurologie, Neurologie, Neurologen, Kopsky, David J., van Eijk, Ruben P.A., Warendorf, Janna K., Keppel Hesselink, Jan M., Notermans, Nicolette C., and Vrancken, Alexander F.J.E.

Published
2022

11. In Response to: Pediatric Myelodysplastic Syndrome with Germline RRAS Mutation: Expanding the Phenotype of RASopathies

Author

SCT patientenzorg, Neurologen, Genetica, Brain, Genetica Klinische Genetica, Cancer, Child Health, Van Engelen, Nienke, Diets, Illja, Bresters, Dorine, Van Den Bergen, Janneke C., Vrancken, Alexander F.J.E., Kuiper, Roland P., Jongmans, Marjolijn C.J., SCT patientenzorg, Neurologen, Genetica, Brain, Genetica Klinische Genetica, Cancer, Child Health, Van Engelen, Nienke, Diets, Illja, Bresters, Dorine, Van Den Bergen, Janneke C., Vrancken, Alexander F.J.E., Kuiper, Roland P., and Jongmans, Marjolijn C.J.

Published
2022

15. Clinical relevance of testing for metabolic vitamin B12 deficiency in patients with polyneuropathy.

Author

Warendorf, Janna K., van Doormaal, Perry T.C., Vrancken, Alexander F.J.E., Verhoeven-Duif, Nanda M., van Eijk, Ruben P.A., van den Berg, Leonard H., and Notermans, Nicolette C.

Subjects
VITAMIN B12 deficiency, DIABETIC neuropathies, POLYNEUROPATHIES, DIETARY supplements, METHYLMALONIC acid, POLYHYDRAMNIOS, VITAMIN B12
Abstract

Determine vitamin B12 threshold levels below which additional testing of methylmalonic acid (MMA) and/or homocysteine (Hcy) is useful to diagnose metabolic vitamin B12 deficiency in patients with polyneuropathy, and how vitamin B12, MMA and Hcy levels relate to the effect of supplementation therapy. In a retrospective cohort study of 331 patients with polyneuropathy, vitamin B12, MMA and Hcy were measured. Linear regression models with vitamin B12 as dependent and Hcy or MMA as covariate were compared, to assess which was best related to vitamin B12. Threshold vitamin B12 levels for metabolic deficiency (defined as elevatede metabolites) were determined using logistic regression with elevated metabolites as dependent and vitamin B12 as covariate. A structured interview was conducted in 42 patients to evaluate response to vitamin B12 supplementation. MMA was best related to vitamin B12. Using elevated MMA for metabolic deficiency, we found 90% sensitivity at a vitamin B12 threshold level <264 pmol/L (358 pg/mL) and 95% sensitivity at <304 pmol/L (412 pg/mL). Improvement after supplementation was reported by 19% patients and stabilization by 24%. 88% of patients with improvement and 90% with stabilization either had absolute deficiency (Vitamin B12 < 148 pmol/L) or metabolic deficiency (elevated MMA and vitamin B12 ≥ 148 pmol/L). There were no additional patients with improvement or stabilization with isolated elevated Hcy. Testing of MMA has additional value in identifying patients with clinically relevant metabolic deficiency when vitamin B12 is below 304 pmol/L (412 pg/mL). Supplementation can be effective in patients with absolute and metabolic deficiency. [ABSTRACT FROM AUTHOR]

Published
2022
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18. Neuropathy associated with immunoglobulin M monoclonal gammopathy : A combined sonographic and nerve conduction study

Author

Goedee, H. Stephan, Notermans, Nicolette C., Visser, Leo H., van Asseldonk, Jan Thies H., Franssen, Hessel, Vrancken, Alexander F.J.E., Nikolakopoulos, Stavros, van den Berg, Leonard H., van der Pol, W. Ludo, Goedee, H. Stephan, Notermans, Nicolette C., Visser, Leo H., van Asseldonk, Jan Thies H., Franssen, Hessel, Vrancken, Alexander F.J.E., Nikolakopoulos, Stavros, van den Berg, Leonard H., and van der Pol, W. Ludo

Published
2019

19. Neuropathy associated with immunoglobulin M monoclonal gammopathy: A combined sonographic and nerve conduction study

Author

ZL Neuromusculaire Ziekten Medisch, Brain, Biostatistiek Onderzoek, JC onderzoeksprogramma Methodologie, Projectafdeling ALS, Regenerative Medicine and Stem Cells, Goedee, H. Stephan, Notermans, Nicolette C., Visser, Leo H., van Asseldonk, Jan Thies H., Franssen, Hessel, Vrancken, Alexander F.J.E., Nikolakopoulos, Stavros, van den Berg, Leonard H., van der Pol, W. Ludo, ZL Neuromusculaire Ziekten Medisch, Brain, Biostatistiek Onderzoek, JC onderzoeksprogramma Methodologie, Projectafdeling ALS, Regenerative Medicine and Stem Cells, Goedee, H. Stephan, Notermans, Nicolette C., Visser, Leo H., van Asseldonk, Jan Thies H., Franssen, Hessel, Vrancken, Alexander F.J.E., Nikolakopoulos, Stavros, van den Berg, Leonard H., and van der Pol, W. Ludo

Published
2019

20. Protocol of a dose response trial of IV immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy (DRIP study)

Author

Kuitwaard, Krista, Fokkink, Willem Jan R., Brusse, Esther, Vrancken, Alexander F.J.E., Eftimov, Filip, Notermans, Nicolette C., van der Kooi, Anneke J., Merkies, Ingemar S.J., Jacobs, Bart C., van Doorn, Pieter A., Amsterdam Neuroscience - Neuroinfection & -inflammation, Neurology, AII - Inflammatory diseases, Amsterdam institute for Infection and Immunity, RS: MHeNs - R1 - Cognitive Neuropsychiatry and Clinical Neuroscience, and Immunology

Subjects
Chronic inflammatory demyelinating polyradiculoneuropathy, IVIg, PERIPHERAL-NERVE-SOCIETY, Maintenance treatment, chronic inflammatory demyelinating polyradiculoneuropathy, maintenance treatment, NEUROLOGICAL-SOCIETIES, Neuroscience(all), MULTIFOCAL MOTOR NEUROPATHY, Clinical Neurology, RANDOMIZED CONTROLLED-TRIAL, GUILLAIN-BARRE-SYNDROME, intravenous immunoglobulin, hemic and lymphatic diseases, Protocol, INTEROBSERVER AGREEMENT, protocol, JOINT TASK-FORCE, IMMUNE-MEDIATED POLYNEUROPATHIES, RCT, EUROPEAN-FEDERATION
Abstract

High peak levels of serum IgG may not be needed for maintenance treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with intravenous immunoglobulin (IVIg). More frequent dosing of IVIg leads to more stable IgG levels and higher trough levels which may be related with improved clinical efficacy. More frequent lower dosing leads to lower peak levels and may induce less systemic side-effects. The DRIP study is a double-blind randomized controlled cross-over intervention study. CIDP patients ≥18years old, proven IVIg dependent and receiving an individually established but stable maintenance dose and interval of IVIg (Kiovig) can be included. One group (A) will be treated with their normal dosage and interval of IVIg and receive a placebo (albumin 0.5%) infusion in between their regular IVIg infusions, for a total of four infusions. The other group (B) will be treated with half their normal IVIg dosage (with the same volume of placebo to maintain the total volume) at half their interval (double their frequency) for four infusions. After a wash-out phase (2 infusions), patients will cross-over to the other treatment group. During the study the total dose of IVIg administered will remain unchanged as before start of the trial. The main objective is to investigate whether high frequent low dosage IVIg treatment is more effective than low frequent high dosage IVIg treatment as maintenance treatment for CIDP. Hand grip strength, as measured by the Martin Vigorimeter, will be used as the primary outcome measure. Secondary objective is to investigate whether high frequent low dosage of IVIg results in less adverse events compared to low frequent high dosage treatment. The DRIP study is currently ongoing and the protocol is presented.

Published
2018

22. Protocol of a dose response trial of IV immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy (DRIP study)

Author

ZL Neuromusculaire Ziekten Medisch, Brain, Apotheek Opleiding, Kuitwaard, Krista, Fokkink, Willem Jan R., Brusse, Esther, Vrancken, Alexander F.J.E., Eftimov, Filip, Notermans, Nicolette C., van der Kooi, Anneke J., Merkies, Ingemar S.J., Jacobs, Bart C., van Doorn, Pieter A., ZL Neuromusculaire Ziekten Medisch, Brain, Apotheek Opleiding, Kuitwaard, Krista, Fokkink, Willem Jan R., Brusse, Esther, Vrancken, Alexander F.J.E., Eftimov, Filip, Notermans, Nicolette C., van der Kooi, Anneke J., Merkies, Ingemar S.J., Jacobs, Bart C., and van Doorn, Pieter A.

Published
2018

23. Maintenance IV immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy

Author

Kuitwaard, Krista, Fokkink, Willem Jan R., Brusse, Esther, Vrancken, Alexander F.J.E., Eftimov, Filip, Notermans, Nicolette C., van der Kooi, Anneke J., Merkies, Ingemar S.J., Jacobs, Bart C., van Doorn, Pieter A., Neurology, and Immunology

Subjects
IVIg, chronic inflammatory demyelinating polyradiculoneuropathy, maintenance treatment, hemic and lymphatic diseases, intravenous immunoglobulin, Neuroscience(all), Clinical Neurology, Journal Article, CIDP, Review
Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients treated with intravenous immunoglobulin (IVIg) usually start with a standard dosage of 2 g/kg bodyweight. Only a minority of patients has a sustained improvement, and most require ongoing maintenance treatment. Preferred IVIg regimens, however, vary considerably between doctors and at present it is unknown which is optimal. As there are also large differences in IVIg dosage and interval requirements between patients, optimal IVIg maintenance treatment of CIDP is even more complex. The lack of evidence-based guidelines on how IVIg maintenance treatment should be administered may potentially lead to under- or overtreatment of this expensive therapy. We provide an overview of published practical IVIg maintenance treatment regimens, IVIg maintenance schedules used in randomized controlled trials and one based upon our own long-term experience on how this treatment could be given in CIDP.

Published
2017

24. The role of nutrition as risk factor for polyneuropathy : a case-control study

Author

Visser, Nora A., Notermans, Nicolette C., de Vries, Jeanne H.M., van den Berg, Leonard H., and Vrancken, Alexander F.J.E.

Subjects
Global Nutrition, Wereldvoeding, nutrition, alcohol, Neuroscience(all), Clinical Neurology, Journal Article, case–control study, polyneuropathy, VLAG
Abstract

The aim of this case–control study is to investigate the role of nutrition as risk factor for polyneuropathy. Three hundred eighteen patients with chronic idiopathic axonal polyneuropathy and 636 matched controls completed a validated food frequency questionnaire that covered nutrient intake and alcohol consumption. As risk estimates, we calculated adjusted odds ratios for the intake of energy and nutrients. Energy and nutrient intake did not differ between patients and controls, regardless of moderate alcohol consumption. Nutrition is not a risk factor for chronic idiopathic axonal polyneuropathy.

Published
2017

25. Drug therapy for chronic idiopathic axonal polyneuropathy

Author

Warendorf, Janna, Vrancken, Alexander F.J.E., van Schaik, Ivo N., Hughes, Richard A.C., Notermans, Nicolette C., Warendorf, Janna, Vrancken, Alexander F.J.E., van Schaik, Ivo N., Hughes, Richard A.C., and Notermans, Nicolette C.

Published
2017

26. Maintenance IV immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy

Author

ZL Neuromusculaire Ziekten Medisch, Brain, Apotheek Opleiding, Kuitwaard, Krista, Fokkink, Willem Jan R., Brusse, Esther, Vrancken, Alexander F.J.E., Eftimov, Filip, Notermans, Nicolette C., van der Kooi, Anneke J., Merkies, Ingemar S.J., Jacobs, Bart C., van Doorn, Pieter A., ZL Neuromusculaire Ziekten Medisch, Brain, Apotheek Opleiding, Kuitwaard, Krista, Fokkink, Willem Jan R., Brusse, Esther, Vrancken, Alexander F.J.E., Eftimov, Filip, Notermans, Nicolette C., van der Kooi, Anneke J., Merkies, Ingemar S.J., Jacobs, Bart C., and van Doorn, Pieter A.

Published
2017

27. Drug therapy for chronic idiopathic axonal polyneuropathy

Author

Opleiding Neurologie, ZL Neuromusculaire Ziekten Medisch, Brain, Warendorf, Janna, Vrancken, Alexander F.J.E., van Schaik, Ivo N., Hughes, Richard A.C., Notermans, Nicolette C., Opleiding Neurologie, ZL Neuromusculaire Ziekten Medisch, Brain, Warendorf, Janna, Vrancken, Alexander F.J.E., van Schaik, Ivo N., Hughes, Richard A.C., and Notermans, Nicolette C.

Published
2017

28. The role of nutrition as risk factor for polyneuropathy: a case-control study

Author

Opleiding Neurologie, ZL Neuromusculaire Ziekten Medisch, Brain, Projectafdeling ALS, Regenerative Medicine and Stem Cells, Visser, Nora A., Notermans, Nicolette C., de Vries, Jeanne H.M., van den Berg, Leonard H., Vrancken, Alexander F.J.E., Opleiding Neurologie, ZL Neuromusculaire Ziekten Medisch, Brain, Projectafdeling ALS, Regenerative Medicine and Stem Cells, Visser, Nora A., Notermans, Nicolette C., de Vries, Jeanne H.M., van den Berg, Leonard H., and Vrancken, Alexander F.J.E.

Published
2017

29. Relationships Between Type 2 Diabetes, Neuropathy, and Microvascular Dysfunction: Evidence From Patients With Cryptogenic Axonal Polyneuropathy

Author

Emanuel, Anna L., primary, Nieuwenhoff, Mariska D., additional, Klaassen, Erica S., additional, Verma, Ajay, additional, Kramer, Mark H.H., additional, Strijers, Rob, additional, Vrancken, Alexander F.J.E., additional, Eringa, Etto, additional, Groeneveld, Geert Jan, additional, and Serné, Erik H., additional

Published
2017
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32. Progressive idiopathic axonal neuropathy: A comparative clinical and histopathological study with vasculitic neuropathy.

Author

Vrancken, Alexander F.J.E., Notermans, Nicolette C., Jansen, Gerard H., Wokke, John H.J., and Said, Gérard

Subjects
*NEUROPATHY, *VASCULAR diseases, *AXONS, *IMMUNOGLOBULINS, *BIOPSY, *VASCULITIS
Abstract

Patients with a progressive disabling idiopathic axonal neuropathy could have a potentially treatable immune mediated neuropathy. To evaluate whether progressive idiopathic axonal neuropathy could be a pathologically difficult to prove vasculitic neuropathy pathologically difficult to prove or if it could be a separate clinical entity (i. e. with the axon as the primary immunological target), we performed a comparative clinical and histopathological study in 10 patients with progressive idiopathic axonal neuropathy, 10 patients with vasculitic neuropathy, and 12 patients with chronic idiopathic axonal polyneuropathy (CIAP). The clinical features and disease course in patients with progressive idiopathic axonal neuropathy and patients with vasculitic neuropathy were similar. Six patients with progressive idiopathic axonal neuropathy had been treated with prednisone and/or intravenous immunoglobulin. Disability decreased in all these six patients, but also in two of the four non-treated patients. Upon reviewing the sural nerve biopsy specimens, vasculitis was found in one patient with progressive idiopathic axonal neuropathy. Vasculitis-associated signs of ischemic injury or inflammation (most notably: large variation in fascicular axonal degeneration, perivascular inflammation, inflammation of the blood vessel wall without lumen obstruction) were found in four patients with progressive idiopathic axonal neuropathy, in all patients with vasculitic neuropathy, but were absent in patients with CIAP. The findings show that there is a small chance of finding sural nerve vasculitis upon scrutinising biopsy examination in progressive idiopathic axonal neuropathy. The presence of vasculitisassociated signs in progressive idiopathic axonal neuropathy suggests that some of these patients could have vasculitic neuropathy, even if vasculitic lesions cannot be demonstrated. However, if inflammatory changes cannot be demonstrated this does not preclude an immune-mediated origin. [ABSTRACT FROM AUTHOR]

Published
2004
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33. Dysfunction of the neuromuscular junction in spinal muscular atrophy types 2 and 3

Author

Wadman, Renske I., Vrancken, Alexander F.J.E., Berg, Leonard H. van den, and Pol, W. Ludo van der

Abstract

Spinal muscular atrophy (SMA) is pathologically characterized by degeneration of anterior horn cells. Recent observations in animal models of SMA and muscle tissue from patients with SMA suggest additional abnormalities in the development and maturation of the neuromuscular junction. We therefore evaluated neuromuscular junction function in SMA with repetitive nerve stimulation.

Published
2012
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