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32 results on '"P. L. Oey"'

1. P5370The effect of endovascular baroreflex amplification on sympathetic nerve activity in patients with resistant hypertension: a proof-of-mechanism study

Author: M E A M Van Kleef, Wilko Spiering, Jens Jordan, Bryan Williams, Peter J. Blankestijn, P L Oey, Jens Tank, Karsten Heusser, and André Diedrich
Subjects: medicine.medical_specialty, Mechanism (biology), business.industry, Internal medicine, medicine, Sympathetic nerve activity, Cardiology, Resistant hypertension, In patient, Baroreflex, Cardiology and Cardiovascular Medicine, business
Published: 2018
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2. Intramedullary femoral nailing through the trochanteric fossa versus greater trochanter tip

Author: H. J. ten Duis, G. A. P. de Kort, Chr. van der Werken, P. L. Oey, C.M. Ansari Moein, W. van der Meulen, and Faculteit Medische Wetenschappen/UMCG
Subjects: AVASCULAR NECROSIS, medicine.medical_specialty, Greater trochanter, Fossa, Greater trochanter tip, Avascular necrosis, FEMUR, Critical Care and Intensive Care Medicine, law.invention, Intramedullary rod, MUSCLE STRENGTH, Trochanteric fossa, law, PERFUSION, medicine, Orthopedics and Sports Medicine, Femur, HEAD, NECK, HIP, integumentary system, biology, business.industry, Piriform fossa, Entry point, Trauma center, Soft tissue, Antegrade femoral nailing, medicine.disease, biology.organism_classification, Surgery, SHAFT FRACTURES, body regions, medicine.anatomical_structure, Intramedullary nailing, RELIABILITY, Emergency Medicine, business
Abstract: In a level 1 university trauma center, an explorative randomized controlled study was performed to compare soft tissue damage and functional outcome after antegrade femoral nailing through a trochanteric fossa (also known as piriform fossa) entry point to a greater trochanter entry point in patients with a femoral shaft fracture.Nineteen patients were enrolled and randomly assigned to two nail insertion groups; ten patients were treated with an Unreamed Femoral Nail(A (R)) (UFN, Synthes(A (R)), Solothurn, Switzerland) inserted at the trochanteric fossa and nine patients were treated with an Antegrade Femoral Nail(A (R)) (AFN, Synthes(A (R)), Solothurn, Switzerland) inserted at the tip of the greater trochanter. The main outcome measures were pain, gait, nerve and muscle function, along with endurance. Magnetic resonance imaging (MRI), electromyography (EMG), and Cybex isokinetic testings were performed at, respectively, 2 and 6 weeks and at a minimum of 12 months after surgery.The MRI and EMG showed, in both groups, signs of iatrogenic abductor musculature lesions (four in the UFN group and four in the AFN group) and superior gluteal nerve injury (five in the UFN group and four in the AFN group). The isokinetic measurements and the patient-reported outcomes showed moderate reduction in abduction strength and endurance, as well as functional impairment with slight to moderate interference with daily life in both groups, with no appreciable differences between the groups.Anatomical localization of the entry point seems to be important for per-operative soft tissue damage and subsequent functional impairment. However, the results of this study did not show appreciable differences between femoral nailing through the greater trochanter tip and nailing through the trochanteric fossa.
Published: 2011
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3. Effect of gastric distension on cardiovascular parameters: gastrovascular reflex is attenuated in the elderly

Author: Paul A. F. Jansen, Louis M. A. Akkermans, L. J. van Schelven, J. M. M. Roelofs, P. L. Oey, and N. P. van Orshoven
Subjects: Mean arterial pressure, Physiology, business.industry, Gastric distension, Cold pressor test, Hemodynamics, Blood pressure, medicine.anatomical_structure, Anesthesia, Heart rate, Reflex, Vascular resistance, Medicine, medicine.symptom, business
Abstract: Stretching the stomach wall in young healthy subjects causes an increase in muscle sympathetic nerve activity and in blood pressure, the gastrovascular reflex. We compared healthy elderly subjects with healthy young subjects to find out whether the gastrovascular reflex attenuates in normal ageing and we studied whether there was a difference in autonomic function or gastric compliance that could explain this possible attenuation. Muscle sympathetic nerve activity, finger blood pressure and heart rate were continuously recorded during stepwise isobaric gastric distension using a barostat in eight healthy young (6 men and 2 women, 27 ± 3.2 years, mean ±s.e.m.) and eight healthy elderly subjects (7 men and 1 woman, 76 ± 1.5 years). Changes in cardiac output and total peripheral arterial resistance were calculated from the blood pressure signal. The baseline mean arterial pressure and muscle sympathetic nerve activity were higher in the elderly group (both P < 0.05) and muscle sympathetic nerve activity increase during the cold pressor test was lower in the elderly group (P = 0.005). During stepwise gastric distension, the elderly subjects showed an attenuated increase in muscle sympathetic nerve activity compared to the young subjects (P < 0.01). The older group tended to show a higher increase in mean arterial pressure (P = 0.08), heart rate (P = 0.06) and total peripheral arterial resistance (P = 0.09) The cardiac output rose slightly in both groups without significant difference between groups. The fundic compliance did not differ between groups. We conclude that stepwise gastric distension caused an increase in muscle sympathetic nerve activity in both groups, but the increase in the elderly was attenuated.
Published: 2004
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4. Small-fibre neuropathy can be detected in patients with chronic idiopathic axonal polyneuropathy

Author: E. L. L. M. De Schryver, N. Notermans, P. L. Oey, L. J. van Schelven, and H. W. de Valk
Subjects: medicine.medical_specialty, Clinical pathology, business.industry, Diaphragmatic breathing, medicine.disease, Gastroenterology, Axonal polyneuropathy, Surgery, Neurology, Diabetes mellitus, Internal medicine, Sensory threshold, medicine, Small Fibre Neuropathy, In patient, Neurology (clinical), business, Burning Pain
Abstract: Background: The main sensory presenting symptoms of chronic idiopathic axonal polyneuropathy (CIAP) are paraesthesias, numbness and burning pain in the feet. Although these symptoms indicate the involvement of small nerve fibres, clinical analysis or electrophysiological investigations have not yet been studied in detail. Method: Cardiovascular autonomic tests and cold and heat pain perception threshold tests were performed in 10 patients with CIAP, 10 patients with diabetes mellitus (DM) and 10 healthy volunteers. The results of the DM group were used to see whether the tests were able to detect small-fibre neuropathy in patients with diabetes and pain. Results: Quantitative sensory threshold and autonomic tests showed more frequent abnormal test results in the patients compared to the healthy control group. The proportion of abnormal test results reached significance for the deep breathing tests in both patient groups and for the cold threshold and heat pain test in patients with CIAP. The spectral analysis of RR intervals showed a significant decrease in the high frequency in both patients with DM and CIAP. Conclusion: The results of this study demonstrated that small-fibre neuropathy can be detected in patients with CIAP.
Published: 2010
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5. Cold Stress Provokes Sympathoinhibitory Presyncope in Healthy Subjects and Hemodialysis Patients With Low Cardiac Output

Author: Gerry Ligtenberg, Peter J. Blankestijn, A. C. van Huffelen, Hendrik A. Koomans, P. L. Oey, and G.H. Wieneke
Subjects: Adult, Tachycardia, Cardiac output, Sympathetic nervous system, Sympathetic Nervous System, Cardiac Output, Low, Hemodynamics, Syncope, Reference Values, Renal Dialysis, Stress, Physiological, Physiology (medical), Hypovolemia, medicine, Humans, Lower Body Negative Pressure, Presyncope, business.industry, Cold pressor test, Neural Inhibition, Middle Aged, medicine.disease, Cold Temperature, medicine.anatomical_structure, Anesthesia, medicine.symptom, Cardiology and Cardiovascular Medicine, business, Vasoconstriction
Abstract: Background Sudden hypotension in progressive hypovolemia or during hemodialysis is attributed to sudden inhibition of sympathetic activity. Critical ventricular underfilling seems responsible for this paradox, but it is unknown why the transition from sympathoactivation accompanying hypovolemia to sympathoinhibition is so abrupt. We studied whether brief fluctuation of sympathetic activity induced by cold pressor test (CPT) evokes sympathoinhibition if applied during low cardiac output. Methods and Results Fourteen healthy subjects underwent CPT, lower-body negative pressure (LBNP; −45 mm Hg for 60 minutes), or the combination thereof. CPT alone caused vasoconstriction and increased muscle sympathetic nerve activity, followed by uneventful relaxation. When applied during reduced cardiac output, tachycardia, and vasoconstriction induced by prior LBNP for 6 minutes, CPT again caused vasoconstriction, now followed by acute hypotension in 10 subjects, and was associated with vasorelaxation, relative bradycardia, and fall in muscle sympathetic nerve activity. Eight subjects also experienced acute LBNP-induced hypotension in the absence of CPT, but not until 17±6 minutes of LBNP. We also performed CPT before and in the final phase of hemodialysis in 8 patients. Before dialysis, the patients tolerated CPT uneventfully, but during hemodialysis, CPT provoked acute hypotension in 5 cases, showing similar withdrawal of vasoconstriction. Conclusions This is the first study showing that brief cold stress, tolerated well in normal circulatory conditions, can provoke sudden sympathoinhibition and hypotension when applied during decreased cardiac output induced by LBNP or hemodialysis. We suggest that during conditions of a decreased cardiac output, subtle sympathetic relaxation such as follows cold stress triggers self-enhancing relaxation that cannot be controlled.
Published: 1997
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6. Response to intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy with only sensory symptoms

Author: G. W. Van Dijk, P. L. Oey, J.H.J. Wokke, H. Franssen, and Nicolette C. Notermans
Subjects: Male, medicine.medical_specialty, Neurology, Chronic inflammatory demyelinating polyneuropathy, Gastroenterology, Cerebrospinal fluid, hemic and lymphatic diseases, Internal medicine, Humans, Medicine, Prospective cohort study, Pathological, Subclinical infection, Neuroradiology, business.industry, Immunoglobulins, Intravenous, Middle Aged, Prognosis, medicine.disease, Surgery, Female, Neurology (clinical), business, Polyneuropathy, Demyelinating Diseases
Abstract: In an open prospective study we analysed the effect of treatment with intravenous immunoglobulin (IvIg) in three patients with clinically pure sensory neuropathy, two of whom met the clinical, electrophysiological, pathological and cerebrospinal fluid research criteria of the American Academy of Neurology for chronic inflammatory demyelinating polyneuropathy. In all patients, subclinical signs of demyelination were present in motor nerves. Treatment with IvIg resulted in improvement of neurological functions in all three patients and in improvement of the disability score in two of them.
Published: 1996
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7. Differential effects of renin-angiotensin-aldosterone system inhibition, sympathoinhibition and diuretic therapy on endothelial function and blood pressure in obesity-related hypertension: a double-blind, placebo-controlled cross-over trial

Author: Peter G. Scheffer, Ilse M. Schrover, Wilko Spiering, Frank L.J. Visseren, P L Oey, A.H. Danser, Johannes Dorresteijn, Internal Medicine, Clinical chemistry, and ICaR - Circulation and metabolism
Subjects: Male, medicine.medical_specialty, Physiology, medicine.medical_treatment, Urology, Placebo, Placebos, Renin-Angiotensin System, chemistry.chemical_compound, Hydrochlorothiazide, Double-Blind Method, SDG 3 - Good Health and Well-being, Internal medicine, Renin–angiotensin system, Internal Medicine, Humans, Medicine, Obesity, Diuretics, Cross-Over Studies, Moxonidine, business.industry, Middle Aged, Aliskiren, medicine.disease, Endocrinology, Blood pressure, chemistry, Pathophysiology of hypertension, Hypertension, Female, Endothelium, Vascular, Diuretic, Cardiology and Cardiovascular Medicine, business, medicine.drug
Abstract: Objectives The objective of this study is to determine the effects of renin-angiotensin-aldosterone system inhibition, sympathoinhibition and diuretic therapy on endothelial function and blood pressure in obesity-related hypertension. Methods A randomized, four-way, double-blind, crossover study in 31 adults with previously untreated obesity-related hypertension, in which the effects of 8 weeks' inhibition of the renin-angiotensin-aldosterone system (using aliskiren 300 mg), sympathoinhibition (using moxonidine 0.4 mg), diuretic therapy (using hydrochlorothiazide 25 mg) or placebo on flow-mediated dilation and 24-h blood pressure were compared. Results The median flow-mediated dilation during placebo was 4.0% [interquartile range (IQR) 2.9-5.5%] and was increased by aliskiren [0.81%, 95% confidence interval (CI) 0.02-1.79], but not by moxonidine (0.20%, 95% CI -0.46 to 1.03) or hydrochlorothiazide (0.39%, 95%CI -0.31%-1.26%). Similarly, compared with placebo, mean 24-h blood pressure was most reduced by aliskiren (-9.8/-6.3 mmHg) and to a lesser degree by hydrochlorothiazide (-5.9/-2.6 mmHg). Moxonidine did not significantly affect blood pressure despite reduction of muscle sympathetic nerve activity. Insulin sensitivity deteriorated during hydrochlorothiazide treatment and was unaffected by aliskiren or moxonidine. Unlike aliskiren and moxonidine, hydrochlorothiazide reduced urinary 8-iso-prostaglandin F2α-VI, a marker of oxidative stress. Vascular stiffness, systemic inflammation, leptin, adiponectin and other oxidative stress markers (plasma malondialdehyde, myeloperoxidase activity and urinary 8-hydroxydeoxyguanosine) were unaffected by treatment. Conclusion Renin inhibition, but not sympathoinhibition or diuretic therapy, improves endothelial function and results in larger reductions of 24-h, office, and central blood pressure in obesity-related hypertension. This adds weight to the hypothesis that inhibition of the renin-angiotensin-aldosterone system is an effective first step in the treatment of obesity-related hypertension.
Published: 2013
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8. Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulins: a double blind, placebo controlled study

Author: L. H. van den Berg, P. L. Oey, Marinus Vermeulen, I. Mollee, J.H.J. Wokke, H. Kerkhoff, Frans G. I. Jennekens, and H. Franssen
Subjects: Adult, Male, medicine.medical_specialty, Placebo-controlled study, Mismatch negativity, Placebo, Double blind, Central nervous system disease, Double-Blind Method, hemic and lymphatic diseases, medicine, Humans, Motor Neuron Disease, business.industry, Muscles, Immunoglobulins, Intravenous, Middle Aged, medicine.disease, Surgery, Clinical trial, Psychiatry and Mental health, Intravenous Immunoglobulins, Anesthesia, Female, Neurology (clinical), business, Research Article, Multifocal motor neuropathy
Abstract: The effect of high dose intravenous immunoglobulin (IVIg) treatment was studied in six patients with multifocal motor neuropathy (MMN). All patients responded to treatment (0.4 g/kg for five consecutive days) in an open trial. The effect of IVIg treatment was confirmed for each patient in a single patient, double blind, placebo controlled trial. Four patients received two IVIg treatments and two placebo treatments, and two patients received one IVIg and one placebo treatment in a randomised order. Five out of six patients responded to IVIg but not to placebo. One patient responded to IVIg in the same manner as to placebo treatment. Thus IVIg treatment can lead to improvement of muscle strength in patients with MMN.
Published: 1995
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9. A new variant of sensory ataxic neuropathy with autosomal dominant inheritance

Author: H. Veldman, P. F. Ippel, J.H.J. Wokke, H. Franssen, G. W. Van Dijk, and P. L. Oey
Subjects: Male, Cerebellum, Pathology, medicine.medical_specialty, Ataxia, Cerebellar Ataxia, Neural Conduction, Sural nerve, Late onset, Neurological disorder, Somatosensory system, Sensory ataxia, Sural Nerve, Evoked Potentials, Somatosensory, Evoked Potentials, Auditory, Brain Stem, medicine, Humans, Motor Neurons, business.industry, Middle Aged, medicine.disease, Pedigree, Peripheral neuropathy, medicine.anatomical_structure, Female, Neurology (clinical), medicine.symptom, Hereditary Sensory and Motor Neuropathy, business, Neuroscience
Abstract: We describe a Dutch family with sensory ataxia in two generations, late onset of symptoms (over the age of 40 years) and slow progression. Clinical, electrophysiological and sural nerve biopsy findings revealed a sensory polyneuropathy due to axonal degeneration of myelinated nerve fibres in four of five investigated siblings. Other neurological abnormalities in the affected family members consisted only of mild eye movement disturbances, probably due to cerebellar involvement. Five other family members were investigated and found unaffected. As the disease is inherited from the affected father to his sons and daughters, this is the first description of a probably autosomal dominant form of late onset hereditary sensory neuropathy with predominant sensory ataxia and minor other neurological abnormalities.
Published: 1995
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10. Features of the Guillain-Barré syndrome in mice following intraperitoneal injection of patient serum

Author: John H. J. Wokke, P. L. Oey, L.H. van den Berg, H. Veldman, G. H. Wieneke, and S.H.W. Notermans
Subjects: Adult, Male, Pathology, medicine.medical_specialty, Ratón, medicine.medical_treatment, Intraperitoneal injection, Polyradiculoneuropathy, Stimulation, H-Reflex, Biological Factors, Mice, medicine, Animals, Humans, Infectious Mononucleosis, Peripheral Nerves, Reflex, Abnormal, Guillain-Barre syndrome, business.industry, medicine.disease, Sciatic Nerve, Pathophysiology, Neurology, Reflex, Neurology (clinical), Sciatic nerve, H-reflex, business, Injections, Intraperitoneal
Abstract: Intraperitoneal injection of serum from a patient with the Guillain-Barré syndrome (GBS) produced GBS-like signs in mice: inadequate respiration and weakness in the legs. We studied the clinical, electrophysiological and pathological features of these mice. Three groups of three mice were injected with patient serum from days 6, 10 and 15 after onset of neurological symptoms. GBS-like signs in mice were observed only with serum from day 6 and improved within 48 h. When serum was frozen and thawed more than once no signs were seen. Electrophysiological measurements of the sciatic nerves of injected and control mice were done before and after serum injection. Five days after injection of patient serum of day 6, the mice showed a significant decrease in the ratio between CMAP amplitude from proximal and distal stimulation and increase in H-M interval from proximal stimulation. These electrophysiological changes returned to normal within 12 days. The sciatic nerve showed no morphological abnormalities. Our results indicate that the observed GBS-like signs in mice are caused by peripheral nerve dysfunction.
Published: 1994
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11. Fourth meeting of the European Neurological Society 25–29 June 1994 Barcelona, Spain

Author: H. Hattig, C. Delli Pizzi, M. C. Addonizio, Michelle Davis, A. R. Giovagnoli, L. Florensa, M. Roth, J. de Kruijk, Francisco Lacruz, Ph. Dewailly, A. Toygar, C. Avendano, P.P. De Deyn, J. F. Hurtevent, F. Lomeila, T. W. Wong, Gordon T. Plant, M. Bud, H. J. Willison, DH Miller, D. W. Langdon, R. Cioni, J. Servan, A. Kaygisiz, E. Racadot, D. B. Schens, E. Picciola, L. Falip, C. Bouchard, J. Jotova, A. Jorge-Santamaria, P. Misra, A. Dufour, C. P. Panagopoulos, A. Venneri, B. Sredni, B. Angelard, M. Janelidze, M. Carreno, J. Obenberger, J. Pouget, H. W. Moser, R. Kaufmann, J. A. Molina, D. Linden, A. Martin Urda, E. Uvestad, A. Krone, J. P. Cochin, J. Mallecourt, A. Cambon-Thomsen, K. Violleau, P. Osschmann, A. M. Durocher, E. Bussaglia, D. M. Danielle, H. Efendi, C. Van Broeckhoven, K. G. Jordan, W. Rautenberg, C. Iniguez, J. M. Delgado, Graham Watson, M. Lawden, Gareth J. Barker, K. Stiasny, James T. Becker, G. Campanella, E. Peghi, A. Poli, A. Haddad, T. Yamawaki, Giacomo P. Comi, S. Sotgiu, B. Ersmark, A. Pomes, M. Ziegler, P. Ferrante, P. Ruppi, H. KuÇukoglu, R. Bouton, U. K. Rinne, P. Vieregge, M. Dary, P. Giunti, Peter J. Goadsby, S. Jung, E. Secor, A. Steinberg, N. Vila, M. A. Hernandez, M. Cursi, A. Enqelhardt, A. Engelhardt, J. Veitch, F. Di Silverio, F. Arnaud, B. Neundörfer, R. Brucher, Dominique Caparros-Lefebvre, B. Meyer, Marianne Dieterich, M. H. Snidaro, R. Gomez, R. Cerbo, M. Ragno, J. M. Vance, S. Nemni, A. Caliskan, F. Barros, I. Velcheva, D. Ceballos-Baumann, V. Barak, A. Avila, N. Antonova, F. Resche, S. Pappata, L. Varela, S. R. Silveira Santos, A. Cammarota, L. Naccache, Y. Nara, E. Tournier-Lasserves, R. Mobner, T. Chase, A. Ensenyat, J. Ulrich, G. Giegerich, M. Rother, M. Revilla, N. Nitschke, K. Honczarenko, E. Basart Tarrats, J. Blin, B. Jacob, J. Santamaria, S. Knezevic, J. L. Castillo, M. Antem, J. Colomer, O. Busse, Didier Hannequin, S. Carrier, J. B. Ruidavets, C. Rozman, J. Bogoussslavsky, J. Pascual Calvet, E. Monros, J. M. Polo, M. Zucconl, Javier Muruzabal, R. R. Allen, R. Rivolta, K. Haugaard, A. Nespolo, K. Hoang-Xuang, G. Bussone, T. Avramidis, E. Corsini, Christiana Franke, T. Vinogradova, H. Boot, K. Vestergaard, G. H. Jansen, N. Argentino, M. Raltzig, W. Linssen, Mark B. Pepys, P. Roblot, L. Lauritzen, E. Fainardi, D. Morin, T. X. Arbizu Urdiain, J. Wollenhaupt, S. Bostantjopoulou, G. Pavesi, A. D. Forman, Giovanni Fabbrini, D. Jean, J. J. Archelos, M. I. Blanchs, M. Del Gobbo, Anna Carla Turconi, Ch. Derouesné, Elio Scarpini, A. Visbeck, P. Castejon, J. P. Renou, F. Mounier-Vehier, G. Potagas, Ch. Duyckaerts, A. Filla, R. Schneider, G. Ronen, K. Nagata, J. P. Vedel, A. Henneberg, G. van Melle, C. Baratti, H. Knott, M. C. Prevett, A. Bes, B. Metin, Jos V. Reempts, L. Martorell, Mefkure Eraksoy, H. O. Handwerker, D. S. Younger, O. Oktem, D. Frongillo, C. Soriano-Soriano, L. Niehaus, F. Zipp, A. Tartaro, S Newman, R. H. Browne, P. Davous, R. Sanchez, M. Muros, M. E. Kornhuber, A. Lavarone, M. Mohr, M. R. Garcia, S. Russell, H. Kellar-Wood, M. R. Tola, B. Ostermeyer, Ch. Tzekov, K. Sartor, E. B. Ringelstein, P. P. Gazzaniga, Paul Krack, H. Fidaner, H. Rico, T. Dbaiss, F. Alameda, E. Torchiana, L. Rumbach, I. Charques, J. M. Bogaard, C. D. Frith, L. J. Rappelle, R. Brenner, A. Joutel, K. Fuxe, G. HÄcker, M. J. Blaser, J. Valls-SolÇ, G. Ulm, M. Alberdi, A. Bock, F. W. Bertelsmann, U. Wieshmann, J. Visa, J. R. Lupski, D. D'Amico, L. M. P. Ramos, A. A. Vanderbark, R. Horn, M. Warmuth, Dietmar Kühne, Mark S. Palmer, C. Ehrenheim, E. Canga, S. Viola, O. Scarpino, P. Naldi, R. Almeida, A. A. Raymond, J. Gamez, Stephan Arnold, A. DiGiovanni, J. Dalmau, C. C. Chari, H. F. Beer, J. C. Koetsier, J. Iriarte, E. Yunis, J. Casadevall, E. Le Guern, E. Stenager, S. R. Benbadis, J. M. Warter, F. Burklin, I. Theodorou, L. Johannesen, G. A. Graveland, X. Leclerc, I. Vecchio, L. Ozelius, G. Nicoletti, R. K. Gherardi, E. Esperet, M. L. Delodovici, F. Cattin, F. Paiau, Giorgio Sacilotto, C. A. J. Broere, D. Chavdarov, J. P. Willmer, C. H. Hawkes, Th. Naegele, E. Ellie, E. Dartigues, M. J. Guardiola, S. Hesse, Z. Levic, Marco Rovaris, P. Saugeir-Veber, B. A. Yaqub, H. F. Durwen, R. Larumbe, J. Ballabrina, M. Sendtner, J. Röther, M. Horstink, C. Kluglein, M.P. Montesi, H. Apaydin, J. Montoya, E. Waubant, Ch. Verellen-Dunoulin, A. Nicolai, J. Lopez-Delval, R. Lemon, G. Cantinho, E. Granieri, A. Zeviani, Wolfgang H. Oertel, U. Ficola, V. Di Piero, V. Fragola, K. Sabev, M. V. Guitera, I. Turki, F. Bolgert, P. Ingrand, J. M. Gobernado, L. M. E. Grimaldi, S. Baybas, B. Eymard, Y. Rolland, Y. Robitaille, Ta. Pampols, P. J. Koehler, A. Carroacedo, J. Vilchez, S. Di Vittorio, I. R. Rise, T. Nagy, M. Kuffner, E. Palazzini, A. Ott, J. Pruim, T. X. Arbizu, E. Manetti, C. Cervera, S. Felber, G. Gursoy, J. Scholz, G. A. Buscaino, M. S. Chen, A. Pascual, J. Hazan, J. U. Gajda, J. G. Cea, G. Bottini, G. Damalik, F. Le Doze, G. Bonaldi, J. M. Hew, C. Messina, A. M. Kennedy, J. M. Carney, N. M. F. Murray, M. Parent, M. Koepp, V. Dimova, D. De Leo, K. Jellinger, G. Salemi, S. Mientus, M. L. Hansen, F. Mazzucchelli, J. Vieth, M. Mauri, E. Bartels, L. Johannsen, C. Humphreys, J. Emile, D. N. Landon, E. Kansu, R. Sanchez-Pernaute, Rsj Frackowiak, M. Gonzalez Torres, L. Oller, C. Machedo, J. Kother, M. Billiard, H. Durak, T. Schindler, A. Frank, A. Uncini, A. Sbriccoli, C. Farinas, D. W. Paty, N. Fast, A. T. Zangaladze, A. Kerkhofs, J. M. Pino Garcia, I. De la Fuente, B. Marini, L. Gomez, I. Rubio, Alessandra Bardoni, C. Brodie, P. Acin, U. Sliwka, S. A. Hawkins, S. Tardieu, F. Vitullo, J. M. Pereira Monteino, R. Gagliardi, T. Jezewski, A. Cano, T. Lempert, F. Abad Alegria, G. Rotondo, D. Ince, C. Martinez Parra, Y. Huang, H. Luders, Y. Steinvil, F. G. A. Van Der Meche, R. Bianchi, A. Sanchez, T. Sevilla, J. M. Ketelslegers, A. Domzal-Stryga, M. Pandolfo, M. O. Josse, K. W. Neff, I. Blanco, G. W. Bruyn, O. W. Witte, J. L. Thibault, G. Andersen, J. Pariset, A. Marcone, R. J. M. Lane, A. Hofman, M. Verin, T. Matilla, P. Bedoucha, J. Roche, M. Lai, M. Collard, A. Ugarte, F. Gallecho, D. Silbersweig, C. Kennard, J. P. Azulay, T. W. Ho, P. L. I. Dellemijn, R. Girardello, F. Baas, B. Voss, F. Rozenberg, E. M. Brocker, V. Stanev, A. A. J. Soeterboek, A. Marra, A. Rey, E. Ertem, M. Sawradewicz-Rybak, J. De Keyser, P. Cavallari, F. Proust, Y. Chevalier, H. C. Hansen, D. Leys, C. A. Davie, K. Hoang-Xuan, C. Bairati, H. van Crevel, Thomas T. Warner, B. Bompais, A. Dobbeleir, T Campbell, C. Macko, C. J. M. Klijn, M. Dussallant, T. P. Berlit, W. Rozenbaum, M. J. van den Bent, W. A. Rocca, M. Muller, H. Hundemer, U. Zifko, M. Campera, F. Drislane, D. Ranoux, T. M. Kloss, Anil Kumar, I. Ruolt, C. Bargnani, B. Marescau, N. A. Losseff, S. Notermans, B. Kint, E. T. Burke, C. Aykut, J. Matias Guiu, P. Maquet, T. Drogendijk, M. Leone, K. von Ammon, M. Pepeliarska, C. Prados, L. DiGiamberardino, T. Logtenberg, G. Lenoir, I. Castaldo, Damhaut, M. Radionova, G. Sirabian, R. 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Frigo, Leif Østergaard, J. L. Munoz Blanco, A. Cruz-Matinez, J. De Reuck, C. Theillet, T. Barroso, V. Oikonen, Florence Lebert, M. Kilinc, C. Cordon-Cardon, G. Stoll, E. Thiery, F. Pulcinelli, J. Solski, M. Schmiegelow, L. J. Polman, P. Fernandez-Calle, C. Wikkelso, M. Ben Hamida, M. Laska, E. Kott, W. Sulkowski, C. Lucas, N. M. Bornstein, D. Schmitz, M. W. Lammers, A. de Louw, R. J. S. Wise, P. A. van Darn, C. Antozzi, P. Villanueva, P. H. E. Hilkens, C. Constantin, W. Ricart, A. Wolf, M. Gamba, P. Maguire, Alessandro Padovani, B. M. Patten, Marie Sarazin, H. Ackermann, L. Durelli, S. Timsit, Sebastian Jander, B. W. Scheithauer, G. Demir, J. P. Neau, P. Barbanti, A. Brand, N. AraÇ, V. Fischer-Gagnepain, R. Marchioli, G. Serratrice, C. Maugard-Louboutin, G. T. Spencer, D. Lücke, G. Mainardi, K. Harmant Van Rijckevorsel, G. B. Creel, R. Manzanares, Francesco Fortunato, A. May, J. Workman, K. Johkura, E. Fernandez, Carlo Colosimo, L. Calliauw, L. Bet, Félix F. Cruz-Sánchez, M. Dhib, H. Meinardi, F. Carrara, J. Kuehnen, C. Peiro, H. Lassmann, K. Skovgaard Olsen, A. McDonald, L. Sciulli, A. Cobo, A. Monticelli, B. Conrad, J. Bagunya, J. Benitez, V. Desnizza, B. Dupont, O. Delrieu, D. Moraes, J. J. Heimans, F. Garcia Rio, M. Matsumto, A. Fernandez, R. Nermni, R. Chalmers, M. J. Marchau, F. Aguado, P. Velupillai, P. J. Martin, P. Tassan, V. Demarin, A. Engelien, T. Gerriets, Comar, J. L. Carrasco, J. P. Pruvo, A. Lopez de Munain, D. Pavitt, J. Alarcon, Chris H. Polman, B. Guldin, N. Yeni, Hartmut Brückmann, N. Wilczak, H. Szwed, R. Causaran, G. Kyriazis, M. E. Westarp, M. Gasparini, N. Pecora, J. M. Roda, E. Lang, V. Scaioli, David R. Fish, D. Caputo, O. Gratzl, R. Mercelis, A. Perretti, G. Steimetz, I. Link, C. Rigoletto, A. Catafau, G. Lucotte, M. Buti, G. Fagiolari, A. Piqueras, C. Godinot, J. C. Meurice, Erodriguez J. Dominigo, F. Lionnet, H. Grzelec, David J. Brooks, P. M. G. Munro, F. X. Weilbach, M. Maiwald, W. Split, B. Widjaja-Cramer, V. Ozturk, J. Colas, E. Brizioli, J. Calleja, L. Publio, M. Desi, R. Soffietti, P. Cortinovis-Tourniaire, E. F. Gonano, G. Cavaletti, S. Uselli, K. Westerlind, H. Betuel, C. O. Dhiver, H. Guggenheim, M. Hamon, R. Fazio, P. Lehikoinen, A. Esser, B. Sadzot, G. Fink, Angelo Antonini, D. Bendahan, V. Di Carlo, G. Galardi, A. F. Boller, M. Aksenova, Del Fiore, V. de la Sayette, H. Chabriat, A. Nicoletti, A. Dilouya, M. L. Harpin, E. Rouillet, J. Stam, A. Wolters, M. R. Delgado, Eduardo Tolosa, G. Said, A. J. Lees, L. Rinaldi, A. Schulze-Bonhage, MA Ron, C. Lefebvre, E. W. Radü, R. Alvarez, M. L. Bots, P. Reganati, S. Palazzi, A. Poggi, N. J. Scolding, V. Sazdovitch, T. Moreau, E. Maes, M. A. Estelies, P. Petkova, Jose-Felix Marti-Masso, G De La Meilleure, N. Mullatti, M. Rodegher, N. C. Notermans, T. A. T. Warner, S. Aktan, J. P. Louboutin, L. Volpe, C. Scheidt, W. Aust, C. M. Wiles, U. Schneider, S. K. Braekken, W. R. Willems, K. Usuku, Peter M. Rothwell, C. Talamon, M. L. Sacchetti, A. Codina, M. H. Marion, A. Santoro, J. Roda, A. Bordoni, D. J. Taylor, S. Ertas, H. H. Emmen, J. Vichez, V. BesanÇon, R. E. Passingham, M. L. Malosio, A. Vérier, M. Bamberg, A. W. Hansen, E. Mostacero, G. Gaudriault, Marie Vidailhet, B. Birebent, K. Strijckmans, F. Giannini, T. Kammer, I. Araujo, J. Nowicki, E. Nikolov, A. Hutzelmann, R. Gherardi, J. Verroust, L. Austoni, A. Scheller, A. Vazquez, S. Matheron, H. Holthausen, J. M. Gerard, M. Bataillard, S. Dethy, V. H. Patterson, V. Ivanez, N. P. Hirsch, F. Ozer, M. Sutter, C. Jacomet, M. Mora, Bruno Colombo, A. Sarropoulos, T. H. Papapetropoulos, M. Schwarz, D. S. Dinner, N. Acarin, B. Iandolo, J. O. Riis, P. R. J. Barnes, F. Taroni, J. Kazenwadel, L. Torre, A. Lugaresi, I. L. Henriques, S. Pauli, S. Alfonso, Pedro Quesada, A. S. T. Planting, J. M. Castilla, Thomas Gasser, M. Van der Linden, A. Alfaro, E. Nobile-Orazio, G. Popova, W. Vaalburg, F. G. A. van der Mech, L. Williams, F. Medina, J. P. Vernant, J. Yaouanq, B. Storch-Hagenlocher, A. Potemkowski, R. Riva, M. H. Mahagne, M. Ozturk, Ve. Drory, N. Konic, C. Jungreis, A. Pou Serradell, J. L. Gauvrit, G. J. Chelune, S. Hermandez, T. Dingus, L. Hewer, Ch. Koch, M. N. Metz-Lutz, G. Parlato, M. Sinaki, Charles Pierrot-Deseilligny, H. C. Diener, J. Broeckx, J. Weill-Fulazza, M. L. Villar, M. Rizzo, O. Ganslandt, C. Duran, N. A. Fletcher, G. Di Giovacchino, Susan T. Iannaccone, C. Kolig, N. Fabre, H. A. Crockard, Rita Bella, M. Tazir, E. Papagiannuli, K. Overgaard, Emma Ciafaloni, I. Lorenzetti, F. Viader, P. A. H. Millac, I. Montiel, L. H. Visser, M. Palomar, P. L. Murgia, H. Pedersen, Rafael Blesa, S. Seddigh, W. O. Renier, I. Lemahieu, H. M. L. Jansen, L. Rosin, J. Galofre, K. Mattos, M. Pondal, G. M. Hadjigeorgiou, D. Francis, L. Cantin, D. Stegeman, M. Rango, A. B. M. F. Karim, S. Schraff, B. Castellotti, I. Iriarte, E. Laborde, T. J. Tjan, R. Mutani, D. Toni, B. Bergaasco, J. G. Young, C. Klotzsch, A. Zincone, X. Ducrocq, M. Uchuya, O. J. Kolar, A. Quattrone, T. Bauermann, Nereo Bresolin, J. Vallée, B. C. Jacobs, A. Campos, Werner Poewe, J. A. Villanueva, A. W. Kornhuber, A. Malafosse, E. Diez-Tejedor, G. Jungreia, M. J. A. Puchner, A. Komiyama, O. Saribas, V. Volpini, L. Geremia, S. Bressi, A. Nibbio, Timothy E. Bates, T. z. Tzonev, E. Ideman, G. A. Damlacik, G. Martino, G. Crepaldi, T. Martino, Kjell Någren, E. Idiman, D. Samuel, J. M. Perez Trullen, Y. van der Graaf, J. O. Thorell, M. J. M. Dupuis, E. Sieber, R. D'Alessandro, C. Cazzaniga, J. Faiss, A. Tanguy, A. Schick, I. Hoksergen, A. Cardozo, R. Shakarishvili, G. K. Wennlng, J. L. Marti-Vilalta, J. Weissenbach, I. L. Simone, Amalia C. Bruni, Darius J. Adams, C. Weiller, A. Pietrangeli, F. Croria, C. Vigo-Pelfrey, Patricia Limousin, A. Ducros, G. Conti, O. Lindvall, E. Richter, M. Zuffi, A. Nappo, T. Riise, J. Wijdenes, M. J. Fernandez, J. Rosell, P. Vermersh, S. Servidei, M. S. C. Verdugo, F. Gouttiere, W. Solbach, M. Malbezin, I. S. Watanabe, A. Tumac, W. I. McDonald, D. A. Butterfield, P. P. Costa, F. deRino, F. Bamonti, J. M. Cesar, C. H. Lahoz, I. Mosely, M. Starck, M. H. Lemaitre, K. M. Stephan, S. Tex, R. Bokonjic, I. Mollee, L. Pastena, M. Gutierrez, F. Boiler, M. C. Martinez-Para, M. Velicogna, O. Obuz, A. Grinspan, M. Guarino, L. M. Cartier, E. Ruiz, D. Gambi, S. Messina, M. Villa, Michael G. Hanna, J. Valk, Leone Pascual, M. Clanet, Z. Argov, B. Ryniewicz, E. Magni, B. Berlanga, K. S. Wong, C. Gellera, C. Prevost, F. Gonzalez-Huix, R. Petraroli, J. E. G. Benedikz, I. Kojder, C. Bommelaer, L. Perusse, M. R. Bangioanni, Guy M. McKhann, A. Molina, C. Fresquet, E. Sindern, Florence Pasquier, M. J. Rosas, M. Altieri, O. Simoncini, M. Koutroumanidis, C. A. F. Tulleken, M. Dary-Auriol, S. Oueslati, H. Kruyer, I. Nishisho, C. R. Horning, A. Vital, G. V. Czettritz, J. Ph. Neau, B. Mihout, A. Ameri, M. Francis, S. Quasthoff, D. Taussig, S. Blunt, P. Valentin, C. Y. Gao, O. Heinzlef, H. d'Allens, C. Coudero, M. Erfas, G. Borghero, P. J. Modrego Pardo, M. C. Patrosso, N. L. Gershfeld, P. A. J. M. Boon, O. Sabouraud, M. Lara, J. Svennevig, G. L. Lenzi, A. Barrio, H. Villaroya, JosÇ M. Manubens, O. Boespflug-Tanguy, M. Carreras, D. A. Costiga, J. P. Breux, S. Lynn, C. Oliveras Ley, A. G. Herbaut, J. Nos, C. Tornali, Y. A. Hekster, J. L. Chopard, J. M. Manubens, P. Chemouilli, A. Jovicic, F. Dworzak, S. Smirne, S. E. Soudain, B. Gallano, D. Lubach, G. Masullo, G. Izquierdo, A. Pascual Leone Pascual, A. Sessa, V. Freitas, O. Crambes, L. Ouss, G. W. Van Dijk, P. Marchettini, P. Confalonieri, M. Donaghy, A. Munnich, M. Corbo, and M. E. L. van der Burg
Subjects: Neurology, business.industry, Media studies, Library science, Medicine, Neurology (clinical), business
Published: 1994
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12. Randomized double-blind placebo-controlled trial to evaluate the effect of the ACTH4–9 analogue ORG 2766 in IDDM patients with neuropathy

Author: D.W. Erkelens, Bert Bravenboer, W.H. Gispen, P. L. Oey, A.C. van Huffelen, P. H. Hendrikse, and C. Groenhout
Subjects: Adult, Male, medicine.medical_specialty, Diabetic neuropathy, Randomization, Injections, Subcutaneous, Endocrinology, Diabetes and Metabolism, medicine.medical_treatment, Placebo-controlled study, Placebo, Perceptual Disorders, Geneeskunde, Vibration perception, Adrenocorticotropic Hormone, Diabetic Neuropathies, Double-Blind Method, Internal Medicine, medicine, Humans, Chemotherapy, business.industry, Middle Aged, medicine.disease, Peptide Fragments, Surgery, Diabetes Mellitus, Type 1, Peripheral neuropathy, Sensory Thresholds, Anesthesia, Anticonvulsants, Female, Complication, business, Psychomotor Performance
Abstract: In this study we report a randomized double-blind, placebo-controlled trial to evaluate the effect of ORG 2766 in IDDM patients with peripheral neuropathy. Sixty-two patients were selected based on the following criteria: abnormal vibration perception threshold above the 95th-percentile adjusted for age and/or abnormal warm temperature threshold, both measured in the right hand. The patients were randomized into two treatment groups after baseline studies: Group 1 was treated with placebo and Group 2 was treated with 3 mg of the ACTH4-9 analogue ORG 2766 every 24 h. The total study period was 1 year. After 1 year of treatment there was a significant improvement in vibration threshold in Group 1 compared to Group 2. No other parameters improved in the study period. The number of patients selected may have been too small to detect a more important treatment effect. We conclude from this study that ORG 2766 can improve vibration threshold, indicating large myelinated fibre function, but does not affect any of the other neurophysiological function tests.
Published: 1994
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13. Small-fibre neuropathy can be detected in patients with chronic idiopathic axonal polyneuropathy

Author: E L L M, de Schryver, L J, van Schelven, N C, Notermans, H W, de Valk, and P L, Oey
Subjects: Male, Electrocardiography, Polyneuropathies, Sensory Thresholds, Chronic Disease, Humans, Female, Middle Aged, Aged
Abstract: The main sensory presenting symptoms of chronic idiopathic axonal polyneuropathy (CIAP) are paraesthesias, numbness and burning pain in the feet. Although these symptoms indicate the involvement of small nerve fibres, clinical analysis or electrophysiological investigations have not yet been studied in detail.Cardiovascular autonomic tests and cold and heat pain perception threshold tests were performed in 10 patients with CIAP, 10 patients with diabetes mellitus (DM) and 10 healthy volunteers. The results of the DM group were used to see whether the tests were able to detect small-fibre neuropathy in patients with diabetes and pain.Quantitative sensory threshold and autonomic tests showed more frequent abnormal test results in the patients compared to the healthy control group. The proportion of abnormal test results reached significance for the deep breathing tests in both patient groups and for the cold threshold and heat pain test in patients with CIAP. The spectral analysis of RR intervals showed a significant decrease in the high frequency in both patients with DM and CIAP.The results of this study demonstrated that small-fibre neuropathy can be detected in patients with CIAP.
Published: 2010

14. Autonoom zenuwstelselstoornis bij diabetes mellitus. Welke verschijnselen zijn er? Is het eenvoudig aan te tonen?

Author: P. L. Oey
Abstract: Patienten met diabetes mellitus en autonome neuropathie hebben een slechte prognose, niet alleen vanwege een verhoogde incidentie van acute hartdood, maar ook vanwege een aanzienlijke cardiovasculaire morbiditeit.
Published: 2010
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15. Hypoxic neuropathy versus diabetic neuropathy An electrophysiological study in rats

Author: W.H. Gispen, G.H. Wieneke, P H Hendriksen, A.C. van Huffelen, and P. L. Oey
Subjects: Blood Glucose, Male, medicine.medical_specialty, Diabetic neuropathy, Neural Conduction, Neuromuscular transmission, Nerve conduction velocity, Diabetes Mellitus, Experimental, Geneeskunde, Hemoglobins, Diabetic Neuropathies, Ischemia, Reference Values, Ganglia, Spinal, Internal medicine, Diabetes mellitus, Animals, Medicine, Hypoxia, Motor Neurons, Afferent Pathways, Electromyography, business.industry, Rats, Inbred Strains, Hypoxia (medical), medicine.disease, Sciatic Nerve, Rats, Endocrinology, Peripheral neuropathy, Neurology, Neurology (clinical), Sciatic nerve, Nervous System Diseases, medicine.symptom, business, Polyneuropathy
Abstract: In the experimental rat model of diabetes a slowing of nerve conduction velocity and a resistance to ischemic conduction failure have been found as an indication of polyneuropathy. The same electrophysiological abnormalities have been demonstrated in a model in which healthy rats are kept under hypoxic conditions (10% O2) for a 10-week period. Two factors are held responsible for the development of diabetic polyneuropathy: metabolic deterioration and hypoxia. However, until now the relative roles of metabolic deterioration and hypoxia in the development of polyneuropathy have not been settled. To test both explanations further with more sophisticated electrophysiological techniques, the H-reflex (motor and sensory NVC) and the stimulated SF-EMG (measures terminal nerve branch and neuromuscular transmission) were measured in 3 groups of 10 rats, a healthy control group, a diabetic group, and a hypoxic group, every 5 weeks, for 6 months. In the control rats an age-related increase in motor and sensory conduction velocity was found, whereas in the diabetic rats as well as in the hypoxic rats a marked decrease in sensory and a slight decrease in motor nerve conduction velocity was observed. The jitter measured in the stimulated SF-EMG was significantly increased in both the diabetic and the hypoxic group. The results of the present study support the possible role of hypoxia, in addition to metabolic factors, in the development of experimental diabetic neuropathy.
Published: 1992
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16. Letters to the editor

Author: P. L. Oey, H. Franssen, R. A. J. A. M. Bernsen, J. H. J. Wokke, M. L. Sales-Luís, M. Galvão, M. Carvalho, G. Sousa, M. M. Alves, R. Serrão, David C. Preston, Eric L. Logigian, David Yarnitsky, Jose L. Ochoa, J. Gert Van, Michael H. Rivner, Thomas R. Swift, Barbara O. Crout, and Karen P. Rhodes
Subjects: Cellular and Molecular Neuroscience, Physiology, business.industry, Physiology (medical), Block (telecommunications), Borrelia Burgdorferi Infection, Medicine, Neurology (clinical), business, Virology
Published: 1991
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17. Small-fiber neuropathy and the 3243A>G mutation in mitochondrial DNA

Author: John H. J. Wokke, F. Henning, P. L. Oey, and W. G. Oerlemans
Subjects: Mitochondrial DNA, Pathology, medicine.medical_specialty, Ataxia, Mitochondrial disease, Clinical Neurology, Biology, medicine.disease, MELAS syndrome, Letter to the Editors, Muscle atrophy, Geneeskunde, medicine.anatomical_structure, Peripheral neuropathy, Neurology, Lactic acidosis, medicine, Neurology (clinical), medicine.symptom, Sensory nerve
Abstract: Sirs: The 3243A>G point mutation in mitochondrial DNA (mtDNA) is associated with the MELAS (mitochondrial encephalopathy with lactic acidosis and stroke-like episodes) syndrome, and sometimes with additional phenotypes like a sensorimotor polyneuropathy [4, 6]. The occurrence of a small-fiber peripheral neuropathy as the presenting clinical manifestation of a 3243A>G point mutation in mtDNA has, according to our knowledge, not been described previously. A 35 year old man was referred to our neuromuscular clinic with a history of a tingling sensation in the toes of both feet, which has been present for two to three years, as well as a restless feeling in the legs for many years. One year before presentation he developed burning pain in the toes and then fingers bilaterally. He reported no weakness and had no complaints suggestive of an autonomic disturbance. There was no history of alcohol or drug abuse. He was otherwise healthy and used no medication. Previously, his mother was diagnosed with the 3243A>G point mutation in mitochondrial DNA at the age of 51 years after presenting with ptosis, external ophthalmoplegia, limbgirdle weakness and long-standing diabetes mellitus. She had a mtDNA mutation load of 0.8% in leukocytes and 50% in skeletal muscle. On examination no muscle atrophy or weakness was evident and all tendon reflexes, including the ankle jerks, were normal. All sensory modalities were intact, including vibration sense testing with a Rydel-Seiffer tuning fork. No ataxia was present and the gait was undisturbed. Extensive investigations including vitamins B1, B6, B12, folic acid, serum lactate, creatine kinase, thyroid function tests, fasting serum glucose and HbA1c, serum lipid profile, angiotensin converting enzyme levels and serum protein electrophoresis were normal. Anti-nuclear, anti-SS-A and SS-B, antigliadin, anti-endomysial and antineutrophil cytoplasmic (cytoplasmic and perinuclear) antibodies as well as cryoglobulins were all negative. Cranial magnetic resonance imaging and electrocardiography were normal. Leukocyte DNA examination revealed the 3243A>G mtDNA point mutation. The percentage of mutated mtDNA in leukocytes was 16%. Motor and sensory nerve conduction studies and F-responses were performed in the upper and lower limbs and were all normal, as was the Hoffman reflex of the soleus muscle. Concentric needle EMG of distal muscles in the upper and lower limbs was normal. Quantitative sensory threshold testing (CASE IV) of the left foot was performed according to the manufacturer’s protocols. Cold thermal threshold and heat pain was evaluated and the results were compared with normative data. For both cold thermal threshold and heat pain the values obtained were above the 99th percentile, indicating hypoesthesia. In addition, cardiovagal autonomic tests assessing sympathetic and parasympathetic responses were normal. After 18 months of follow-up, no progression of symptoms or involvement of large sensory or motor fibers is evident. We suggest a direct causal relationship between the documented mutation in mtDNA and the small-fiber neuropathy in this patient, as extensive investigations did not reveal another explanation. The exact prevalence of neuropathies in patients with MELAS and the 3243A>G mutation in mtDNA is unknown, but estimates range from 5 to 97% [1, 4, 5]. Small-fiber involvement, isolated or in combination with large-fiber involvement, is probably underrecognised. Most studies included only patients with abnormalities on nerve conduction studies, thereby effectively excluding patients with isolated small-fiber neuropathies. In patients presenting with sensorimotor neuropathies, small-fiber involvement may be overshadowed by large-fiber involvement and thus go undiagnosed. Furthermore, these studies provide no information regarding the prevalence of neuropathies in mutation carriers without the MELAS syndrome. Very little is known about the possible mechanism of peripheral nerve involvement in mitochondrial cytopathies. Mitochondrial failure may just be one of a number of upstream events ultimately leading to axonal degeneration via the activation of calpain, a final pathway probably common to many neuropathies [2]. Furthermore, in a study of vinblastinetreated mice mitochondrial dysfunction correlated strongly with and preceded neurite degeneration [3]. In conclusion, we describe a patient with the MELAS 3243A>G point mutation in mtDNA who presented with clinical and electrophysiological findings suggestive of an isolated small-fiber neuropathy. We postulate that mitochondrial cytopathies should be considered in the differential diagnosis of small-fiber neuropathies. Further studies are needed to confirm the relationship between mitochondrial disorders and small-fiber neuropathies.
Published: 2007

18. Effect of gastric distension on cardiovascular parameters: gastrovascular reflex is attenuated in the elderly

Author: N P, van Orshoven, P L, Oey, L J, van Schelven, J M M, Roelofs, P A F, Jansen, and L M A, Akkermans
Subjects: Sympathetic Nervous System, Valsalva Maneuver, Stomach, Hemodynamics, Stomach Diseases, Blood Pressure, Research Papers, Cold Temperature, Electrocardiography, Heart Rate, Regional Blood Flow, Reflex, Pressure, Humans, Vascular Resistance, Cardiac Output, Aged, Compliance
Abstract: Stretching the stomach wall in young healthy subjects causes an increase in muscle sympathetic nerve activity and in blood pressure, the gastrovascular reflex. We compared healthy elderly subjects with healthy young subjects to find out whether the gastrovascular reflex attenuates in normal ageing and we studied whether there was a difference in autonomic function or gastric compliance that could explain this possible attenuation. Muscle sympathetic nerve activity, finger blood pressure and heart rate were continuously recorded during stepwise isobaric gastric distension using a barostat in eight healthy young (6 men and 2 women, 27 ± 3.2 years, mean ±s.e.m.) and eight healthy elderly subjects (7 men and 1 woman, 76 ± 1.5 years). Changes in cardiac output and total peripheral arterial resistance were calculated from the blood pressure signal. The baseline mean arterial pressure and muscle sympathetic nerve activity were higher in the elderly group (both P < 0.05) and muscle sympathetic nerve activity increase during the cold pressor test was lower in the elderly group (P = 0.005). During stepwise gastric distension, the elderly subjects showed an attenuated increase in muscle sympathetic nerve activity compared to the young subjects (P < 0.01). The older group tended to show a higher increase in mean arterial pressure (P = 0.08), heart rate (P = 0.06) and total peripheral arterial resistance (P = 0.09) The cardiac output rose slightly in both groups without significant difference between groups. The fundic compliance did not differ between groups. We conclude that stepwise gastric distension caused an increase in muscle sympathetic nerve activity in both groups, but the increase in the elderly was attenuated.
Published: 2004

19. Sympathetic activity is increased in polycystic kidney disease and is associated with hypertension

Author: Ihht Klein, Gerry Ligtenberg, Hein A. Koomans, Peter J. Blankestijn, P. L. Oey, and University of Groningen
Subjects: Adult, Male, Mean arterial pressure, medicine.medical_specialty, Sympathetic nervous system, Sympathetic Nervous System, INHIBITION, Renal function, BLOOD-PRESSURE, MASS, Kidney, Plasma renin activity, Reference Values, Internal medicine, Heart rate, Polycystic kidney disease, medicine, Humans, Muscle, Skeletal, Letters to the Editor, Polycystic Kidney Diseases, business.industry, ADULTS, General Medicine, medicine.disease, NERVOUS-SYSTEM, HYPERTROPHY, REDUCTION, Endocrinology, medicine.anatomical_structure, Blood pressure, Nephrology, Hypertension, Cardiology, Female, CHRONIC-RENAL-FAILURE, business, RESISTANCE, Kidney disease
Abstract: Hypertension is common in patients with polycystic kidney disease (PKD). This study addresses the hypothesis that sympathetic activity is enhanced in hypertensive PKD patients, not only when renal function is impaired but also when renal function is still normal. Muscle sympathetic nerve activity (MSNA, peroneal nerve), plasma renin activity (PRA), heart rate, and BP were studied in PKD patients with normal and with impaired renal function and in matched controls. In hypertensive patients with normal renal function, MSNA and mean arterial pressure (MAP) were higher than in normotensive patients (23 +/- 5 versus 15 +/- 7 bursts/min; 110 +/- 10 versus 90 +/- 3 mmHg; P
Published: 2001

20. Autonomic function in patients with hereditary motor and sensory neuropathy type I and Lambert-Eaton myasthenic syndrome

Author: S, Kalmijn, P L, Oey, J H, Wokke, and G H, Wieneke
Subjects: Adult, Chromosome Aberrations, Male, Adolescent, Reflex, Abnormal, Chromosome Disorders, Pressoreceptors, Galvanic Skin Response, Middle Aged, Autonomic Nervous System, Reflex, Pupillary, Vasomotor System, Lambert-Eaton Myasthenic Syndrome, Reference Values, Humans, Female, Child, Hereditary Sensory and Motor Neuropathy, Genes, Dominant
Abstract: Noninvasive tests of four autonomic organ systems (vasomotor control, baroreceptor reflexes, sudomotor function and pupillary reflexes) were performed on nine patients with hereditary motor and sensory neuropathy (HMSN) type I and three patients with Lambert-Eaton myasthenic syndrome (LEMS). The results were compared with those of 33 control subjects. Autonomic dysfunction was considered present when at least two of the four organ system tests were abnormal. The three patients with LEMS had abnormal results in two or more different systems, whereas only one of the nine patients with HMSN type I had two abnormal test results. This study demonstrates that autonomic dysfunction is not a common finding in patients with HMSN type I and its presence should alert us to find the cause of this autonomic disorder.
Published: 1999

21. Temporary muscle weakness in the early phase of distraction during femoral lengthening. Clinical and electromyographical observations

Author: P L, Oey, R H, Engelbert, P M, van Roermond, and G H, Wieneke
Subjects: Male, Adolescent, Electromyography, Muscle Fibers, Skeletal, Ilizarov Technique, Leg Length Inequality, H-Reflex, Postoperative Complications, Reaction Time, Humans, Muscle Hypotonia, Female, Femur, Child, Muscle, Skeletal, Follow-Up Studies
Abstract: Limb lengthening by distraction osteogenesis has a high complication rate. Much of the response of muscle and nerve to distraction is still unknown. Thirteen children, mean age 12.6 yr (8.4-17.3) were surgically treated by the Ilizarov procedure for acquired and congenital femoral limb-length discrepancy. All children showed a decrease in muscle strength in the quadriceps, shortly after the operation, followed by an improvement before distraction started. After an elongation in the early phase of distraction (1 to 2 cm), muscle weakness was again observed and the muscle strength gradually increased after ending of distraction. To provide an explanation for this clinical observation, in one patient (limb lengthening of 4.1 cm) muscle strength measurements were extended with investigations of Hoffman (H) reflex of m. vastus medialis and determination of muscle-fiber conduction velocity of m. vastus lateralis by using the invasive method (IMFCV). The examinations were performed every two weeks during 20 weeks and 12 weeks after removing the cast. A severe decrease in muscle strength of the corrected limb was found after 1.2 cm of distraction with a recovery in muscle strength before lengthening was ended. EMG study showed the same tendency. Denervation was observed as evidence by positive sharp waves and reduced IMFCV findings. Evidence for reinnervation before lengthening was ended, was found by an increased range of velocities consisting of a combination of slow potentials and gradual increase of the velocity of reinnervated fibers (increased Fast/Slow ratio). The latencies of M waves and H-M interval from both legs separated as well after 2.25 cm of distraction. At the end of the follow-up period, the H-M interval reached the preoperative value. It is suggested that these neurogenic changes are an effect of axonal dysfunction and the local effect due to intraoperative trauma and stretching might affect nerve blood flow adversely.
Published: 1999

22. Stomach distension increases efferent muscle sympathetic nerve activity and blood pressure in healthy humans

Author: G.H Wieneke, J. M. M. Roelofs, P Rossi, P. L. Oey, L.M.A Akkermans, and G.I Andriesse
Subjects: Adult, Male, Sympathetic Nervous System, Blood Pressure, Pressoreceptors, Distension, Efferent Pathways, Catheterization, Heart Rate, Reference Values, medicine, Humans, business.industry, Gastric distension, Stomach, Cold pressor test, Muscle, Smooth, Microneurography, Abdominal distension, Middle Aged, Barostat, Blood pressure, medicine.anatomical_structure, Neurology, Anesthesia, Vascular resistance, Linear Models, Female, Neurology (clinical), medicine.symptom, business
Abstract: Although the enteric nervous system is usually described as a separate and independent entity, animal studies show that gastric distension causes a reflex increase in arterial pressure and a sympathetically mediated increase in heart rate and peripheral vascular resistance. To assess the influence of gastric distension on sympathetic outflow and blood pressure, we recorded muscle sympathetic nerve activity (MSNA) from the peroneal nerve by microneurography in eight healthy volunteers. The stomach was distended by means of a barostat, using a single staircase protocol by which pressure was increased by 2 mmHg every 3 min. Gastric sensory function was assessed at each distension step by using a visual analog scale (VAS) for sensations of fullness, nausea and pain. For comparison, we also performed a cold pressor test. The MSNA increased on barostat-induced gastric distension with an almost concomitant elevation of blood pressure. The increase in both was proportional to the intragastric pressure and both decreased towards initial values after the end of distension. Heart rate increased inconsistently and only at higher distension pressures that were associated with high VAS scores. The opposite was found for the cold pressor test. The results of this study confirm the existence of a functional relationship between gastrointestinal distension and cardiovascular function. Decrease in this gastrovascular response may play a role in postprandial hypotension in the elderly, since the MSNA responses to simulated microgravity decrease with age.
Published: 1999

23. Differences between hereditary motor and sensory neuropathy type 2 and chronic idiopathic axonal neuropathy. A clinical and electrophysiological study

Author: W. H. J. P. Linssen, P. F. Ippel, Nicolette C. Notermans, J.H.J. Wokke, B.G.M. van Engelen, H. Franssen, G. W. Van Dijk, Y. van der Graaf, P. L. Oey, L. L. Teunissen, and A.A.W.M. Gabreëls-Festen
Subjects: Adult, Male, medicine.medical_specialty, Pathology, Weakness, Neural Conduction, Disability Evaluation, Atrophy, Sex Factors, Tibialis anterior muscle, medicine, Humans, Neurons, Afferent, Family history, Age of Onset, Muscle, Skeletal, Creatine Kinase, Aged, Denervation, Motor Neurons, business.industry, Middle Aged, medicine.disease, Prognosis, Axons, Surgery, Electrophysiology, Female, Neurology (clinical), Age of onset, medicine.symptom, business, Hereditary motor and sensory neuropathy, Hereditary Sensory and Motor Neuropathy, Polyneuropathy
Abstract: To evaluate whether chronic idiopathic axonal polyneuropathy (CIAP) should be considered as hereditary motor and sensory neuropathy type 2 (HMSN type 2), we compared the clinical features of 48 patients with CIAP with those of 47 patients with HMSN type 2. In addition, we studied electrophysiological data in 20 patients with CIAP and in 20 patients with HMSN type 2. We found, in patients with HMSN type 2, that the initial symptoms were predominantly motor and that weakness and handicap were more severe and skeletal deformities more frequent, compared with those of CIAP patients. Electrophysiologically, the tibialis anterior muscle showed more denervation in patients with HMSN type 2, consistent with the predominance of motor symptoms. There was no important effect of age of onset on clinical features in HMSN type 2 patients. We conclude that in an individual patient with a sensory or sensorimotor idiopathic axonal polyneuropathy and no family history of polyneuropathies, the diagnosis HMSN type 2 is unlikely. However, if motor symptoms predominate, the diagnosis of HMSN type 2 should be considered.
Published: 1997

24. Subclinical diabetic polyneuropathy: early detection of involvement of different nerve fibre types

Author: G.H. Wieneke, Bert Bravenboer, A.C. van Huffelen, P. L. Oey, and P H Hendriksen
Subjects: Adult, Male, medicine.medical_specialty, Diabetic neuropathy, Neural Conduction, Electromyography, Reflex, Pupillary, Diabetic Neuropathies, Internal medicine, Diabetes mellitus, medicine, Humans, Thermosensing, Subclinical infection, Aged, Neurologic Examination, medicine.diagnostic_test, business.industry, Peripheral Nervous System Diseases, Extremities, Middle Aged, medicine.disease, Psychiatry and Mental health, medicine.anatomical_structure, Endocrinology, Cardiology, Terminal nerve, Surgery, Neurology (clinical), business, Polyneuropathy, Pupillometry, Demyelinating Diseases, Research Article
Abstract: Nerve conduction studies, tests of autonomic function and terminal nerve branches, and soleus muscle H reflexes were applied to 60 patients with insulin dependent diabetes mellitus who had no clinical symptoms but abnormal vibratory or temperature perception thresholds indicating subclinical neuropathy. In most patients neurophysiological examination yielded a broad spectrum of neural dysfunction. The perception threshold for cold stimuli was sometimes selectively impaired and abnormal pupillometry results were common, suggesting that small fibres are vulnerable in the early stage of diabetic neuropathy. The arms were less frequently and less severely affected than the legs, an effect that may be related to nerve length. The neurophysiological test results did not change in 30 patients followed up for one year.
Published: 1993

25. Evaluation of electrophysiological and clinical tests in an exploratory trial of Org 2766 in motor neuron disease

Author: L.F.G.M. Hesselmans, Y. van der Graaf, W.H. Gispen, Frans G. I. Jennekens, George H. Wieneke, D.M. Groenhout, and P. L. Oey
Subjects: Adult, medicine.medical_specialty, Weakness, Time Factors, Action Potentials, Isometric exercise, Disease, Placebo, Geneeskunde, Placebos, Adrenocorticotropic Hormone, Double-Blind Method, Isometric Contraction, Medicine, Humans, Motor Neuron Disease, Evoked Potentials, Genetics (clinical), Aged, Analysis of Variance, business.industry, Muscles, Motor neuron, Middle Aged, Peptide Fragments, Motor unit, Electrophysiology, medicine.anatomical_structure, Neurology, Anesthesia, Pediatrics, Perinatology and Child Health, Multivariate Analysis, Physical therapy, Anticonvulsants, Neurology (clinical), Analysis of variance, medicine.symptom, business, Follow-Up Studies
Abstract: Twenty four patients with motor neuron disease (MND) participated in a double-blind, placebo-controlled trial with the ACTH 4-9 analog, Org 2766. Patients were examined three times during an 8 week treatment period, using a summated score for several manually and functionally tested muscles (sum score), myometry, jitter, fibre density (FD), macro motor unit potential (MUP), and supramaximal evoked muscle action potentials. No differences were found between Org 2766 and placebo treated patients. In an open 1 yr follow-up study, 5 out of 13 patients treated with Org 2766 died; the others showed continued progression of weakness. The methods used for assessment of muscle function were compared. The highest intertest reliability was obtained in the sum score and myometry. Mean differences that might be detectable were relatively small for the sum score and myometry, and large for FD and MUP. We concluded that clinical function testing and myometry are superior to electromyographic measurements for assessment of changes in MND patients
Published: 1993

26. Is the corrected QT interval a reliable indicator of the severity of diabetic autonomic neuropathy

Author: A.C. van Huffelen, Bert Bravenboer, W.H. Gispen, P. L. Oey, D.W. Erkelens, and P H Hendriksen
Subjects: Male, medicine.medical_specialty, Valsalva Maneuver, Endocrinology, Diabetes and Metabolism, Autonomic Nervous System, Reflex, Pupillary, Constriction, Geneeskunde, Electrocardiography, Diabetic Neuropathies, Heart Rate, Diabetes mellitus, Internal medicine, Internal Medicine, Humans, Medicine, Glycated Hemoglobin, Advanced and Specialized Nursing, Diabetic Autonomic Neuropathy, medicine.diagnostic_test, business.industry, Middle Aged, medicine.disease, Surgery, Diabetes Mellitus, Type 1, Peripheral neuropathy, Reflex, Cardiology, Regression Analysis, Female, business, Complication, Pupillometry
Abstract: Objective— We investigated whether the corrected QT interval correlated with two other tests for diagnosing autonomic dysfunction in 60 type I diabetic patients with proven peripheral neuropathy. The mean age ± SD was 48.3 ± 11.2 yr, the mean duration of diabetes was 24.9 ± 11.4 yr, and the mean HbA1 was 9.3 ± 2.4%. Research Design and Methods— All patients underwent three autonomic function tests: 1) the standard five cardiovascular Ewing tests, each scored 0 (normal), 0.5 (borderline), or 1.0 (abnormal). We used the sum of the abnormal findings for the analysis, the cardiovascular autonomic score; 2) measurement of the corrected QT interval taken from a routine electrocardiogram recording; and 3) static and dynamic pupillometry: measurement of dark adapted pupil diameter as percentage of total iris diameter and of pupil constriction latency using an infrared light reflex technique. Results— No significant correlation was found between age, duration of diabetes, or HbA1 and any of the autonomic function tests, except for one between age and cardiovascular autonomic score (r = 0.3202, P = 0.0126). Corrected QT interval did not correlate with cardiovascular autonomic score, pupil diameter, or constriction latency. A significant inverse correlation was found between cardiovascular autonomic score and pupil diameter (r = −0.4861, P < 0.001) and constriction latency (r = 0.3783, P < 0.001). Pupil diameter and constriction latency correlated well (r = −0.4276, P < 0.001). Conclusions— The corrected QT interval did not correlate with cardiovascular autonomic tests nor pupillometry results. The corrected QT interval therefore should not be used for the diagnosis of the severity of diabetic autonomic neuropathy.
Published: 1993

27. Muscle conduction velocity and morphology after prolonged hypoxemia and diabetes in rats

Author: P H, Hendriksen, P L, Oey, B K, van Veen, W, Wallinga-de Jonge, and H, Veldman
Subjects: Male, Electromyography, Muscles, Neural Conduction, Action Potentials, Animals, Rats, Wistar, Hypoxia, Diabetes Mellitus, Experimental, Rats
Abstract: One of the electrophysiological abnormalities in the experimental rat model of chronic hypoxia (10% O2) and in the experimental rat model of diabetes is an increase in jitter in the stimulated single fibre EMG, which is thought to result from a primary disorder of the axon with its terminal branches. But muscle fibre alterations that influence the propagation of muscle action potentials can also increase jitter. The contribution of possible changes in muscle conduction velocity and muscle morphology to jitter were investigated in the present study. Muscle conduction velocities were determined and compared with the morphological properties of muscle fibers in muscles of control, chronic hypoxic and terminal-stage diabetic rats. The mean muscle conduction velocities were in the same range in the three groups. The muscle fibre type composition and the mean muscle fibre diameters were about the same in the hypoxic and the control rats, whereas the muscles of the diabetic rats showed a higher percentage of intermediate type muscle fibres, which is suggestive of muscle degeneration, and a smaller mean muscle fibre diameter in comparison with muscles of the hypoxic and the control rats. It is concluded that the similarities between the electrophysiological properties of the muscles despite differences in their morphology, indicate that there is primary axonal degeneration in diabetic hypoxic rats.
Published: 1992

28. Subclinical diabetic neuropathy: similarities between electrophysiological results of patients with type 1 (insulin-dependent) and type 2 (non-insulin-dependent) diabetes mellitus

Author: Bert Bravenboer, P. L. Oey, J. D. Banga, P H Hendriksen, and G.H. Wieneke
Subjects: medicine.medical_specialty, Diabetic neuropathy, Endocrinology, Diabetes and Metabolism, Biopsy, Neural Conduction, Sural nerve, Clinical neurophysiology, Autonomic Nervous System, Gastroenterology, Asymptomatic, H-Reflex, Nerve Fibers, Diabetic Neuropathies, Sural Nerve, Internal medicine, Diabetes mellitus, Internal Medicine, Medicine, Humans, Neurons, Afferent, Ulnar Nerve, Subclinical infection, Motor Neurons, business.industry, Middle Aged, medicine.disease, Endocrinology, medicine.anatomical_structure, Diabetes Mellitus, Type 1, Diabetes Mellitus, Type 2, medicine.symptom, Tibial Nerve, business, Polyneuropathy, Sensory nerve
Abstract: Type 1 (insulin-dependent) and Type 2 (non-insulin-dependent) diabetic patients share many clinical and biochemical characteristics. However, sural nerve biopsies from patients with advanced and chronic neuropathy show ultrastructural differences between these two groups. We investigated whether at a subclinical stage of the illness, when Type 1 and Type 2 diabetic patients are clinically uniform and the histopathological nerve alterations are not advanced, comparison between the two diabetes groups might show differences in nerve fibre involvement related to the different pathogeneses of the neuropathies. A total of 88 diabetic patients (52 Type 1 and 36 Type 2), with a subclinical form of polyneuropathy were selected. The clinical neurophysiological examination consisted of motor and sensory nerve conduction studies, Hoffmann (H)-reflex, single fibre electromyography and static as well as dynamic pupillometry. With regard to clinical neurophysiological abnormalities, the severity of the polyneuropathy appeared to be equal in both groups. Despite the absence of clinical symptoms the neurophysiological abnormalities were pronounced and it was impossible to differentiate Type 1 diabetic patients from Type 2 diabetic patients on a clinical neurophysiology basis when correcting for differences in age, height, and duration of illness. In the Type 1 diabetic group as well as in the Type 2 diabetic group the autonomic nerve fibres and nerves in the legs were more frequently affected than the thick myelinated nerves in the arms. These findings do not support the assumption that there is a difference in the manifestation of polyneuropathy between Type 1 and Type 2 diabetic patients.
Published: 1992

29. Neuro-musculo-skeletal manifestations in primary Sjögren's syndrome

Author: A A, Kruize, R J, Hené, P L, Oey, L, Kater, and J W, Bijlsma
Subjects: Sjogren's Syndrome, Muscular Diseases, Humans, Joint Diseases, Nervous System Diseases
Abstract: Primary Sjögren's syndrome is a systemic autoimmune disorder whose main characteristics are dryness of the eyes and mouth, caused by lymphocytic infiltration of the exocrine glands. Patients may also show signs of extraglandular involvement of lung, liver, kidney and vessel walls, as well as of the central and peripheral nervous systems, muscles and joints. This article presents a review of the literature on extraglandular involvement of the peripheral and central nervous systems, muscles and joints. Several data support the hypothesis that vasculitis is the underlying mechanism. The need for an extended inventory of the extraglandular manifestations, preferentially linked to immunoserological and -histological investigations to gain more insight into the aetiology and pathogenesis is stressed. As far as the clinical picture is concerned, myalgia and arthralgia are often reported, but myositis and arthritis are rare. Data about the prevalence of peripheral and central nervous system involvement are conflicting: factors contributing to these differences are discussed. As insight into prognosis and therapy will strongly depend on the diagnostic criteria used, the need for international agreement on these is emphasized.
Published: 1992

30. Reducción de la hiperactividad simpática mediada por enalapril en pacientes con insuficiencia renal crónica

Author: Peter J. Blankestijn, P. L. Oey, F. Boomsma, I. H. H. Klein, G. Ligtenberg, and L.-T. Dijkhorst-Oei
Subjects: Internal Medicine, Cardiology and Cardiovascular Medicine
Published: 2000
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31. Riboflavin-responsive lipid-storage myopathy and glutaric aciduria type II of early adult onset

Author: P. L. Oey, H. A. Veder, Hans R. Scholte, M. de Visser, I. E. M. Luyt-Houwen, M. H. M. Vaandrager-Verduin, Pieter A. Bolhuis, R.B.H. Schutgens, and Other departments
Subjects: medicine.medical_specialty, Adolescent, Diet therapy, Riboflavin, Biology, Asymptomatic, Glutarates, Metabolic Diseases, Muscular Diseases, Prednisone, Internal medicine, Carnitine, medicine, Humans, Myopathy, Child, Glutaric aciduria, Fatty Acids, Infant, Lipid metabolism, Fibroblasts, Lipid Metabolism, Mitochondria, Muscle, Pedigree, Endocrinology, Female, Neurology (clinical), medicine.symptom, medicine.drug
Abstract: A 17-year-old girl with progressive lipid-storage myopathy for 2 years had low muscle carnitine levels. There was no therapeutic response to prednisone and DL-carnitine-HCl. Chemical findings indicated glutaric aciduria type II. Riboflavin therapy and a fat-restricted, carbohydrate-enriched diet resulted in dramatic improvement. Low carnitine concentrations in plasma and muscle were observed in three asymptomatic sisters who had normal urinary excretion patterns. There was impaired mitochondrial beta-oxidation in cultured skin fibroblasts from the index patient and all three siblings.
Published: 1986

32. [Acute intermittent porphyria with clinical symptoms of an atypical polyradiculoneuropathy]

Author: P L, Oey, P, Fleury, E A, Hische, and F M, Zuyderhoudt
Subjects: Diagnosis, Differential, Porphyrias, Adolescent, Acute Disease, Polyradiculoneuropathy, Humans, Female, Aminolevulinic Acid, Quadriplegia, Menstruation
Published: 1985

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32 results on '"P. L. Oey"'

1. P5370The effect of endovascular baroreflex amplification on sympathetic nerve activity in patients with resistant hypertension: a proof-of-mechanism study

2. Intramedullary femoral nailing through the trochanteric fossa versus greater trochanter tip

3. Effect of gastric distension on cardiovascular parameters: gastrovascular reflex is attenuated in the elderly

4. Small-fibre neuropathy can be detected in patients with chronic idiopathic axonal polyneuropathy

5. Cold Stress Provokes Sympathoinhibitory Presyncope in Healthy Subjects and Hemodialysis Patients With Low Cardiac Output

6. Response to intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy with only sensory symptoms

7. Differential effects of renin-angiotensin-aldosterone system inhibition, sympathoinhibition and diuretic therapy on endothelial function and blood pressure in obesity-related hypertension: a double-blind, placebo-controlled cross-over trial

8. Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulins: a double blind, placebo controlled study

9. A new variant of sensory ataxic neuropathy with autosomal dominant inheritance

10. Features of the Guillain-Barré syndrome in mice following intraperitoneal injection of patient serum

11. Fourth meeting of the European Neurological Society 25–29 June 1994 Barcelona, Spain

12. Randomized double-blind placebo-controlled trial to evaluate the effect of the ACTH4–9 analogue ORG 2766 in IDDM patients with neuropathy

13. Small-fibre neuropathy can be detected in patients with chronic idiopathic axonal polyneuropathy

14. Autonoom zenuwstelselstoornis bij diabetes mellitus. Welke verschijnselen zijn er? Is het eenvoudig aan te tonen?

15. Hypoxic neuropathy versus diabetic neuropathy An electrophysiological study in rats

16. Letters to the editor

17. Small-fiber neuropathy and the 3243A>G mutation in mitochondrial DNA

18. Effect of gastric distension on cardiovascular parameters: gastrovascular reflex is attenuated in the elderly

19. Sympathetic activity is increased in polycystic kidney disease and is associated with hypertension

20. Autonomic function in patients with hereditary motor and sensory neuropathy type I and Lambert-Eaton myasthenic syndrome

21. Temporary muscle weakness in the early phase of distraction during femoral lengthening. Clinical and electromyographical observations

22. Stomach distension increases efferent muscle sympathetic nerve activity and blood pressure in healthy humans

23. Differences between hereditary motor and sensory neuropathy type 2 and chronic idiopathic axonal neuropathy. A clinical and electrophysiological study

24. Subclinical diabetic polyneuropathy: early detection of involvement of different nerve fibre types

25. Evaluation of electrophysiological and clinical tests in an exploratory trial of Org 2766 in motor neuron disease

26. Is the corrected QT interval a reliable indicator of the severity of diabetic autonomic neuropathy

27. Muscle conduction velocity and morphology after prolonged hypoxemia and diabetes in rats

28. Subclinical diabetic neuropathy: similarities between electrophysiological results of patients with type 1 (insulin-dependent) and type 2 (non-insulin-dependent) diabetes mellitus

29. Neuro-musculo-skeletal manifestations in primary Sjögren's syndrome

30. Reducción de la hiperactividad simpática mediada por enalapril en pacientes con insuficiencia renal crónica

31. Riboflavin-responsive lipid-storage myopathy and glutaric aciduria type II of early adult onset

32. [Acute intermittent porphyria with clinical symptoms of an atypical polyradiculoneuropathy]

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