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ABSTRACT: Critical illness may affect the autonomic nervous system. Decreased cardiovascular autonomic function measured by heart rate variability (HRV) has been reported in critically ill patients but limited information exists about other autonomic functions. The cold face test (CFT) and skin wrinkle test (SWT) have never been investigated in critically ill patients. Feasibility and safety of the CFT and SWT were investigated in critically ill patients. Exclusion criteria: polyneuropathy, autonomic neuropathy, admission after stroke, spinal cord injury or cardiac arrest. For the CFT, a cold pack was applied to the forehead to measure the maximal increase in RR interval. The simulated SWT was used and wrinkling was assessed on a five-point scale. HRV was investigated using power spectral analysis of continuous 5-min ECG recordings. Twelve critically ill patients were included (mean age 54). No adverse effects for the CFT and SWT were noted. The CFT could be performed in 10 patients and showed an abnormal response in 9. The SWT could be performed in 11 patients; results were abnormal in 6. HRV analysis showed decreased HRV in all patients. CFT and HRV responses were correlated with each other, no correlation was found between SWT and CFT or HRV results. The CFT and SWT are feasible and safe in critically ill patients. Cardiovascular dysfunction may be more prevalent in critical illness than peripheral sympathetic dysfunction. Influence of confounders and further validation of these tests needs to be investigated.
Journal of Neurology 12/2012; · 3.47 Impact Factor
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ABSTRACT: The objective of this prospective study was to investigate the long-term effect of one or more local corticosteroid injections in patients with carpal tunnel syndrome and whether a good response can be predicted. Follow-up visits took place at 3 weeks, 6 months, and 1 year after the first corticosteroid injection. Thirty of the 120 patients (25%) had a good outcome with a single injection, 11 additional patients (9%) needed a second injection, and five patients (4%) needed a third injection to reach a good outcome after 1 year. Of patients with an initial good treatment response, 28 (52%) had a good outcome after 1 year compared with 18 (27 %) who had an initially moderate or no response to treatment. One-third of patients with carpal tunnel syndrome had a long-term beneficial effect from corticosteroid injection, especially when they had a good initial response.
The Journal of hand surgery, European volume. 12/2012;
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ABSTRACT: Achieving long-term remission after a limited more intense treatment period would prevent prolonged use of corticosteroids or IV immunoglobulin (IVIg) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). In this prospective cohort study we present long-term follow-up data on patients included in a multicenter randomized controlled trial comparing 6 monthly pulses of dexamethasone with 8 months of daily prednisolone.
Treatment effect was assessed with the Inflammatory Neuropathy Cause and Treatment disability scale and the Rivermead Mobility Index and was categorized using the CIDP Disease Activity Status (CDAS) scale.
By March 2011, 39 out of 40 patients were included with a median follow-up of 4.5 years. Cure (>5 years off treatment) or remission according to the CDAS criteria after 1 or 2 courses of pulsed dexamethasone or daily prednisolone was achieved in 10 out of 39 patients (26%). Half of the patients who were in remission after initial treatment experienced a relapse (median treatment-free interval: 17.5 months for dexamethasone, 11 months for prednisolone). Alternative diagnosis was made in 7 out of 12 (58%) who did not respond to any therapy and in none of the treatment-responsive patients.
Cure or long-term remission can be achieved in about one-quarter of patients with CIDP after 1 or 2 courses of pulsed dexamethasone or 8-month daily prednisolone. In treatment-nonresponsive patients, the diagnosis CIDP should be reconsidered.
This study provides Class IV evidence that pulsed dexamethasone or 8-month daily prednisolone can lead to long-term remission in CIDP.
Neurology 03/2012; 78(14):1079-84. · 8.31 Impact Factor
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K Kuitwaard,
L H van den Berg,
M Vermeulen,
E Brusse,
E A Cats,
A J van der Kooi,
N C Notermans,
W-L van der Pol, I N van Schaik,
S I van Nes,
W C J Hop,
P A van Doorn
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ABSTRACT: Different preparations of intravenous immunoglobulin (IVIg) are considered to have comparable clinical efficacy but this has never been formally investigated. Some patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) report that some IVIg brands are more effective than others. A liquid IVIg preparation is more user friendly and potentially can be infused at a faster rate.
The primary objective was to compare the efficacy of two different IVIg brands in CIDP. The secondary objective was to compare their safety.
This was an investigator-initiated multi-centre randomised controlled double-blind trial. Twenty-seven patients with active but stable CIDP treated with their individual stable IVIg (Gammagard S/D) maintenance dose and interval were randomised to receive four infusions of freeze-dried 5% IVIg (Gammagard S/D) or the new liquid 10% IVIg (Kiovig). The overall disability sum score (ODSS) was used as the primary outcome scale. The equivalence margin was defined as a difference of ≤1 point in mean ΔODSS between treatment groups. Main secondary outcome scales were the MRC sum score and the Vigorimeter.
Repeated measurements analysis of variance, adjusted for baseline ODSS, showed a clinically insignificant treatment difference of 0.004 (95% CI -0.4 to 0.4). We also found no significant differences in any of the other outcome measures. Besides a lower occurrence of cold shivers in patients randomised to Kiovig (p=0.03), no significant differences were found in the occurrence of adverse events.
This trial demonstrated equal clinical efficacy between a freeze-dried and a liquid IVIg preparation for maintenance treatment of CIDP.
Journal of neurology, neurosurgery, and psychiatry 12/2010; 81(12):1374-9. · 4.87 Impact Factor
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ABSTRACT: We describe monozygotic twin sisters, born to consanguineous Moroccan parents, who are highly discordant for the manifestations of Gaucher disease. Both carry Gaucher genotype N188S/N188S. One has severe visceral involvement, epilepsy, and a cerebellar syndrome. Her twin does not manifest any symptoms or signs of Gaucher disease but suffers from type 1 diabetes mellitus. The concurrence of a mild Gaucher mutation with a severe phenotype, as well as the occurrence of highly discordant phenotypes in a pair of monozygotic twins, is discussed.
Blood Cells Molecules and Diseases 11/2010; 46(1):39-41. · 2.35 Impact Factor
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ABSTRACT: Charcot-Marie-Tooth disease type 1A (CMT1A) is known as a demyelinating hereditary neuropathy. Secondary axonal dysfunction is the most important determinant of disease severity. In adult patients, clinical progression may be because of further axonal deterioration as was shown with compound muscle action potential (CMAP) amplitude reductions over time. The motor unit number estimation (MUNE) technique may be more accurate to determine the number of axons as it is not disturbed by the effect of reinnervation. The purpose of this study was to investigate the number and size of motor units in relation to age in patients and controls.
In a cross-sectional design, we assessed arm and hand strength and performed electrophysiological examinations, including CMAP amplitudes and MUNE of the thenar muscles using high-density surface EMG in 69 adult patients with CMT1A and 55 age-matched healthy controls.
In patients, lower CMAP amplitudes and MUNE values were related to hand weakness. The CMAP amplitude and MUNE value of the thenar muscles were significantly lower in patients than in controls. CMAP amplitudes declined with age in controls, but not in patients. MUNE values declined with age in both patients and controls.
The age-dependent decrease in the number of motor units was not significantly different between patients with CMT1A and controls, indicating that loss of motor units in adult patients is limited.
European Journal of Neurology 04/2010; 17(8):1098-104. · 3.69 Impact Factor
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P Y K Van den Bergh,
R D M Hadden,
P Bouche,
D R Cornblath,
A Hahn,
I Illa,
C L Koski,
J-M Léger,
E Nobile-Orazio,
J Pollard,
C Sommer,
P A van Doorn, I N van Schaik
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ABSTRACT: Consensus guidelines on the definition, investigation, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been previously published in European Journal of Neurology and Journal of the Peripheral Nervous System.
To revise these guidelines.
Disease experts, including a representative of patients, considered references retrieved from MEDLINE and Cochrane Systematic Reviews published between August 2004 and July 2009 and prepared statements that were agreed in an iterative fashion.
The Task Force agreed on Good Practice Points to define clinical and electrophysiological diagnostic criteria for CIDP with or without concomitant diseases and investigations to be considered. The principal treatment recommendations were: (i) intravenous immunoglobulin (IVIg) (Recommendation Level A) or corticosteroids (Recommendation Level C) should be considered in sensory and motor CIDP; (ii) IVIg should be considered as the initial treatment in pure motor CIDP (Good Practice Point); (iii) if IVIg and corticosteroids are ineffective, plasma exchange (PE) should be considered (Recommendation Level A); (iv) if the response is inadequate or the maintenance doses of the initial treatment are high, combination treatments or adding an immunosuppressant or immunomodulatory drug should be considered (Good Practice Point); (v) symptomatic treatment and multidisciplinary management should be considered (Good Practice Point).
European Journal of Neurology 03/2010; 17(3):356-63. · 3.69 Impact Factor
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ABSTRACT: In patients who remain in a coma after cardiopulmonary resuscitation (CPR), the bilateral absence of cortical N20 responses of median nerve somatosensory evoked potentials (SSEP) 24 hours after admission invariably correlates with a poor neurologic outcome. Nowadays, CPR patients are treated with mild hypothermia, with simultaneously administered sedative drugs, hampering clinical neurologic assessment. We investigated whether SSEP performed during hypothermia can reliably predict a poor neurologic outcome.
Between July 2006 and April 2008, this multicenter prospective cohort study included adult comatose patients admitted after CPR and treated with induced mild hypothermia (32-34 degrees C). SSEP was performed during hypothermia, and in patients who remained comatose after rewarming, a second SSEP was performed. Neurologic outcome was assessed 30 days after admission with the Glasgow Outcome Scale.
Seventy-seven consecutive patients were included in 2 hospitals. In 13 patients (17%), the cortical N20 response during hypothermia was bilaterally absent. In 9 of these 13 patients in whom SSEP could be repeated during normothermia, the N20 response was also absent, yielding a positive predictive value of 1.00 (95% confidence interval [CI] 0.70-1.00). All 13 patients with absent SSEP during hypothermia had a poor neurologic outcome, yielding a positive predictive value of 1.00 (95% CI 0.77-1.00).
The results of this pilot study show that bilaterally absent cortical N20 responses of median nerve somatosensory evoked potentials performed during mild hypothermia after resuscitation can predict a poor neurologic outcome. We started a larger multicenter prospective cohort study to confirm these results.
Neurology 11/2009; 73(18):1457-61. · 8.31 Impact Factor
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ABSTRACT: Previous studies suggest that in patients with ischaemic stroke, White people often present with large-vessel and Black people with small-vessel strokes. This study investigates the relation between large- and small-vessel disease, and ethnicity in White, Black, and Asian patients in Amsterdam, The Netherlands.
In a hospital-based population of 668 patients ethnicity was determined by self-identification. The relation between ethnicity and carotid stenosis, as an indicator of large-vessel disease, was determined using univariate analysis, and adjusted for age, gender, hypertension and smoking. Subsequently the relation between ethnicity and lacunar infarcts, as a manifestation of small-vessel disease, was investigated.
The odds ratio for having carotid stenosis, compared to White patients, was 0.55 (0.23-1.33) for Blacks, 0.53 (0.18-1.52) for Asians, and 0.64 (0.14-2.85) for other ethnicities. The adjusted odds ratio for a non-White patient compared to a White patient was 0.44 (0.19-1.02) (P = 0.05). The non-White patients more often presented with lacunar infarcts compared to Whites.
We found an association between White patients and the presence of carotid artery stenosis. Not only in Black but also in Asian patients the association with carotid artery stenosis was substantially lower. In the non-White population there was an association with lacunar infarcts compared to Whites.
European Journal of Neurology 03/2009; 16(4):522-7. · 3.69 Impact Factor
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ABSTRACT: Charcot Marie Tooth type 1a (CMT1a) is a primarily demyelinating neuropathy, characterized by slowly progressive muscle weakness, atrophy, and sensory loss, and is most pronounced in both feet and hands. There is increasing evidence that muscle weakness is determined by motor axonal dysfunction.
To investigate in patients with CMT1a whether motor axon loss, as estimated with motor unit number estimation (MUNE) and compound muscle action potential (CMAP), is related to hand function and manual dexterity.
Hand function, manual dexterity, and axon loss were studied in 48 patients with proven CMT1a. Using high-density surface EMG on the thenar muscles, MUNE was determined and CMAPs were measured.
Pinch strength, clawing of the fingers, and manual dexterity correlated significantly with MUNE and CMAP (amplitude and area), while sensory impairments did not. Grip strength correlated significantly with CMAP amplitude but did not become significant with MUNE and CMAP area. Neurophysiologic variables were particularly associated with fine motor function of the hand.
Motor axon loss is likely to be the major cause of hand dysfunction and impaired manual dexterity in Charcot Marie Tooth type 1a (CMT1a). In a clinical setting, the evaluation of the hands of patients with CMT1a should thus be mainly directed toward the evaluation of fine motor functions.
Neurology 11/2008; 71(16):1254-60. · 8.31 Impact Factor
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ABSTRACT: Despite high-dose intravenous immunoglobulin (IVIG) is widely used in treatment of a number of immune-mediated neurological diseases, the consensus on its optimal use is insufficient. To define the evidence-based optimal use of IVIG in neurology, the recent papers of high relevance were reviewed and consensus recommendations are given according to EFNS guidance regulations. The efficacy of IVIG has been proven in Guillain-Barré syndrome (level A), chronic inflammatory demyelinating polyradiculoneuropathy (level A), multifocal mononeuropathy (level A), acute exacerbations of myasthenia gravis (MG) and short-term treatment of severe MG (level A recommendation), and some paraneoplastic neuropathies (level B). IVIG is recommended as a second-line treatment in combination with prednisone in dermatomyositis (level B) and treatment option in polymyositis (level C). IVIG should be considered as a second or third-line therapy in relapsing-remitting multiple sclerosis, if conventional immunomodulatory therapies are not tolerated (level B), and in relapses during pregnancy or post-partum period (good clinical practice point). IVIG seems to have a favourable effect also in paraneoplastic neurological diseases (good practice point) [corrected],stiff-person syndrome (level A), some acute-demyelinating diseases and childhood refractory epilepsy (good practice point).
European Journal of Neurology 10/2008; 15(9):893-908. · 3.69 Impact Factor
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ABSTRACT: Gaucher disease is a lysosomal storage disorder, which is classically divided into three types. Type I Gaucher disease is differentiated from types II and III disease by the absence of nervous system involvement. However, an increasing number of reports has emerged on neurological manifestations in patients with type I Gaucher disease. Whether a strict division in three different phenotypes is still valid has been the subject of debate. The main objective of this study was to provide scientific arguments whether a distinction between type I (non-neuronopathic) and types II and III (neuronopathic) Gaucher disease should be maintained. We investigated retrospectively a large Dutch cohort of type I Gaucher disease patients for the prevalence of neurological manifestations and provide an overview of the literature on this topic. A diagnosis of a neurological disease was made 34 times in 75 patients. Forty-five patients reported at least one neurological symptom during the median follow-up time of 11 years. The literature search revealed 86 studies in which type I Gaucher disease patients or carriers of a glucocerebrosidase mutation were described with a neurological disease or a condition which is known to be associated with neurological disease. In conclusion, the term non-neuronopathic Gaucher disease does not seem to be an appropriate characterization of type I Gaucher disease. However, the neurological signs and symptoms in type I Gaucher disease are of a totally different kind from and, in the majority of cases, of much less severity than the signs and symptoms associated with types II and III disease Therefore, type I disease should be classified as a separate phenotype.
Journal of Inherited Metabolic Disease 07/2008; 31(3):337-49. · 3.58 Impact Factor
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ABSTRACT: chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated disease. Current treatments are aimed at modulating the immune response to achieve remission and maintain functional status. However, many patients fail to make a long-term recovery with current treatments.
to review the literature on immunotherapy for CIDP.
We used the Search Strategy of the Cochrane Neuromuscular Disease Review Group to search Medline and Embase. Randomised and non-randomised studies examining the effects of any therapeutic agent in patients with CIDP were selected. The references of relevant articles were scanned to identify additional reports of interest.
An overview of the different treatments for CIDP is provided. Emphasis has been placed on evidence from randomised controlled trials but open non-randomised studies are discussed if appropriate. We include a treatment algorithm and provide our views on current treatments, ongoing trials and possible directions for further research.
Expert opinion on biological therapy 06/2008; 8(5):643-55. · 3.22 Impact Factor
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ABSTRACT: Three case studies illustrate that suspected anoxic-ischaemic coma often requires careful differential diagnosis to detect treatable conditions. A 47-year-old man underwent cardiopulmonary resuscitation for ventricular fibrillation caused by myocardial ischaemia. He exhibited rhythmic eyelid movements while in a coma. Epilepsy was suspected, and the patient regained consciousness after being treated with antiepileptic drugs. A 34-year-old man underwent cardiopulmonary resuscitation for multiple episodes of ventricular fibrillation. Treatment was directed toward myocardial ischaemia and included anticoagulants. The patient had bilateral, fixed dilated pupils. A CT of the brain showed traumatic contrecoup haemorrhage in the left temporal lobe with signs of transtentorial herniation. The patient died. A 74-year-old woman was found unconscious at home. An ECG performed by the paramedics showed ST segment elevation in the precordial leads. Anoxic-ischaemic coma following cardiac arrest was suspected. However, a coronary angiogram was normal and a CT of the brain revealed subarachnoid haemorrhage caused by a ruptured intracranial aneurysm. She recovered after cranial surgery.
Nederlands tijdschrift voor geneeskunde 03/2008; 152(6):297-301.
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Neuromuscular Disorders 02/2008; 18(1):85-9. · 2.80 Impact Factor
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R. D. M. Hadden,
E. Nobile-Orazio,
C. Sommer,
A. Hahn,
I. Illa,
E. Morra,
J. Pollard,
R. Hughes,
P. Bouche,
D. Cornblath,
E. Evers,
C. L. Koski,
J. M. Léger,
P. Van den Bergh,
P. van Doorn, I. N. van Schaik
01/2008: pages 362 - 375; , ISBN: 9780470753279
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M P J Garssen,
R van Koningsveld,
P A van Doorn,
I S J Merkies,
M Scheltens-de Boer,
J A van Leusden, I N van Schaik,
W H J P Linssen,
F Visscher,
A M Boon,
C G Faber,
J Meulstee,
M J J Prick,
L H van den Berg,
H Franssen,
J A P Hiel,
P Y K van den Bergh,
C J M Sindic
Journal of neurology, neurosurgery, and psychiatry 10/2007; 78(9):1012-3. · 4.87 Impact Factor
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ABSTRACT: Pregabalin is increasingly being used for the treatment ofneuropathic pain, often as the first-line choice. The question is, however, whether this choice is based on evidence. Seven trials have been published on the effect ofpregabalin in patients with postherpetic neuralgia and painful diabetic neuropathy. These trials more frequently report a 50% reduction in pain in pregabalin treated patients than in patients treated with placebo (number needed to treat 4.3). Dizziness and somnolence are the most frequent adverse events of pregabalin. The number needed to harm for adverse events leading to discontinuation of treatment varies from 3.7 to 113.1 in these studies. Pregabalin has not been compared head-to-head with other drugs commonly used for neuropathic pain. Indirect comparison reveals the effectiveness of pregabalin is comparable with that of carbamazepin, tramadol, and gabapentin; pregabalin is possibly less effective than amitriptylin. However, taking into account its price and the lack of clinical experience and evidence, using pregabalin as first-line choice is not recommended.
Nederlands tijdschrift voor geneeskunde 08/2007; 151(28):1561-5.
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ABSTRACT: Each of the various neuromuscular diseases is rare. Consequently, solid epidemiological data are not available and it is often difficult to find sufficient patients for studies. For this reason, the Dutch neuromuscular database, CRAMP (Computer Registry of All Myopathies and Polyneuropathies), was developed in 2004 by the Dutch Neuromuscular Research Support Centre, to store information on patient characteristics and diagnoses (based on Rowland and McLeod's classification) in a uniform and easily retrievable manner. Care was taken to preserve data confidentiality. It is envisaged that CRAMP will prove particularly useful for studies in which multicentre collaboration is needed to recruit a sufficiently large number of patients. More than 10,000 patients with neuromuscular diseases (4,837 female, 5,476 male) have been registered since 2004, half of whom (n=5059) have peripheral nerve disorders.
Neuromuscular Disorders 02/2007; 17(1):33-7. · 2.80 Impact Factor
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R D M Hadden,
E Nobile-Orazio,
C Sommer,
A Hahn,
I Illa,
E Morra,
J Pollard,
R A C Hughes,
P Bouche,
D Cornblath,
E Evers,
C L Koski,
J M Léger,
P Van den Bergh,
P van Doorn, I N van Schaik
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ABSTRACT: Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological and haematological features. Objectives. To prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein (paraproteinaemic demyelinating neuropathy, PDN).
Search of MEDLINE and the Cochrane library, review of evidence and consensus agreement of an expert panel.
In the absence of adequate data, evidence based recommendations were not possible but the panel agreed the following good practice points: (1) Patients with PDN should be investigated for a malignant plasma cell dyscrasia. (2) The paraprotein is more likely to be causing the neuropathy if the paraprotein is immunoglobulin (Ig)M, antibodies are present in serum or on biopsy, or the clinical phenotype is chronic distal sensory neuropathy. (3) Patients with IgM PDN usually have predominantly distal and sensory impairment, with prolonged distal motor latencies, and often anti-myelin associated glycoprotein antibodies. (4) IgM PDN sometimes responds to immune therapies. Their potential benefit should be balanced against their possible side-effects and the usually slow disease progression. (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy, clinically, electrophysiologically, and in response to treatment. (6) For POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with haemato-oncology advice.
European Journal of Neurology 09/2006; 13(8):809-18. · 3.69 Impact Factor
