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1.
BMC Neurol ; 22(1): 245, 2022 Jul 05.
Article in English | MEDLINE | ID: mdl-35790912

ABSTRACT

BACKGROUND: Patient involvement in discharge planning of patients with stroke can be accomplished by providing personalized outcome information and promoting shared decision-making. The aim of this study was to develop a patient decision aid (PtDA) for discharge planning of hospitalized patients with stroke. METHODS: A convergent mixed methods design was used, starting with needs assessments among patients with stroke and health care professionals (HCPs). Results of these assessments were used to develop the PtDA with integrated outcome information in several co-creation sessions. Subsequently, acceptability and usability were tested to optimize the PtDA. Development was guided by the International Patient Decision Aids Standards (IPDAS) criteria. RESULTS: In total, 74 patients and 111 HCPs participated in this study. A three-component PtDA was developed, consisting of: 1) a printed consultation sheet to introduce the options for discharge destinations, containing information that can be specified for each individual patient; 2) an online information and deliberation tool to support patient education and clarification of patient values, containing an integrated "patients-like-me" model with outcome information about discharge destinations; 3) a summary sheet to support actual decision-making during consultation, containing the patient's values and preferences concerning discharge planning. In the acceptability test, all qualifying and certifying IPDAS criteria were fulfilled. The usability test showed that patients and HCPs highly appreciated the PtDA with integrated outcome information. CONCLUSIONS: The developed PtDA was found acceptable and usable by patients and HCPs and is currently under investigation in a clinical trial to determine its effectiveness.


Subject(s)
Patient Discharge , Stroke , Decision Support Techniques , Health Personnel , Humans , Patients , Stroke/therapy
2.
Qual Life Res ; 31(12): 3317-3330, 2022 Dec.
Article in English | MEDLINE | ID: mdl-35622294

ABSTRACT

PURPOSE: This study aims to systematically review and critically appraise the content validity of the adult versions of the Patient-Reported Outcomes Measurement Information System Physical Function (PROMIS-PF) item bank and its derivative measures in any adult population. METHODS: MEDLINE and EMBASE were searched in October 2021 for studies on measurement properties of PROMIS-PF measures in an adult population. Studies were included if the study described the development of a PROMIS-PF measure or investigated its relevance, comprehensiveness, or comprehensibility. Assessment of the methodological quality of eligible studies, rating of results, and summarizing evidence was performed following the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) methodology for assessing content validity. A modified GRADE approach was used to determine the level of evidence. RESULTS: Three development studies and eight studies on the content validity of one or more of the PROMIS-PF measures were identified. The methodological quality of most studies was rated doubtful. There was low to high level evidence for sufficient relevance, comprehensiveness, and comprehensibility of most PROMIS-PF measures for healthy seniors and various disease populations. We found low to moderate level evidence for insufficient relevance of PROMIS-PF measures for patients with conditions that affected only one body part, and insufficient comprehensibility of the PROMIS-PF measures for minority elderly. CONCLUSION: Most PROMIS-PF measures demonstrate sufficient content validity in healthy seniors and various disease populations. However, the quality of this evidence is generally low to moderate, due to limitations in the methodological quality of the studies.


Subject(s)
Patient Reported Outcome Measures , Quality of Life , Adult , Humans , Aged , Quality of Life/psychology , Consensus , Health Status , Information Systems
3.
Arch Phys Med Rehabil ; 103(8): 1582-1591, 2022 08.
Article in English | MEDLINE | ID: mdl-34998711

ABSTRACT

OBJECTIVE: The present study aimed to create a shorter version of the Action Research Arm Test (ARAT) without compromising its measurement properties. DESIGN: Secondary analysis of stroke recovery cohorts that used the ARAT to measure upper limb impairment. SETTING: Rehabilitation centers. PARTICIPANTS: Patients with stroke from 5 different stroke recovery cohorts (N=1425). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: A decision tree version of the ARAT (ARAT-DT) was developed using chi-square automated interaction detection. In an independent validation subset, criterion validity, agreement of ARAT-DT with original ARAT scores and score categories, and construct validity with the Fugl-Meyer Upper Extremity Scale score were determined. RESULTS: In total, 3738 ARAT measurements were available involving 1425 subjects. Chi-square automated interaction detection analysis in the development subset (n=2803) revealed an optimized decision tree with a maximum of 4 consecutive items. In the validation data set (n=935), the ARAT-DT differed by a mean of 0.19 points (0.3% of the total scale) from the original ARAT scores (limits of agreement=-5.67 to 6.05). The ARAT-DT demonstrated excellent criterion validity with the original ARAT scores (intraclass correlation coefficient=0.99 and ρ=0.99) and scoring categories (κw=0.97). The ARAT-DT showed very good construct validity with the Fugl-Meyer Upper Extremity Scale (ρ=0.92). CONCLUSION: A decision tree version of the ARAT was developed, reducing the maximum number of items necessary for ARAT administration from 19 to 4. The scores produced by the decision tree had excellent criterion validity with original ARAT scores.


Subject(s)
Stroke Rehabilitation , Stroke , Decision Trees , Disability Evaluation , Health Services Research , Humans , Recovery of Function , Stroke/complications , Upper Extremity
4.
BMC Health Serv Res ; 21(1): 1350, 2021 Dec 18.
Article in English | MEDLINE | ID: mdl-34922534

ABSTRACT

INTRODUCTION: Approximately two-thirds of the patients admitted to the hospital with an ischemic stroke are discharged directly home. Discontinuity of care may result in avoidable patient harm, re-admissions and even death. We hypothesized that the transfer of information is most essential in this patient group since any future care for these patients relies solely on the information that is available to the care provider responsible at that time. AIM: The objective of this study was to evaluate the continuity of transmural care in ischemic stroke patients by assessing 1) the transfer of clinical information through discharge letters to general practitioners (GPs), 2) subsequent documentation of this information and early follow-up by GPs and 3) the documentation of medication-related information in discharge letters, at GPs and community pharmacies (CPs). METHODS: This prospective cohort study was conducted from September 2019 through March 2020 in OLVG, Amsterdam, the Netherlands, in patients with a first stroke discharged directly home. Outcome measures were derived from national guidelines and regional agreements. Results were analyzed using descriptive analysis. RESULTS: A total of 33 patients were included. Discharge letters (n = 33) and outpatient clinic letters (n = 24) to GPs contained most of the essential items, but 16% (n = 9) of the letters were sent in time. GPs (n = 31) infrequently adhered to guidelines since 10% (n = 3) of the diagnoses were registered using the correct code and 55% (n = 17) of the patients received follow-up shortly after discharge. Medication overviews were inaccurately communicated to GPs since 62% (n = 150) of all prescriptions (n = 243) were correctly noted in the discharge letter. Further loss of information was seen as only 39% (n = 95) of all prescriptions were documented correctly in GP overviews. We found that 59% (n = 144) of the prescriptions were documented correctly in CP overviews. CONCLUSION: In this study, we found that discontinuity of care occurred to a varying extent throughout transmural care in patients with a first stroke who were discharged home.


Subject(s)
Patient Discharge , Stroke , Humans , Netherlands , Patient Transfer , Prospective Studies , Stroke/therapy
5.
BMC Health Serv Res ; 20(1): 1049, 2020 Nov 17.
Article in English | MEDLINE | ID: mdl-33203405

ABSTRACT

BACKGROUND: Ischemic stroke patients with a good outcome in terms of motor functioning and communication are likely to be discharged home without further rehabilitation. A significant number of these patients experience cognitive and emotional problems resulting in lower quality of life and decreased participation in society. This paper presents the protocol of a study examining the clinical effectiveness, cost-effectiveness and implementation of an intervention focused on screening and patient-tailored care for cognitive and emotional problems as compared to usual care in patients discharged home after ischemic stroke. METHODS / DESIGN: A multicenter, patient-blinded, cluster randomized controlled trial will be performed. Centers will be randomized (1:1) to the intervention group or the usual care group. Patients (> 18 years old) with a neurological confirmed diagnosis of ischemic stroke who can be discharged home without follow-up treatment at an outpatient rehabilitation clinic will be included. In the intervention group, patients will receive a short, individualized, semi-structured consultation by specialized nurses in addition to usual care. This consultation includes 1) screening for cognitive and emotional problems, 2) screening for restrictions in participation, 3) promotion of self-management strategies and 4) a decision tool for referral to rehabilitation services. The intervention will be performed approximately 6 weeks after the stroke at the neurology outpatient clinics and will take approximately 60 min. The control group will receive care as usual. Both groups will be followed-up at 6 weeks, 3 months and 12 months after stroke. The primary outcome will be the level of participation measured with the Restriction subscale of the Utrecht Scale for Evaluation of Rehabilitation on the level of Participation (USER-Participation-R) at 12 months. A cost-effectiveness analysis and process evaluation will be performed alongside. DISCUSSION: This trial is the first to evaluate clinical effectiveness, cost-effectiveness and implementation of screening and patient-tailored care for cognitive and emotional problems compared to care as usual in patients discharged home after ischemic stroke. Potentially, this will improve the outcomes for patients with frequently occurring cognitive and emotional problems after stroke. TRIAL REGISTRATION: Netherlands Trial Register: NL7295 , registered 25 September 2018.


Subject(s)
Brain Ischemia , Ischemic Stroke , Stroke Rehabilitation , Stroke , Adolescent , Cognition , Cost-Benefit Analysis , Humans , Multicenter Studies as Topic , Netherlands , Patient Discharge , Quality of Life , Randomized Controlled Trials as Topic , Stroke/therapy
6.
Patient Educ Couns ; 105(5): 1123-1129, 2022 05.
Article in English | MEDLINE | ID: mdl-34462248

ABSTRACT

OBJECTIVE: The aim of this study was to gain insight into experiences of patients with acute stroke regarding information provision and their preferred involvement in decision-making processes during the initial period of hospitalisation. METHODS: A sequential explanatory design was used in two independent cohorts of patients with stroke, starting with a survey after discharge from hospital (cohort 1) followed by observations and structured interviews during hospitalisation (cohort 2). Quantitative data were analysed descriptively. RESULTS: In total, 72 patients participated in this study (52 in cohort 1 and 20 in cohort 2). During hospitalisation, the majority of the patients were educated about acute stroke and their treatment. Approximately half of the patients preferred to have an active role in the decision-making process, whereas only 21% reported to be actively involved. In cohort 2, 60% of the patients considered themselves capable to carefully consider treatment options. CONCLUSIONS: Active involvement in the acute decision-making process is preferred by approximately half of the patients with acute stroke and most of them consider themselves capable of doing so. However, they experience a limited degree of actual involvement. PRACTICE IMPLICATIONS: Physicians can facilitate patient engagement by explicitly emphasising when a decision has to be made in which the patient's opinion is important.


Subject(s)
Decision Making , Stroke , Hospitalization , Humans , Patient Participation , Stroke/therapy , Surveys and Questionnaires
7.
J Neurol ; 268(2): 403-408, 2021 Feb.
Article in English | MEDLINE | ID: mdl-32691235

ABSTRACT

BACKGROUND AND PURPOSE: There are concerns that the coronavirus disease 2019 (COVID-19) outbreak negatively affects the quality of care for acute cardiovascular conditions. We assessed the impact of the COVID-19 outbreak on trends in hospital admissions and workflow parameters of acute stroke care in Amsterdam, The Netherlands. METHODS: We used data from the three hospitals that provide acute stroke care for the Amsterdam region. We compared two 7-week periods: one during the peak of the COVID-19 outbreak (March 16th-May 3th 2020) and one prior to the outbreak (October 21st-December 8th 2019). We included consecutive patients who presented to the emergency departments with a suspected stroke and assessed the change in number of patients as an incidence-rate ratio (IRR) using a Poisson regression analysis. Other outcomes were the IRR for stroke subtypes, change in use of reperfusion therapy, treatment times, and in-hospital complications. RESULTS: During the COVID-19 period, 309 patients presented with a suspected stroke compared to 407 patients in the pre-COVID-19 period (IRR 0.76 95%CI 0.65-0.88). The proportion of men was higher during the COVID-19 period (59% vs. 47%, p < 0.001). There was no change in the proportion of stroke patients treated with intravenous thrombolysis (28% vs. 30%, p = 0.58) or endovascular thrombectomy (11% vs 12%, p = 0.82) or associated treatment times. Seven patients (all ischemic strokes) were diagnosed with COVID-19. CONCLUSION: We observed a 24% decrease in suspected stroke presentations during the COVID-19 outbreak, but no evidence for a decrease in quality of acute stroke care.


Subject(s)
COVID-19 , Pandemics , Stroke/therapy , Aged , Aged, 80 and over , COVID-19/epidemiology , Emergency Medical Services , Female , Hospitalization , Humans , Incidence , Ischemic Stroke/complications , Ischemic Stroke/epidemiology , Male , Middle Aged , Netherlands/epidemiology , Poisson Distribution , Quality of Health Care , Reperfusion , Retrospective Studies , Stroke/complications , Stroke/epidemiology , Thrombectomy/statistics & numerical data , Thrombolytic Therapy/statistics & numerical data , Time-to-Treatment , Treatment Outcome
8.
J Neurol ; 255(6): 903-9, 2008 Jun.
Article in English | MEDLINE | ID: mdl-18484238

ABSTRACT

OBJECTIVE: We present the electrophysiologic data at baseline of 37 patients who were included in our prospective study on sporadic adult-onset progressive muscular atrophy (PMA). The aim was to correlate electrophysiological signs of lower motor neuron (LMN) loss with clinical signs of LMN loss, and to determine the prognostic value of the distribution of electrophysiological abnormalities in patients who presented clinically with only lower motor neuron signs. METHODS: Thirty-seven patients, who met our inclusion criteria for a prospective study on sporadic adult-onset PMA, underwent extensive standardized electrophysiological examination at baseline, consisting of concentric needle EMG in three regions (cervical, thoracic and lumbosacral) and standardized nerve conduction studies. RESULTS: Denervation on needle EMG was found in 88 % of clinically affected and in 40 % of clinically unaffected limb regions. All patients with a segmental or distal phenotype at baseline who developed generalized weakness had denervation in the thoracic region. Motor nerve conduction abnormalities were found in a substantial number of nerves and included reduced CMAP amplitude, increased distal motor latency, decreased motor conduction velocity, and F-wave abnormalities. Signs of demyelination and sensory nerve conduction abnormalities were rare. CONCLUSIONS: Our electrophysiological data in patients recently diagnosed with sporadic progressive muscular atrophy are consistent with widespread LMN loss. Progression in patients with a segmental or distal onset of PMA may be likely if denervation is found in clinically unaffected regions, including the thoracic region.


Subject(s)
Electrodiagnosis/methods , Motor Neuron Disease/diagnosis , Motor Neuron Disease/physiopathology , Motor Neurons/pathology , Muscular Atrophy, Spinal/diagnosis , Muscular Atrophy, Spinal/physiopathology , Adult , Aged , Amyotrophic Lateral Sclerosis/diagnosis , Amyotrophic Lateral Sclerosis/physiopathology , Disease Progression , Electromyography , Female , Humans , Male , Middle Aged , Muscle Weakness/diagnosis , Muscle Weakness/etiology , Muscle Weakness/physiopathology , Muscle, Skeletal/innervation , Muscle, Skeletal/physiopathology , Nerve Degeneration/diagnosis , Nerve Degeneration/etiology , Nerve Degeneration/physiopathology , Neural Conduction/physiology , Peripheral Nerves/physiopathology , Predictive Value of Tests , Prospective Studies
9.
J Neurol Sci ; 394: 132-137, 2018 11 15.
Article in English | MEDLINE | ID: mdl-30261428

ABSTRACT

INTRODUCTION: Patients with complete occlusion of the internal carotid artery (CAO) are vulnerable to cerebral hypoperfusion. Since cerebral hypoperfusion is associated with accelerated cognitive decline, patients with CAO may have an increased risk of cognitive impairment. We aimed to assess the prevalence and profile of cognitive impairment in patients with CAO and to explore the relation between hemodynamic impairment and cognitive functioning. METHODS: We systematically searched Medline and EMBASE for studies including patients with symptomatic or asymptomatic CAO subjected to cognitive testing that were published between 1980 and 2017. We did not include patients with carotid stenosis. We obtained data on type of study, patient characteristics, cerebral imaging and neuropsychological testing. In addition, we extracted data on potential causes of systemic hemodynamic impairment and the presence and stage of cerebral hemodynamic impairment. We assessed methodological quality of included studies with the Newcastle-Ottawa Scale. RESULTS: We found eight studies comprising 244 patients (mean age 61 years, 76% male, 93% symptomatic CAO). The proportion of patients with cognitive impairment ranged from 54 to 71% in four studies; in the other four studies patients with CAO performed worse on cognitive testing than controls, but results were not quantified. Impairment was reported in all cognitive domains. We found no data on the association between systemic hemodynamic impairment and cognitive functioning. Studies that assessed whether cerebral hemodynamic impairment was associated with cognitive functioning showed conflicting results. CONCLUSION: In patients with CAO, cognitive impairment is present in about half to two-thirds of patients and is not restricted to specific cognitive domains. The effect of systemic and cerebral hemodynamic impairment on cognitive functioning in patients with CAO deserves further study.


Subject(s)
Carotid Stenosis/complications , Cognition Disorders/etiology , Databases, Bibliographic/statistics & numerical data , Humans
10.
Ned Tijdschr Geneeskd ; 161: D826, 2017.
Article in Dutch | MEDLINE | ID: mdl-28378696

ABSTRACT

BACKGROUND: Carotid sinus massage is a widely used method for diagnosis and treatment of supraventricular tachycardia and carotid sinus hypersensitivity. Complications, mostly neurological, can occur but are rare. Carotid stenosis is a risk factor for complications. Hearing a carotid bruit on auscultation indicates stenosis, and is a contraindication for performing carotid sinus massage. However, the sensitivity of auscultation is insufficient. CASE DESCRIPTION: A 71-year-old man with a history of hypertension and hypercholesterolemia presented to the cardiology accident and emergency department with palpitations. A supraventricular tachycardia was found on examination, for which carotid sinus massage was performed. The patient developed severe aphasia and right-sided hemiparesis caused by an extensive stroke, and died a few days later. CONCLUSION: The chance of complications following carotid sinus massage is slight; however, this type of complication can have severe consequences. Safer alternative methods may be used for patients with supraventricular tachycardia. In older patients with vascular risk factors, more extensive diagnostic investigations for carotid stenosis should be considered in the diagnostic workup for carotid sinus hypersensitivity.


Subject(s)
Carotid Sinus/physiopathology , Massage , Stroke/etiology , Aged , Carotid Arteries , Humans , Male , Syncope
11.
J Neurol Neurosurg Psychiatry ; 77(6): 743-7, 2006 Jun.
Article in English | MEDLINE | ID: mdl-16705197

ABSTRACT

BACKGROUND: Multifocal motor neuropathy (MMN) is characterised by asymmetrical weakness and muscle atrophy, in the arms more than the legs, without sensory loss. Despite a beneficial response to treatment with intravenous immunoglobulins (IVIg), weakness is slowly progressive. Histopathological studies in MMN revealed features of demyelination and axon loss. It is unknown to what extent demyelination and axon loss contribute to weakness. Unlike demyelination, axon loss has not been studied systematically in MMN. Aims/ METHODS: To assess the independent determinants of weakness in MMN, 20 patients with MMN on IVIg treatment were investigated. Using a standardised examination in each patient, muscle strength was determined in 10 muscles. In the innervating nerve of each muscle, axon loss was assessed by concentric needle electromyography, and conduction block or demyelinative slowing by motor nerve conduction studies. Multivariate analysis was used to assess independent determinants of weakness. RESULTS: Needle electromyography abnormalities compatible with axon loss were found in 61% of all muscles. Axon loss, and not conduction block or demyelinative slowing, was the most significant independent determinant of weakness in corresponding muscles. Furthermore, axon loss and conduction block were independently associated with each other. CONCLUSION: Axon loss occurs frequently in MMN and pathogenic mechanisms leading to axonal degeneration may play an important role in the outcome of the neurological deficit in patients with MMN. Therapeutic strategies aimed at prevention and reduction of axon loss, such as early initiation of treatment or additional (neuroprotective) agents, should be considered in the treatment of patients with MMN.


Subject(s)
Axons/pathology , Motor Neuron Disease/physiopathology , Neurodegenerative Diseases/physiopathology , Adult , Demyelinating Diseases/physiopathology , Electromyography , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Male , Motor Neuron Disease/drug therapy , Muscle Weakness/etiology , Muscle Weakness/physiopathology , Muscular Atrophy/etiology , Muscular Atrophy/physiopathology , Neural Conduction
12.
Neurology ; 54(7): 1518-21, 2000 Apr 11.
Article in English | MEDLINE | ID: mdl-10751269

ABSTRACT

Nine patients with multifocal motor neuropathy who had previously responded favorably to IV immunoglobulins (IVIg) were treated with interferon-beta1a. Muscle strength and disability were evaluated. In six patients there was no effect of treatment. Four patients deteriorated in such a way that IVIg had to be restarted during the study. Three patients showed an improvement that was more pronounced than on IVIg. These patients had a shorter disease duration and were less affected clinically and electrophysiologically than those who did not respond.


Subject(s)
Demyelinating Autoimmune Diseases, CNS/drug therapy , Interferon-beta/therapeutic use , Motor Neuron Disease/drug therapy , Action Potentials/drug effects , Adult , Age of Onset , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Injections, Subcutaneous , Interferon beta-1a , Male , Middle Aged , Motor Neurons/drug effects , Motor Neurons/physiology , Muscle Contraction/drug effects , Neural Conduction/drug effects , Neurophysins , Peripheral Nerves/drug effects , Peripheral Nerves/physiopathology , Psychomotor Performance/drug effects , Treatment Outcome
13.
Neurology ; 58(11): 1593-6, 2002 Jun 11.
Article in English | MEDLINE | ID: mdl-12058084

ABSTRACT

Described are patients initially diagnosed with progressive spinal muscular atrophy (PSMA), in whom further evaluation established another diagnosis. The authors prospectively investigated incident and prevalent cases of PSMA. Seventeen of 89 patients, after initial registration, were later excluded because reassessment revealed a diagnosis other than PSMA. In 11 of the 17 patients with a revised diagnosis, a potential treatment was available: multifocal motor neuropathy (7), chronic inflammatory demyelinating polyneuropathy (2), inflammatory myopathy (1), and MG (1). Other misdiagnoses included myopathy, syringomyelia, ALS, idiopathic chronic axonal polyneuropathy, and idiopathic brachial plexus neuropathy. One patient with a possible herniated lumbar disk recovered spontaneously.


Subject(s)
Diagnostic Errors , Muscular Atrophy, Spinal/diagnosis , Adult , Aged , Aged, 80 and over , Diagnosis, Differential , Female , Follow-Up Studies , Humans , Male , Middle Aged , Motor Neuron Disease/diagnosis , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis , Prospective Studies
14.
Neurology ; 54(1): 26-32, 2000 Jan 11.
Article in English | MEDLINE | ID: mdl-10636121

ABSTRACT

BACKGROUND: Several patients have been reported with an asymmetric sensory or sensorimotor demyelinating neuropathy not fulfilling the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy or multifocal motor neuropathy. OBJECTIVE: To present the clinical, electrophysiologic, radiologic, and pathologic features of six patients with an asymmetric sensory or sensorimotor demyelinating neuropathy. RESULTS: All six patients were initially affected in only one limb; in four patients the neuropathy progressed to other limbs in an asymmetric fashion during several years. On electrophysiologic examination, evidence of multifocal demyelination and conduction block in motor and sensory nerves was found in all patients. MRI of the brachial plexus revealed swollen nerves and an increased signal intensity on T2-weighted imaging in four patients. A biopsy sample taken from the brachial plexus of one patient revealed evidence of inflammation. All patients showed a beneficial response to IV immunoglobulin treatment. Thirty-four similar patients have been reported previously, many of whom were initially diagnosed as having various other (nontreatable) diseases. CONCLUSIONS: The authors propose calling this neuropathy "multifocal inflammatory demyelinating neuropathy" and considering it as a distinct clinical entity to facilitate early diagnosis of this treatable disorder.


Subject(s)
Demyelinating Diseases/diagnosis , Neuritis/diagnosis , Adult , Biopsy , Brachial Plexus/pathology , Demyelinating Diseases/pathology , Demyelinating Diseases/physiopathology , Diagnosis, Differential , Electrophysiology/methods , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Motor Neuron Disease/diagnosis , Neural Conduction , Neuritis/pathology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis
15.
Neuromuscul Disord ; 13(9): 744-50, 2003 Nov.
Article in English | MEDLINE | ID: mdl-14561498

ABSTRACT

Context. Maximal voluntary isometric contraction, a method quantitatively assessing muscle strength, has proven to be reliable, accurate and sensitive in amyotrophic lateral sclerosis. Hand-held dynamometry is less expensive and more quickly applicable than maximal voluntary isometric contraction. Objective. To investigate if hand-held dynamometry is as reliable and valid as maximal voluntary isometric contraction in measuring muscle strength in patients with an adult-onset, non-hereditary progressive lower motor neuron syndrome. Design. Two testers performed maximal voluntary isometric contraction and hand-held dynamometry measurements in six muscle groups bilaterally in patients with progressive lower motor neuron syndrome to assess reliability and validity of both the methods. Setting. Outpatient units of an academic medical center. Patients. A consecutive sample of 19 patients with non-hereditary progressive lower motor neuron syndrome (median disease duration 32.5 months, range 10-84) was tested. Outcome measures. Comparison between maximal voluntary strength contractions as measured by hand-held dynamometry and maximal voluntary isometric contraction. Results. Low intra- and interrater variation in all muscle groups were found, intraclass correlation coefficients vary between 0.86 and 0.99 for both methods. Both methods correlated well in all muscle groups with Pearson's correlation coefficients ranged between 0.78 and 0.98. Scatter plots indicated a trend to under-estimate muscle strength above 250 N by hand-held dynamometry as compared with maximal voluntary isometric contraction. Conclusions. For longitudinal evaluation of muscle strength in patients with progressive lower motor neuron syndrome (i.e. between 0 and 250 N), muscle strength can be accurate quantified with both hand-held dynamometry and maximal voluntary isometric contraction. Hand-held dynamometry has the advantage of being cheap and quickly applicable. However, our results indicate that hand-held dynamometry is less sensitive than maximal voluntary isometric contraction in detecting subnormal muscle strength in strong muscle groups (i.e. >250 N), due to limited strength of the tester.


Subject(s)
Hand Strength/physiology , Hand/physiopathology , Isometric Contraction/physiology , Motor Neuron Disease/physiopathology , Muscle, Skeletal/physiopathology , Adult , Aged , Diagnosis, Differential , Electromyography/methods , Female , Humans , Male , Middle Aged , Motor Neuron Disease/diagnosis , Reproducibility of Results , Sensitivity and Specificity
16.
J Neurol ; 250(11): 1279-92, 2003 Nov.
Article in English | MEDLINE | ID: mdl-14648143

ABSTRACT

This review discusses the most important lower motor neuron syndromes. This relatively rare group of syndromes has not been well described clinically. Two subgroups can be distinguished: patients in whom motor neurons (lower motor neuron disease (LMND)) are primarily affected or motor axons and their surrounding myelin (multifocal motor neuropathy (MMN)), both leading to muscle atrophy and weakness. Both hereditary and sporadic forms of LMND have been described. The discussion of recent advances in the genetic knowledge of several hereditary forms of LMND may lead to a better understanding of the pathophysiology and the development of therapeutic strategies. By contrast, the pathogenesis of sporadic LMND is largely unknown. It is, therefore, difficult to consider the various sporadic forms of LMND, discussed in this review, as separate diseases. Because the diagnostic and therapeutic options may differ, it would seem rational to consider sporadic LMND as a spectrum of syndromes which can be distinguished from each other on the basis of clinical presentation.MMN is a lower motor neuron syndrome with presumed immunemediated pathogenesis. Evidence of motor conduction block on nerve conduction studies and a positive response to treatment with intravenous immunoglobulins (IVIg) are considered the most relevant criteria for the diagnosis of MMN. As it is treatable, it is important to distinguish MMN from LMND. Careful electrophysiological analysis in the search for conduction block is, therefore, required in all adult patients with pure lower motor neuron syndromes. For the individual patient, distinction between the various lower motor neuron syndromes is important as it enables the physician to provide adequate information over the disease course in LMND and to facilitate early treatment in MMN.


Subject(s)
Motor Neuron Disease , Adolescent , Adult , Aged , Central Nervous System/pathology , Child , Child, Preschool , Clinical Trials as Topic , Disease Progression , Female , Humans , Infant , Infant, Newborn , Male , Middle Aged
17.
J Neurol ; 248(4): 290-6, 2001 Apr.
Article in English | MEDLINE | ID: mdl-11374093

ABSTRACT

We describe three members each of two families presenting with a hereditary form of lower motor neuron disease with adult onset and rapid progression and compare their pathological and clinical features with hereditary lower motor neuron disease with adult onset, as described in the literature. No involvement of upper motor neurons was found either clinically or pathologically. Disease progression was rapid, and the majority of patients died from respiratory failure within 1-5 years after onset of disease. On pathological examination of the spinal cord we found ballooned neurons, neuronophagia and gliosis in family A, which have been regarded as characteristic pathological features of infantile-onset spinal muscular atrophy (SMA). In family B specific neuronal changes were observed that also occur in patients with amyotrophic lateral sclerosis (ALS). An autosomal dominant mode of inheritance would seem likely in both families. In family A the pathological findings and the clinical presentation with symmetrical proximal limb weakness show similarities with autosomal dominant SMA. Based on the finding of pathological features in family B that also occur in ALS, together with the distal asymmetrical muscle weakness and bulbar signs and a high age at onset we hypothesize that the members of family B suffered from familial ALS. The disease forms in both families in our opinion further broaden the spectrum of motor neuron disease.


Subject(s)
Amyotrophic Lateral Sclerosis/genetics , Motor Neuron Disease/genetics , Respiratory Insufficiency/etiology , Spinal Cord/pathology , Adult , Age of Onset , Amyotrophic Lateral Sclerosis/pathology , Disease Progression , Humans , Leg/innervation , Male , Middle Aged , Motor Neuron Disease/pathology , Muscle Weakness , Pedigree , Prognosis , Time Factors
18.
J Neurol ; 249(3): 330-6, 2002 Mar.
Article in English | MEDLINE | ID: mdl-11993534

ABSTRACT

Disease progression in multifocal motor neuropathy (MMN) was studied by comparing severity and duration of disease. We assessed disease severity by determining muscle weakness, disability, conduction block (CB), and distal and proximal compound muscle action potential (CMAP)-amplitude in 38 patients with MMN in whom disease duration ranged from 6 months to 34 years. As indicator for an ongoing immune-mediated process, the response to one course of IVIg treatment was measured in 34 patients and associated with disease severity. With increasing disease duration, weakness and disability became significantly more severe, and the distal and proximal CMAP-amplitude decreased significantly. The number of CBs was significantly higher in patients with a disease duration longer than 10 years than in those affected less than 10 years. Thirty of the 34 patients responded to IVIg treatment. Non-responsiveness to IVIg was not associated with any of the disease variables. Severe and widespread weakness was significantly associated with a response > or = 2 on the MRC-sum-score. Our results provide evidence for a slowly progressive disease course of MMN. The good response to IVIg treatment in patients with severe and prolonged disease indicates that progression may be the result of an ongoing immune-mediated process. These findings imply that early treatment may prevent future progression of weakness and disability in patients with MMN.


Subject(s)
Immunoglobulins/therapeutic use , Motor Neuron Disease/physiopathology , Motor Neuron Disease/therapy , Action Potentials , Adult , Disease Progression , Female , Follow-Up Studies , Humans , Immunoglobulin G/administration & dosage , Immunoglobulin G/therapeutic use , Immunoglobulins/administration & dosage , Injections, Intravenous , Male , Middle Aged , Muscle Weakness/etiology , Neuropsychological Tests , Retrospective Studies , Time Factors
19.
BMJ Open ; 4(12): e007065, 2014 Dec 31.
Article in English | MEDLINE | ID: mdl-25552615

ABSTRACT

INTRODUCTION: Patients with transient ischaemic attack (TIA) or stroke are at risk for cognitive impairment and dementia. Currently, there is no known effective strategy to prevent this cognitive decline. Increasing evidence exists that physical exercise is beneficial for cognitive function. However, in patients with TIA or stroke who are at risk of cognitive impairment and dementia, only a few trials have been conducted. In this study, we aim to investigate whether a physical exercise programme (MoveIT) can prevent cognitive decline in patients in the acute phase after a TIA or minor ischaemic stroke. METHODS AND ANALYSIS: A single-blinded randomised controlled trial will be conducted to investigate the effect of an aerobic exercise programme on cognition compared with usual care. 120 adult patients with a TIA or minor ischaemic stroke less than 1 month ago will be randomly allocated to an exercise programme consisting of a 12-week aerobic exercise programme and regular follow-up visits to a specialised physiotherapist during the period of 1 year or to usual care. Outcome measures will be assessed at the baseline, and at the 1-year and 2-year follow-up. The primary outcome is cognitive functioning measured with the Montreal Cognitive Assessment (MoCA) test and with additional neuropsychological tests. Secondary outcomes include maximal exercise capacity, self-reported physical activity and measures of secondary prevention. ETHICS AND DISSEMINATION: The study received ethical approval from the VU University Amsterdam Ethics committee (2011/383). The results of this study will be published in peer-reviewed journals and presented at international conferences. We will also disseminate the main results to our participants in a letter. TRIAL REGISTRATION NUMBER: The Nederlands Trial Register NTR3884.


Subject(s)
Cognition Disorders/prevention & control , Exercise Therapy/methods , Ischemic Attack, Transient/complications , Stroke/complications , Adult , Female , Humans , Male , Secondary Prevention/methods , Single-Blind Method
20.
NeuroRehabilitation ; 34(3): 401-7, 2014.
Article in English | MEDLINE | ID: mdl-24473242

ABSTRACT

BACKGROUND: Despite the beneficial effect of cardiac rehabilitation after myocardial infarction, a rehabilitation program to improve cardiorespiratory fitness and influence secondary prevention has not been implemented for ischemic stroke and transient ischemic attack (TIA). OBJECTIVE: To investigate the safety and feasibility of a post-stroke care including an exercise program after minor ischemic stroke or TIA. METHODS: In a randomised controlled trial, 20 patients with a recent minor stroke or TIA without cardiac contraindications were randomly assigned to one of the two interventions; post-stroke care without exercise or post-stroke care with exercise. Patients were evaluated at baseline, 6 and 12 months. RESULTS: Eighteen patients completed the intervention. In none of the patients cardiopulmonary contraindications for the maximal exercise test and exercise program were found. No cardiovascular events occurred during the maximal exercise tests and exercise program. After one year, significantly more patients in the post-stroke care with exercise group achieved the composite endpoint of optimal medical therapy. CONCLUSIONS: Post-stroke care including an exercise program is safe and feasible in the acute phase after minor stroke or TIA and might be a way to increase effectiveness of secondary stroke prevention. We are currently conducting a larger trial to validate these results.


Subject(s)
Exercise Therapy , Ischemic Attack, Transient/rehabilitation , Physical Fitness , Secondary Prevention/methods , Stroke Rehabilitation , Aged , Exercise Test , Feasibility Studies , Female , Humans , Male , Middle Aged , Motivation , Risk Factors
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