Development of a patient decision aid for discharge planning of hospitalized patients with stroke.

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.

The patient-reported outcomes measurement information systems (PROMIS®) physical function and its derivative measures in adults: a systematic review of content validity.

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.

Reducing the Number of Test Items of the Action Research Arm Test Poststroke: A Decision Tree Analysis✰.

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.

Experiences with information provision and preferences for decision making of patients with acute stroke.

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.

Transition of care in stroke patients discharged home: a single-center prospective cohort study.

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.

Impact of the COVID-19 outbreak on acute stroke care.

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.

Screening and patient-tailored care for emotional and cognitive problems compared to care as usual in patients discharged home after ischemic stroke (ECO-stroke): a protocol for a multicenter, patient-blinded, cluster randomized controlled trial.

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.

Cognitive functioning in patients with carotid artery occlusion; a systematic review.

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.

[A severe stroke following carotid sinus massage]. / Een ernstig herseninfarct na carotismassage.

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.

Safety and feasibility of post-stroke care and exercise after minor ischemic stroke or transient ischemic attack: MotiveS & MoveIT.

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.

A randomised controlled trial of aerobic exercise after transient ischaemic attack or minor stroke to prevent cognitive decline: the MoveIT study protocol.

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.

Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy.

OBJECTIVE: Identification and examination of all patients with multifocal motor neuropathy (MMN) in the Netherlands to document the clinical spectrum and response to IV immunoglobulin (IVIg) and to determine correlates of outcome. METHODS: A national cross-sectional descriptive study was performed. Ninety-seven patients were identified; 88 participated. Logistic regression analysis was used to study determinants of outcome. RESULTS: Age at onset was younger in men than in women (38 vs 45 years, p = 0.05). Onset of weakness was in distal arm (61%) or distal leg (34%), and occasionally in the upper arm (5%). Initial diagnosis was motor neuron disease in one-third of patients. Brisk, but not pathologic, reflexes in weakened muscles were found in 8%. Conduction blocks were most frequently detected in the ulnar (80%) and median (77%) nerves, but occasionally only between Erb and axilla (6%), or in the musculocutaneous nerve (1%). Ninety-four percent responded to IVIg therapy: nonresponders had longer disease duration before the first treatment (p = 0.03). Seventy-six percent received IVIg maintenance treatment at the time of this study (median duration 6 years; range 0-17): the median dose increased over the years from 12 to 17 g per week (p < 0.01). Independent determinants of more severe weakness and disability were axon loss (p < 0.001; p < 0.0001) and longer disease duration without IVIg (p = 0.03; p = 0.07). CONCLUSION: The results of this study may help aid recognition the clinical picture of MMN. Early IVIg treatment may help to postpone axonal degeneration and permanent deficits. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that IVIg improves muscle strength of patients with MMN and disability (defined as an increase of >or=1 Medical Research Council grade in at least 2 muscle groups without decrease in other muscle groups) in 94% (95% confidence interval, 86.8%-97.4%) of patients.

Interpretation of electrodiagnostic findings in sporadic progressive muscular atrophy.

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.

Axon loss is an important determinant of weakness in multifocal motor neuropathy.

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.

The spectrum of lower motor neuron syndromes.

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.

Comparison of maximal voluntary isometric contraction and hand-held dynamometry in measuring muscle strength of patients with progressive lower motor neuron syndrome.

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.

Sporadic lower motor neuron disease with adult onset: classification of subtypes.

The discovery of the genetic basis of hereditary lower motor neuron disease (LMND) and the recognition of multifocal motor neuropathy as a distinct clinical entity necessitate a new classification of LMND. To this end, we studied the clinical and electrophysiological features of 49 patients with sporadic adult-onset LMND in a cross-sectional study. Disease duration was more than 4 years to exclude the majority of patients with amyotrophic lateral sclerosis. Based on the pattern of weakness, we identified three groups: 13 patients with generalized weakness (group 1); eight patients with symmetrical, distal muscle weakness (group 2); and 28 patients with non-generalized asymmetrical weakness of the arms in most patients (group 3). Group 3 could be subdivided into patients with weakness in predominantly the distal (group 3a) or the proximal (group 3b) muscle groups, both with disease progression to adjacent spinal cord segments. Distinctive features of group 1 were an older age at onset, more severe weakness and muscle atrophy, lower reflexes, greater functional impairment, more widespread abnormalities on concentric needle EMG, respiratory insufficiency and serum M-protein. In groups 2 and 3, concentric needle EMG findings also suggested a more widespread disease process. Retrospectively, the prognosis of sporadic adult-onset LMND appears to be favourable, because clinical abnormalities were still confined to one limb in most patients after a median disease duration of 12 years. We propose to classify the patients in the different subgroups as slowly progressive spinal muscular atrophy (group 1), distal spinal muscular atrophy (group 2), segmental distal spinal muscular atrophy (group 3a) and segmental proximal spinal muscular atrophy (group 3b). The described clinical phenotypes may help to distinguish between different LMND forms.

Demyelination and axonal loss in multifocal motor neuropathy: distribution and relation to weakness.

Multifocal motor neuropathy (MMN) is characterized by a slowly progressive, asymmetric weakness of the limbs without sensory loss. The arms are usually affected to a greater extent than the legs, and distal muscles more than proximal muscles. The distribution of electrophysiological abnormalities and its correlation with weak muscle groups in MMN have not been investigated systematically. The aim of the present study was to assess whether electrophysiological abnormalities have a preferential or random distribution, whether electrophysiological abnormalities in a nerve correlate with weakness in the innervated muscles, and whether these results are relevant for the development of optimal electrodiagnostic protocols. We compared the pattern of weakness and electrophysiological abnormalities in 39 patients with a lower motoneuron syndrome and a positive response to intravenous immunoglobulins. All patients underwent an extensive standardized electrophysiological examination. Electrophysiological evidence of demyelination was found more often in the nerves of the arms and was distributed randomly over lower arm, upper arm and shoulder segments. Electrophysiological evidence of axonal loss presented more frequently in longer nerves, occurring most often in the leg nerves. For the arm nerves, it is possible that the length dependence of axonal loss is due to the random distribution of demyelinating lesions that lead to axonal degeneration. Weakness was associated with features of demyelination and axonal loss in the nerves of the arm, and with features of axonal loss in leg nerves. However, a substantial number (approximately one-third) of electrophysiological abnormalities were found in nerves innervating non-weakened muscles. These results imply that in MMN, conduction block is most likely to be found in long arm nerves innervating weakened muscles, but if conduction block cannot be detected in these nerves, the electrophysiological examination should be extended to other arm nerves including those innervating non-weakened muscles.

Multifocal motor neuropathy: long-term clinical and electrophysiological assessment of intravenous immunoglobulin maintenance treatment.

We performed a long-term follow-up study of 11 patients with multifocal motor neuropathy (MMN) who received maintenance treatment with intravenous immunoglobulins (IVIg). Patients were treated initially with one full course of IVIg (0.4 g/kg for 5 days) followed by one IVIg infusion (0.4 g/kg) every week. During follow-up, the frequency and dosage of IVIg infusions were determined for each patient and ranged from one infusion every 1 to 7 weeks and an average dose of 7 to 48 g per week. During the 4- to 8-year follow-up period, muscle strength was assessed by measuring the MRC (Medical Research Council) sumscore of 20 muscle groups and by performing hand-held dynamometry on a selection of weak muscle groups. Systematic electrophysiological studies were performed before treatment and each year during IVIg maintenance treatment. Disability was assessed with the upper limb and lower limb subscales of the Guy's Neurological Disability Scale before treatment, after the first full course of IVIg and at the last follow-up examination. Muscle strength improved significantly within 3 weeks of the start of IVIg treatment and was still significantly better at the last follow-up examination than before treatment, even though it decreased slightly and significantly during the follow-up period. Upper limb disability was significantly better after the first full course of IVIg than before treatment. Conduction block disappeared in six nerve segments but new conduction block appeared in eight nerve segments during the follow-up period. Changes consistent with improvement (remyelination or reinnervation) occurred in 13 nerves during follow-up and changes consistent with worsening (demyelination or axon loss) occurred in 14 nerves. Electrophysiological changes consistent with improvement were significantly associated with the presence of conduction block before IVIg treatment. In conclusion, IVIg maintenance treatment has a beneficial long-term effect on muscle strength and upper limb disability but may not prevent a slight decrease in muscle strength. The electrophysiological findings imply that IVIg treatment favourably influences the mechanisms of remyelination or reinnervation but that axon loss cannot be prevented.