A prospective study analyzing one-year multidimensional outcomes in living lung transplant donors.

The success of living-donor lobar lung transplantation (LDLLT) largely depends on donor outcome; but to date, no authors have studied health-related quality of life (HRQOL) of donors. We prospectively evaluated multidimensional outcomes before and 1 year after donor lobectomies. Patient-reported HRQOL, dyspnea, psychological status and sleep quality, and physiological pulmonary function were determined. All donors were alive without any limitations in their activities of daily living after 1 year. Postoperative pulmonary function was better than the estimated preoperative values; but, with respect to HRQOL, four of the eight subscales of the Medical Outcomes Study 36-item short form (SF-36) deteriorated significantly after donation. In addition, dyspnea assessed by the modified Medical Research Council scale also worsened significantly. In contrast, postoperative anxiety assessed by the Hospital Anxiety and Depression Scale significantly improved from baseline. The donors whose recipients died reported lower SF-36 scores with worsening sleep quality measured by Pittsburgh Sleep Quality Index. Thus, although postoperative pulmonary functions in donors were preserved, their HRQOL and dyspnea deteriorated postoperatively. Moreover, HRQOL and sleep quality were impaired in recipients who experienced poor outcomes. To capture the comprehensive outcomes in LDLLT donors after donation, patient-reported outcomes should be analyzed separately from physiological outcomes.

High-resolution computed tomography and health-related quality of life in Mycobacterium avium complex disease.

SETTING: Kyoto, Japan. OBJECTIVE: To validate the St George's Respiratory Questionnaire (SGRQ) in pulmonary Mycobacterium avium-intracellulare complex disease and to analyse the significance of high-resolution computed tomography (HRCT) findings as determinants of health-related quality of life (HRQoL) after adjusting for clinical and physiological parameters. DESIGN: Eighty-five patients completed the SGRQ, pulmonary function tests and other patient-reported measurements. HRCT findings were assessed using an established computed tomography (CT) scoring method. RESULTS: The SGRQ was validated with good internal consistency, test-retest reliability and significant correlations with most physiological variables and other patient-reported measurements. White blood cell counts, C-reactive protein levels, sputum culture results, treatment history, total CT scores, and consolidation, cavity and lobar volume-decrease CT component scores were significantly correlated with the SGRQ total and component scores. Stepwise multiple regression analyses revealed that the consolidation, cavity and lobar volume-decrease component scores were correlated with the SGRQ total and/or component scores. The total CT scores had the strongest relationships with the SGRQ total scores among the various clinical parameters tested, including microbiological, radiological, physiological and laboratory findings (32.8% of variance). CONCLUSION: HRCT findings, particularly consolidation, cavity and lobar volume-decreases, were the most significant clinical parameters related to patient HRQoL.

Long-term complications and prognosis of chronic beryllium disease.

BACKGROUND: Chronic beryllium disease (CBD) is a rare disease, and there are no previous reports that have followed CBD patients over several decades. Thus, the long-term complications and prognosis of this illness still remain unclear. OBJECTIVE: The aim of this study was to investigate long-term complications and prognosis of CBD patients. STUDY DESIGN AND METHODS: This was a retrospective study based on the medical records of all CBD patients diagnosed at Kyoto University Hospital between the period 1973 to the present day. Ultimately, ten patients whose diagnoses had been made during the period 1973 to 1977 were included. Long-term physiological and radiological change, complications and prognosis of these patients were investigated. RESULTS: Three patients completely remitted, and one died of cor-pulmonale. Among the remaining six patients, four have been followed up for more than thirty years in our institute. The majority developed mixed patterns of lung function impairment, cavity lesions of the lung, pneumothorax, and respiratory infections. CONCLUSIONS: Long-term prognosis of CBD was poor with several complications due to chronic parenchymal and airway lesions.

Dominance of ipsilateral corticospinal pathway in congenital mirror movements.

OBJECTIVE: To clarify the mechanism of congenital mirror movements. DESIGN: The triple stimulation technique (TST) and the silent period were used to investigate a patient with congenital mirror movements. The TST was used to calculate the ratio of ipsilateral to contralateral corticospinal tracts from the two hemispheres to the spinal motor neurones. RESULTS: Transcranial magnetic stimulation over unilateral M1 induced larger ipsilateral than contralateral motor evoked potentials on both sides. Only 9% of spinal motor neurones innervating the abductor digitorum minimi were excited by contralateral primary motor cortex (M1) stimulation, while 94% were excited by the ipsilateral M1 stimulation. The silent period was examined during mirror movements and with voluntary contraction of the right first dorsal interosseus mimicking mirror movements. Left M1 stimulation (through the crossed corticospinal tract) did not show any difference in silent period between the two conditions, while right M1 stimulation (through the uncrossed tract) caused a longer silent period during mirror movements than during voluntary contractions. CONCLUSIONS: The results suggest that mirror movements may be caused by a strong connection between ipsilateral M1 and the mirror movements conveyed through a dominant ipsilateral corticospinal pathway.

Longitudinal changes in health status using the chronic respiratory disease questionnaire and pulmonary function in patients with stable chronic obstructive pulmonary disease.

Long-term changes in health status have been less evaluated in patients with chronic obstructive pulmonary disease (COPD), in comparison to the changes in forced expiratory volume in 1 s (FEV1). Accordingly, we examined the clinical course of health status as well as pulmonary function in COPD patients, and investigated the relationship between the change in health status and the change in pulmonary function in a 3-year longitudinal study involving 224 patients with COPD. Health status using the Chronic Respiratory Disease Questionnaire (CRQ) and pulmonary function were measured at baseline and every six months over three years. We used the random effects model for the slopes to estimate the longitudinal changes. A total of 147 patients completed the 3-year study. The dyspnoea, fatigue, and emotional function domains of the CRQ declined slowly but significantly over 3 years (p = 0.001, 0.003, and 0.004, respectively) with a mean decline rate of 0.08/year. This means that it would take about 6 years to reach the minimal important change of 0.5 on the CRQ. The mean decline in post-bronchodilator FEV1 was 60 ml/year. None of the changes in any of the domains of the CRQ were significantly correlated with the changes in pulmonary function. We have found that, in comparison to the decline in pulmonary function, health status evaluated by the CRQ declined significantly but very slowly in three of four domains over three years in patients with COPD. Furthermore, we have demonstrated that there was no significant relationship between the change in health status and the change in pulmonary function.

Short-lasting impairment of tactile perception by 0.9Hz-rTMS of the sensorimotor cortex.

To test whether low-frequency repetitive transcranial magnetic stimulation (rTMS) of sensorimotor cortex (SM1) has prolonged effects on somatosensory function, eight subjects were given 900 TMS pulses over the left hand SM1 (0.9Hz, 90% of the resting motor threshold) or at sites 3 cm anterior or posterior to it. Tactile threshold of the right hand was increased for a short duration after rTMS over SM1, but two-point discrimination and median nerve SEPs were unaffected after rTMS at any sites.

Health status measured with the CRQ does not predict mortality in COPD.

One purpose of measuring health status is to predict future outcomes. The aim of this study was to investigate the ability of health status derived from the Chronic Respiratory Disease Questionnaire (CRQ) to predict mortality in chronic obstructive pulmonary disease (COPD). One-hundred and forty-three patients with COPD were recruited. Health status, using the CRQ, and pulmonary function were measured at entry. Mortality after 7 yrs was then assessed. Univariate and multivariate Cox proportional hazards analyses were performed to predict those factors related to mortality. Of all the patients, 13 could not be followed up and 40 had died. The survival rate was 69% at 7 yrs. Univariate regression analyses revealed that the dyspnoea and emotional function domains and the total score of the CRQ were weakly but significantly correlated with mortality from all causes. However, multivariate regression analyses revealed that age and forced expiratory volume in one second were the strongest predictors of mortality, and health status was not a significant factor. Although there was a weak but significant relationship between health status and subsequent mortality in chronic obstructive pulmonary disease, it was not significant after an adjustment for age and pulmonary function. Mortality cannot be predicted from Chronic Respiratory Disease Questionnaire scores.

Abnormal cortical mechanisms of voluntary muscle relaxation in patients with writer's cramp: an fMRI study.

Although it is hypothesized that there is abnormal motor inhibition in patients with dystonia, the question remains as to whether the mechanism related to motor inhibition is specifically impaired. The objective of the present study was to clarify the possible abnormalities of the mechanisms underlying voluntary muscle relaxation during motor preparation and execution in patients with writer's cramp, using event-related functional MRI. Eight patients with writer's cramp and 12 age-matched control subjects participated in the study. Two motor tasks were employed as an experimental paradigm. In the relaxation task, subjects were asked to hold their right wrist in the horizontal plane by maintaining moderate contraction of wrist extensor muscles in the premotor phase; they relaxed those muscles voluntarily just once during each fMRI scanning session. In the contraction task, subjects extended the right wrist voluntarily from the same premotor state as for the relaxation task. Five axial images covering the primary sensorimotor cortex (SMC) and supplementary motor area (SMA) were obtained once every second. Activated volumes in the left SMC and the SMA were significantly reduced in patients for both muscle relaxation and contraction tasks. These data suggest that there is impaired activation in both SMC and SMA in voluntary muscle relaxation and contraction in patients with writer's cramp. This implies that abnormalities of both inhibitory and excitatory mechanisms in motor cortices might play a role in the pathophysiology of focal dystonia.

Changes in indices of airway hyperresponsiveness during one year of treatment with inhaled corticosteroids in patients with asthma.

We analyzed the changes in indices of airway hyperresponsiveness, including hypersensitivity and hyperreactivity, during one year of treatment with inhaled corticosteroids. We then investigated on which of them the inhaled corticosteroids had a primary effect. Fifty outpatients with asthma were recruited and treated with inhaled beclomethasone dipropionate. They underwent bronchoprovocation tests on the initial visit and at 3, 6, and 12 months. The dose of methacholine required to produce a 20% fall in the forced expiratory volume in 1 second (PD20-FEV1) was measured to evaluate airway hypersensitivity. A relatively novel index, the percent change in the forced vital capacity (deltaFVC%) at the PD20-FEV1, was assessed as a marker of airway hyperreactivity. PD20-FEV1 and deltaFVC% were assumed to indicate the horizontal shift of the dose-response curve and the vertical change in the maximal response plateau, respectively. Log(PD20-FEV1) and deltaFVC% continued to improve throughout the year (p < 0.001 and p = 0.002, respectively). Log(PD20-FEV1) improved significantly at the 3-month evaluation (p < 0.001), and deltaFVC% improved at the 6-month evaluation (p = 0.012). Log(PD20-FEV1) had no or weak relationships with deltaFVC% at all evaluation points. In conclusion, inhaled corticosteroids continued not only to reverse the leftward shift of the curve, but also to restore the plateau. Furthermore, their effect was reflected primarily by the former rather than the latter: They should be followed separately to examine how much airway inflammation is reduced.

IKKalpha controls formation of the epidermis independently of NF-kappaB.

The IKKalpha and IKKbeta catalytic subunits of IkappaB kinase (IKK) share 51% amino-acid identity and similar biochemical activities: they both phosphorylate IkappaB proteins at serines that trigger their degradation. IKKalpha and IKKbeta differ, however, in their physiological functions. IKKbeta and the IKKgamma/NEMO regulatory subunit are required for activating NF-kappaB by pro-inflammatory stimuli and preventing apoptosis induced by tumour necrosis factor-alpha (refs 5,6,7,8,9,10,11). IKKalpha is dispensable for these functions, but is essential for developing the epidermis and its derivatives. The mammalian epidermis is composed of the basal, spinous, granular and cornified layers. Only basal keratinocytes can proliferate and give rise to differentiated derivatives, which on full maturation undergo enucleation to generate the cornified layer. Curiously, keratinocyte-specific inhibition of NF-kappaB, as in Ikkalpha-/- mice, results in epidermal thickening but does not block terminal differentiation. It has been proposed that the epidermal defect in Ikkalpha-/- mice may be due to the failed activation of NF-kappaB. Here we show that the unique function of IKKalpha in control of keratinocyte differentiation is not exerted through its IkappaB kinase activity or through NF-kappaB. Instead, IKKalpha controls production of a soluble factor that induces keratinocyte differentiation.

A comparison of the individual best versus the predicted peak expiratory flow in patients with chronic asthma.

In the management of patients with asthma, peak expiratory flow (PEF) monitoring is used and based on the individual best PEF or the predicted PEE Recent international guidelines have recommended the use of the best PEF rather than the predicted PEF as an index, although there is little evidence to support which index is more appropriate. Therefore, we investigated the relationship between the best PEF and the predicted PEF in 166 consecutive asthmatic patients to see which value would be the better basis for their PEF monitoring. All eligible patients had undergone treatment for their asthma for over 6 months and were asked to measure their PEF four times a day. The best PEF was defined as the maximal PEF achieved at any time from all previous measurements. The predicted PEF was calculated based on a report on the standard PEF in normal Japanese subjects. The mean best PEF was significantly higher than the mean predicted PEF (p < 0.001). There was a strong correlation between the best PEF and the predicted PEF (r = 0.77, p < 0.001). However, in 72 patients (43%) the ratio of the best PEF to the predicted PEF was over 110%, and in 20 patients (12%) the ratio was lower than 90%. The best PEF was higher than the predicted PEF in 76 patients (46%) and lower in 22 patients (13%) by more than 50 L/min. These results suggest that when the predicted PEF was used as the index, pulmonary function was either underestimated or overestimated in over half of these patients. Therefore, the best PEF may be the better index for the management of patients with asthma.

Implication of sensorimotor integration in the generation of periodic dystonic myoclonus in subacute sclerosing panencephalitis (SSPE).

To clarify the mechanism of periodic dystonic myoclonus in subacute sclerosing panencephalitis (SSPE), a 22-year-old patient with a clinical diagnosis of SSPE was electrophysiologically investigated. Involuntary movements consisted of generalized dystonic posturing which occurred quasiperiodically once every 4 to 8 seconds. Effects of sensory stimuli and voluntary movements were studied by means of polygraphic recording of surface electromyogram (EMG), scalp electroencephalogram (EEG), and magnetoencephalogram (MEG). EEG showed quasi-periodic, generalized, transient complexes synchronous to each dystonic myoclonus, which were preceded by a slow negative EEG shift at the parietal region by approximately 5 seconds. Neither external stimuli nor self-paced movements alone influenced the periodicity of dystonic myoclonus or EEG complexes. In the reaction time task, however, the external stimuli given as an imperative cue to execute a motor task elicited dystonic myoclonus and generalized EEG complexes only if they were presented in the latter segment of the interval between the two successive EEG complexes while the slow negative EEG shift appeared. These findings suggest that EEG complexes and periodic movements spontaneously occur when cortical excitability reaches a certain critical level, but both phenomena are elicited even before if the sensory stimuli as an imperative signal requiring motor execution are presented. This finding most likely implies involvement of the sensorimotor integration mechanism in these periodic phenomena.

Desynchronization and synchronization of central 20-Hz rhythms associated with voluntary muscle relaxation: a magnetoencephalographic study.

To clarify the cortical mechanisms involved in motor inhibition, modulation of cortical rhythms around 20 Hz during voluntary muscle relaxation was compared to that during muscle contraction in ten normal volunteers, using a whole head type neuromagnetometer. Each subject relaxed or contracted the right forearm muscles, from which electromyograms were recorded. After bandpass filtering magnetoencephalographic signals into frequency bands of 6-10, 10-14, 14-18, 18-22, 22-26, and 26-30 Hz, the signals of each frequency band were rectified and averaged with respect to the onset of motor trial. The relaxation task showed movement-related 20-Hz desynchronization over bilateral central areas beginning a few seconds before the termination of muscle contraction. Twenty-hertz desynchronization was present also for the contraction task at the same location within each subject. The two tasks had a positive correlation among subjects in both the percent decrease (r2=0.76, P<0.01) and the peak time (r2=0.61, P<0.05) for the contralateral 20-Hz desynchronization. After the muscle relaxation, desynchronization was followed by conspicuous movement-related synchronization of the 20-Hz passband in the contralateral central areas, which was significantly larger than that after the contraction (P<0.001). The results suggest that the voluntary muscle relaxation involves the modulation of central rhythms starting a few seconds before the actual event, and the 20-Hz desynchronization has a similar temporal property in the muscle relaxation and contraction. The 20-Hz synchronization in the contralateral central area after the muscle relaxation may be associated with the temporally arrayed termination of the ongoing muscle contraction.

Stages of disease severity and factors that affect the health status of patients with chronic obstructive pulmonary disease.

BACKGROUND: We hypothesized that the factors which may influence health status would differ in patients at different disease stages of chronic obstructive pulmonary disease (COPD). The present study investigated how impairments in health status were distributed in male patients at each disease stage according to the British Thoracic Society (BTS) guidelines, and analysed the contribution of the clinical indices, the dyspnoea rating and the psychological status to the health status of patients at the three disease stages of COPD. METHODS: A total of 218 consecutive male patients with stable COPD were recruited from our outpatient clinic. All eligible patients completed pulmonary function testing, progressive cycle ergometry, a dyspnoea rating [Medical Research Council (MRC) dyspnoea scale], an assessment of their anxiety and depression [Hospital Anxiety and Depression Scale (HADS)], and an assessment of their health status [the St. George's Respiratory Questionnaire (SGRQ)]. The patients were categorized into three groups: mild COPD with a FEV1 at 60-79% of the predicted value, moderate COPD at 40-59% of the predicted value, and severe COPD at below 40% of the predicted value. RESULTS: Twenty-five patients (11%) had mild COPD, 72 patients (33%) had moderate COPD, and 121 patients (56%) had severe COPD. Significant differences were observed for the total score and for three components on the SGRQ among patients at the three stages (one-way ANOVA, P<0.05). The scores for the total SGRQ and for the activity component were significantly higher for patients with severe COPD than for patients with moderate COPD [Fisher's least-significant-difference (LSD) method, P<0.05], and also significantly higher for moderate COPD patients than for mild COPD patients. The maximal oxygen uptake (VO2 max) correlated significantly with the total SGRQ score in the mild patients [Pearson's correlation coefficient (r) = -0.67], but not in the moderate or severe patients. The MRC dyspnoea scale had strong correlations with the SGRQ in all patient groups (r = 0.53 to approximately 0.70). Anxiety and depression on the HADS showed moderate correlations with the SGRQ score in the mild and severe patients (r = 0.51 to approximately 0.57). Multiple regression analysis showed that in patients with mild COPD, the MRC and VO2 max accounted for the total score on the SGRQ. Anxiety on the HADS plus the MRC scale accounted for the total score on the SGRQ in patients with moderate COPD, and anxiety on the HADS, the MRC scale and the FEV1 significantly influenced the SGRQ severe COPD patients. CONCLUSIONS: The disease staging proposed by the BTS guidelines can separate patients with COPD according to impairments in their health status. Furthermore, the factors that influence health status differed in patients at the three disease stages. Our findings support the boundaries used in disease staging and some recommendations from the BTS guidelines.

Membranous lipodystrophy presenting with palilalia: a PET study of cerebral glucose metabolism.

A case of membranous lipodystrophy (Nasu-Hakola disease; NHD) associated with palilalia was reported. A 38-year-old Japanese woman developed walking difficulty in her twenties. At age 35 she manifested neuropsychiatric symptoms characterized by euphoria, palilalia and dementia. A bone marrow biopsy showed periodic acid Schiff-positive membranous cystic lesions in the adipose tissue. Positron emission tomography with (18F)-2-fluoro-2-deoxy-D-glucose disclosed that regional cerebral glucose metabolism was decreased in the bilateral frontal white matter with mild hypometabolism in the thalamus and basal ganglia; all predominantly on the right. Taken together with the previous postmortem findings, it is postulated that frontal lobe hypofunction, predominantly in the right hemisphere, produced the unique neuropsychiatric symptoms in this patient.

The effects of oxitropium bromide on exercise performance in patients with stable chronic obstructive pulmonary disease. A comparison of three different exercise tests.

The purpose of the present study was to compare the characteristics of three different exercise tests in evaluating the effects of oxitropium bromide on exercise performance. Thirty-eight males with stable chronic obstructive pulmonary disease (COPD) (FEV(1) = 40.8 +/- 16.5% predicted; mean +/- SD) completed randomized, double-blind, placebo-controlled, crossover studies for each exercise test. The exercise tests were performed 60 min after the inhalation of either oxitropium bromide 400 microg or placebo. The patients performed 6-min walking tests (6MWT) on Days 1 and 2, progressive cycle ergometry (PCE) on Days 3 and 4, and cycle endurance tests at 80% of the maximal workload of PCE on Days 5 and 6. Spirometry was conducted before and at 45 and 90 min after the inhalation. Oxitropium bromide significantly increased FEV(1) as compared with placebo. Oxitropium bromide increased the endurance time significantly, by 19% (p < 0.001), and caused a small but significant increase in the 6-min walking distance by 1% (p < 0.05), but induced no significant increase in maximal oxygen consumption (V O(2)max) in PCE. The responses in these three exercise tests were different, and we conclude that the endurance test was the most sensitive in detecting the effects of inhaled anticholinergic agents on exercise performance in patients with stable COPD. An endurance procedure may be performed to detect clinical changes in evaluating the effects of oxitropium bromide on exercise performance.

A comparison of the level of dyspnea vs disease severity in indicating the health-related quality of life of patients with COPD.

STUDY OBJECTIVES: To compare categorizations of the level of dyspnea with the staging of disease severity as defined by the FEV(1) in representing how the health-related quality of life (HRQOL) is distributed in patients with COPD. DESIGN: Cross-sectional study. SETTING: Outpatient clinic at the respiratory department of a university hospital. PATIENTS: A total of 194 consecutive male patients with stable, mild-to-severe COPD. MEASUREMENTS: The score distributions for the components of the St. George's respiratory questionnaire (SGRQ) were used as disease-specific HRQOL measures, and the scores from the Medical Outcomes Study Short Form 36-item questionnaire (SF-36) were used as generic HRQOL measures. These scores were stratified according to the level of dyspnea, as defined by the Medical Research Council (MRC) dyspnea scale, and the stage of disease severity, as defined by the American Thoracic Society (ATS). Differences in the HRQOL scores among the subgroups were compared by an analysis of variance (ANOVA). Multiple pairwise comparisons were made with Fisher's least significant difference (LSD) method, with the overall alpha-level set at 0.05. RESULTS: In those groups classified according to the level of dyspnea, significant differences were observed for the scores on the SGRQ and SF-36 (ANOVA, p < 0.05). The scores for activity and impact, and the total scores of the SGRQ and all scales, except for bodily pain and general health on the SF-36, were significantly worse for patients with severe dyspnea (MRC scale grades, 3, 4, and 5, respectively) than for those with moderate dyspnea (MRC grade level, 2; Fisher's LSD method, p < 0.05). Significant differences were recognized among the different stages of disease severity with respect to the scores from all scales of the SF-36, except for bodily pain, and all scores from the SGRQ (ANOVA, p < 0.05). However, differences in the scores on the SGRQ and SF-36 between patients with ATS stage II disease (FEV(1), 35 to 49% predicted) and stage III disease (FEV(1), < 35% predicted) were not statistically significant. CONCLUSIONS: Using the SGRQ and SF-36, the HRQOL of patients with COPD was more clearly separated by the level of dyspnea than by the ATS disease staging. In addition to the ATS disease staging, categorizations based on the level of dyspnea may be useful to clinicians in terms of the HRQOL of COPD patients.

Comparison of risperidone and mosapramine addition to neuroleptic treatment in chronic schizophrenia.

There is little information regarding the effects of risperidone addition to neuroleptic treatment in chronic schizophrenia. As a preliminary study, 10 neuroleptic-treated schizophrenic inpatients received risperidone (high 5HT2A/D2 ratio, i.e. the ratio between 5HT2A and D2 receptor occupancy) and mosapramine (low 5HT2A/D2 ratio) in a randomized, single-blind, crossover, add-on study consisting of 8 weeks of treatment each with risperidone and mosapramine. Although both additions resulted in significant, albeit modest, improvement, there was no significant difference in the scores on the Positive and Negative Syndrome Scale for Schizophrenia between risperidone and mosapramine addition. These results suggest that risperidone and mosapramine bring about comparable effects in add-on design. Thus, risperidone with a high 5HT2A/D2 ratio does not seem to be better than mosapramine with a low 5HT2A/D2 ratio when combined with conventional neuroleptics. Further studies including a large number of patients and a double-blind design are needed.

Severe motor-dominant neuropathy with IgM M-protein binding to the NeuAcalpha2-3Galbeta- moiety.

We report the occurrence of a relapsing, severe predominantly motor neuropathy in a 75-year-old man with an IGM-K M-protein binding to gangliosides GM2, GM3, GM4, GD1a, GT1b and LM1. Motor nerve conduction velocities were slowed with conduction block. A superficial peroneal nerve biopsy specimen revealed segmental demyelination and remyelination. The patient improved after repeated plasma exchanges, and the antibody titer decreased in association with clinical recovery. This IgM M-protein has a unique, previously unreported binding specificity for terminal NeuAcalpha2-3Galbeta- moiety in common to all gangliosides bound by the antibody except GM2. M-proteins with this affinity may be involved in the pathogenesis of this and other cases of motor-dominant demyelinating neuropathy.