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Purpose of Review: Chronic idiopathic axonal polyneuropathy (CIAP), a common neurological condition, is considered to be a benign neurological condition with a small risk of disability. However, many studies have shown a reduced quality of life and a nonnegligible affection of daily activities in patients with CIAP. Here we summarize recent data about CIAP., Recent Findings: We discuss some of the latest articles regarding risk factors, comorbidities, and possible pathogenic factors regarding CIAP. Patients with chronic polyneuropathy have impaired walking capacity, disturbed balance, and an increased risk of falls. Idiopathic polyneuropathy has a negative impact on activities of daily living. Patients with CIAP may develop plantar ulcers and neuropathic arthropathy. Small fiber involvement may occur, and two recent studies indicate that neuropathic pain is present in about two thirds of the CIAP group. Furthermore, patients with CIAP with neuropathic pain have increased fatigue and poorer emotional well being., Summary: Despite the relatively mild motor impairment seen in most patients with CIAP, the condition causes limitations in life with decreased mobility, pain, and affection of basal daily activities. Because the pathogenesis of CIAP in unclear, there is no disease modifying treatment. Further studies regarding pathogenesis, and randomized controlled clinical trials regarding possible treatment options are needed.

Polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS-PNP) has a chronic and slowly progressive course but can lead to significant disability and reduced quality of life (QoL). The aim of this study was to analyze QoL in MGUS-PNP patients and to determine its predictors. Our study included 51 patients diagnosed with MGUS-PNP (23.5% with IgM, 66.7% IgG or IgA, 7.8% undetermined paraprotein, 2.0% light chains). QoL was assessed using the SF-36 questionnaire. The Medical Research Council Sum Score (MRC-SS), INCAT disability and sensory scores, ataxia score, Krupp's Fatigue Severity Scale and Beck's Depression Inventory were also used. Total SF-36 score was 50.0 ± 21.4 and no difference was observed between IgM and IgG/IgA MGUS-PNP. Physical composite score was worse than mental (44.4 ± 21.4 vs. 54.5 ± 20.9). Following factors showed correlation with SF-36 total score in univariate analysis: INCAT disability score, MRC-SS, INCAT sensory score, level of ataxia, fatigue and depression (p < 0.01). Significant predictors of worse SF-36 total score in our MGUS-PNP patients were depression (β = - 0.46, p < 0.01), fatigue (β = - 0.32, p < 0.01) and INCAT disability score (β = - 0.27, p < 0.01). QoL in MGUS-PNP is equally affected in patients with different types of paraprotein. MGUS-PNP patients with more severe functional disability, fatigue and depression need special attention of clinicians since they could be at higher risk to have worse QoL. This should be taken into account when treating subjects with MGUS-PNP.

Approximately one of three people with diabetes is affected by distal symmetric sensorimotor polyneuropathy (DSPN) which is associated with marked impairment in quality of life due to partly excruciating neuropathic pain on the one hand and painless foot ulcers on the other hand. The prevalence of painful DSPN may reach up to one quarter of patients with diabetes, while DSPN may be asymptomatic in up to half of the patients affected. Regrettably, DSPN still remains underdiagnosed. Typical neuropathic symptoms include pain, paresthesias and numbness particularly in the feet and calves. The management of DSPN includes three cornerstones: (1) lifestyle modification, causal treatment aimed at near-normoglycemia and multifactorial cardiovascular risk intervention, (2) pathogenesis-derived treatment and (3) symptomatic treatment of neuropathic pain. Multimodal pain treatment should not only aim at pain relief, but also allow for improvement in quality of sleep, mobility, and general quality of life.

In this study, we assessed the modifications over time of daily activities and quality of life (QoL) in 32 subjects with anti-myelin-glycoprotein (MAG) antibody neuropathy. A widespread panel including clinical scores and patient-reported questionnaires, in compliance of the terms by the International Classification of Functioning, Disability, and Health (ICF) of the World Health Organization (WHO), was employed at enrollment (T0) and at follow-up evaluation (T1) after a mean interval of 15.4 ± 5.7 months. The Sensory Modality Sum score (SMS) at four limbs showed a significant worsening over time (mean score 27.2 ± 3.9 at T0 vs 25.7 ± 3 at T1 at upper limbs, P = .03; 20.5 ± 4.8 at T0 vs 18.6 ± 5.9 at T1 at lower limbs, P = .04). The Visual Analogue Scale (VAS) for pain significantly worsened at upper limbs at T1 (mean values 0.84 ± 1.95 at T0 vs 1.78 ± 2.6 at T1, P = .03). All the other tests did not show significant differences between T0 and T1. In the subgroup who underwent rituximab (15/32 treated before T0, 3/32 patients treated between T0 and T1 with median interval of 1 year), no significant differences were observed between T0 and T1. Despite the quite long follow-up, statistical significance was not achieved either for the limited number of patients or for the lack of sensitive outcome measures. In our cohort, the significant worsening of the SMS and VAS after a median of 14 months can be considered as a reliable expression of the natural history of the disease, and suggest that these scales might represent possible outcome measures in anti-MAG antibody neuropathy., (© 2019 Peripheral Nerve Society.)

Introduction and Aim: Multifocal motor neuropathy (MMN) is a rare, chronic disorder with potentially severe and progressive disability, which may affect patients' quality of life (QoL). Since there is still small number of studies that predominantly investigated QoL in patients with MMN, we sought to analyze QoL in these patients., Materials and Methods: Our study comprised 17 patients diagnosed with MMN at the same clinic. Following scales were used: SF-36 questionnaire, INCAT disability scale, Krupp's Fatigue Severity scale, and Beck Depression Inventory., Results: Physical domains of QoL were slightly more affected than mental ones, but with no statistical significance (64.8 ± 22.3 vs. 70.0 ± 19.5, p > 0.05). Total SF-36 score was 69.2 ± 19.9. INCAT arm disability score at testing was found to correlate with the total SF-36 score (rho = -0.603, p < 0.05). INCAT arm disability score at diagnosis (rho = -0.57, p < 0.05) and at testing (rho = -0.48, p = 0.05) correlated with physical composite score (PCS). Disease duration (rho = -0.51, p < 0.05) and INCAT arm disability score at testing (rho = -0.60, p = 0.01) were associated with mental composite score (MCS)., Conclusion: QoL in patients with MMN was reduced, especially in physical domains. Although arm disability was the most significant parameter which affected QoL of MMN patients in both physical and mental aspects, longer disease duration should not be underestimated as a psychological burden for these patients., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Chronic polyneuropathy is a disabling condition of the peripheral nerves, characterized by symmetrical sensory motor symptoms and signs. There is paucity of studies on the etiological spectrum of polyneuropathy and its impact on quality of life (QoL). The present cross-sectional study in a referral based tertiary care center in North India found diabetic neuropathy as the commonest cause (25.5%) amongst 212 patients with chronic polyneuropathy. Idiopathic axonal polyneuropathy was present in 14.2% patients. Leprosy presenting as confluent mononeuritis multiplex constituted 11.3% of the patients. Additionally, it revealed a significantly worse QoL in these patients in all domains measured by short form (SF-36). This is the first study conducted in India to determine the QoL in chronic neuropathy patients. The current study demonstrates the clinical feasibility and applicability of the SF-36 generic health status in patients with polyneuropathies., (© 2018 Peripheral Nerve Society.)

Background: Fatigue often occurs as long-term complication in chronically critically ill (CCI) patients after prolonged intensive care treatment. The Multidimensional Fatigue Inventory (MFI-20) has been established as valid instrument to measure fatigue in a wide range of medical illnesses. Regarding the measurement of fatigue in CCI patients, the psychometric properties of the MFI-20 have not been investigated so far. Thus, the present study examines reliability and validity of the MFI-20 in CCI patients., Methods: A convenience sample of n = 195 patients with Critical Illness Polyneuropathy (CIP) or Myopathy (CIM) were recruited via personal contact within four weeks (t1) following the transfer from acute care ICU to post-acute ICU at a large rehabilitation hospital. N = 113 (median age 61.1 yrs., 72.6% men) patients were again contacted via telephone three (t2) and six (t3) months following the transfer to post-acute ICU. The MFI-20, the Euro-Quality of Life (EQ-5D-3 L) and the Structured Clinical Interview for the Diagnostic and Statistical Manual of mental disorders DSM-IV (SCID-I) were applied within this prospective cohort study., Results: The internal consistency Cronbach's α was adequate for the MFI-total and all but the subscale Reduced Motivation (RM) (range: .50-.91). Item-to-total correlations (range: .22-.80) indicated item redundancy for the subscale RM. Confirmatory Factor analyses (CFAs) revealed poor model fit for the original 5-factor model of the MFI-20 (t2/t3, Confirmatory Fit Index, CFI = .783/ .834; Tucker-Lewis Index, TLI = .751/ .809; Root Mean Square Error of Approximation, RMSEA = .112/ .103). Among the alternative models (1-, 2-, 3-factor models), the data best fit to a 3-factor solution summarizing the highly correlated factors General -/ Physical Fatigue/ Reduced Activity (GF/ PF/ RA) (t2/ t3, CFI = .878/ .896, TLI = .846/ .869, RMSEA = .089/ .085, 90% Confidence Interval .073-.104/ .066-.104). The MFI-total score significantly correlated with the health-related quality of life (range: -.65-(-).66) and the diagnosis of major depression (range: .27-.37)., Conclusions: In the present sample of CCI patients, a reliable and valid factor structure of the MFI-20 could not be ascertained. Especially the subscale RM should be revised. Since the factors GF, PF and RA cannot be separated from each other and the unclear factorial structure in the present sample of CCI patients, the MFI-20 is not recommended for use in this context., Trial Registration: German Clinical Trials Registration DRKS00003386 . Registered 13 December 2011, retrospectively registered.

Background: Prior clinical trials have suggested that home-based Ig treatment in multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and its variant Lewis-Sumner syndrome (LSS) is safe and effective and is less costly than hospital-administered intravenous immunoglobulin (IVIg)., Methods: A French prospective, dual-center, cost minimization analysis was carried out to evaluate IVIg administration (5% concentrated) at home versus in hospital with regard to costs, patients' autonomy, and patients' quality of life. The primary endpoint was the overall cost of treatment, and we adopted the perspective of the payer (French Social Health Insurance)., Results: Twenty-four patients aged 52.3 (12.2) years were analyzed: nine patients with MMN, eight with CIDP, and seven with LSS. IVIg (g/kg) dosage was 1.51 ± 0.43 in hospital and 1.52 ± 0.4 at home. Nine-month total costs per patient extrapolated to 1 year of treatment were €48,189 ± 26,105 versus €91,798 ± 51,125 in the home and hospital groups, respectively ( p  <   .0001). The most frequently reported factors for choosing home treatment were the good tolerance and absence of side effects of IVIg administration, as well as a good understanding of the advantages and drawbacks of home treatment (75% of respondents). The mRankin scores before and after switch to home treatment were 1.61 ± 0.72 and 1.36 ± 0.76, respectively ( p  =   .027)., Discussion: The switch from hospital-based to home-based IVIg treatment for patients with immune neuropathy represents potentially significant savings in the management of the disease.

Introduction: Generic health-related quality-of-life (HRQOL) patient-reported outcome measures have been used in patients with chronic immune-mediated polyneuropathies. We have created a disease-specific HRQOL instrument., Methods: The chronic acquired polyneuropathy patient-reported index (CAP-PRI) was developed and validated in multiple steps. Items were initially generated through patient and specialist input. The performance of the preliminary 20 items was analyzed via a prospective, 5-center study involving chronic immune-mediated polyneuropathy patients., Results: Data analysis suggested modification to a 15-item scale with 3 response categories rather than 5. The final CAP-PRI was validated in another prospective, 5-center study. The CAP-PRI appeared to be a unidimensional outcome measure that fit the Rasch model in our multicenter cohort. It correlated appropriately with outcome measures commonly used in this patient population., Conclusions: The CAP-PRI is a simple disease-specific HRQOL measure that appears to be useful for clinical care and possibly also for clinical trials. Muscle Nerve 54: 9-17, 2016., (© 2015 Wiley Periodicals, Inc.)

Background: Polyneuropathy presumably lowers quality of life (QoL). However, there is a lack of systematic studies that assess QoL changes and biomarkers of polyneuropathy as determinants of QoL. We aimed to investigate the relationship between every specific aspect of QoL and the clinical parameters used to assess the impairment of motor, sensory (large and small fibers), and autonomic nerves in polyneuropathy., Methods: Polyneuropathy patients were recruited from September 2013 to March 2014; QoL was assessed using (1) the WHO Quality of Life-BREF (WHOQoL), (2) the European Quality of Life-5 Dimensions, and (3) the Brief Pain Inventory Short Form. Neuropathy examinations included nerve conduction studies, autonomic function tests, quantitative sensory testing (QST), and intraepidermal nerve fiber (IENF) density assessment of skin biopsies., Results: There were 61 polyneuropathy patients (male/female = 38/23, mean age 58.14 ± 12.95 years). Patients had a lower QoL than age-and gender-matched controls in the physical and psychological domains of the WHOQoL. Among the biomarkers for different nerve fiber categories, only the small fiber neuropathy assessments were significantly related to all domains of the WHOQoL. In contrast, the parameters of the large fiber neuropathy were independent of QoL. Patients with abnormal temperature thresholds and a lower IENF density had lower WHOQoL scores compared to patients with normal thresholds and IENF densities. Warm threshold of the foot in QST was linearly correlated with all domains of the WHOQoL., Conclusions: QoL scores were reduced in polyneuropathy, and biomarkers of small fiber neuropathy, i.e., warm threshold and IENF density were discriminating predictors of QoL.

Background: Distal sensory polyneuropathy (DSP) is a common complication of HIV disease. Its effects on quality of life (QOL) and function have not been well described., Objective: The study objectives were: (1) to compare QOL and lower extremity function in people with HIV-related DSP and people with HIV disease who do not have DSP, (2) to determine the extent to which function predicts QOL, (3) to evaluate the agreement of 2 function scales, and (4) to describe the use of pain management resources., Design: This was a cross-sectional survey study with predictive modeling and measurement tool concordant validation., Methods: A demographic questionnaire, the Medical Outcomes Study HIV Health Survey, the Lower Extremity Functional Scale (LEFS), the Lower Limb Functional Index (LLFI), and a review of medical records were used. General linear modeling was used to assess group differences in QOL and the relationship between function and QOL. Bland-Altman procedures were used to assess the agreement of the LEFS and the LLFI., Results: Usable data for analyses were available for 82 of the 94 participants enrolled. The 67% of participants who reported DSP symptoms tended to be older, had HIV disease longer, and were more likely to receive disability benefits. Participants without DSP had better LLFI, LEFS, and physical health summary scores. In multivariate models, lower limb function predicted physical and mental health summary scores. The LLFI identified participants with a lower level of function more often than the LEFS. Participants with DSP were more likely to use medical treatment, physical therapy, and complementary or alternative treatments., Limitations: A sample of convenience was used; the sample size resulted in a low power for the mental health summary score of the Medical Outcomes Study HIV Health Survey., Conclusions: Quality of life and function were more impaired in participants with HIV disease and DSP. The LLFI was more likely to capture limitations in function than the LEFS. Participants with DSP reported more frequent use of pain management resources., (© 2014 American Physical Therapy Association.)

The aim of this study was to examine the impact of social support on quality of life (QoL) in patients with polyneuropathy. One hundred and fifty-four patients with polyneuropathy were enrolled from a neuromuscular clinic. The QoL Instrument and the Medical Outcome Study-Social Support Survey (MOS-SSS) were used to assess QoL and social support, respectively. Disease severity and clinical factors were also assessed. Neuropathy patients had a lower QoL compared to a previously published normative sample (p < 0.0001) and an MOS-SSS comparable to other patients with chronic disease. Social support correlated weakly with the self esteem and emotional well being mental health dimensions (rs :0.20-0.38) but not the physical health QoL (PH-QoL) domains. Physical and mental QoL also correlated significantly with presence of pain (rs : -0.39 and -0.42, respectively) and number of autonomic symptoms (rs : -0.39 and -0.30, respectively). Social support independently predicts MH-QoL when controlling for age, gender, pain, and the Toronto Clinical Neuropathy Score (TCNS; p < 0.0001). TCNS and gender are independently related to PH-QoL (p < 0.05). This study demonstrates that improved social support serves as an independent predictor of MH-QoL when controlling for age, gender, pain, and severity of neuropathy. Future studies examining the effects of improving social support on QoL in patients with polyneuropathy are recommended., (© 2013 Peripheral Nerve Society.)

Peripheral neuropathy (PN) is a common impairment which may impact upon quality of life (QoL). Neuropathic pain (NeP) occurs in up to 50% of patients with PN. We hypothesized that disability and impaired quality of life resulting from PN is primarily associated with presence of NeP. Our aim was to determine using prospectively identified PN patients presenting to a tertiary care neuromuscular clinic if presence of NeP (PN+NeP) had greater impact upon QoL than with absence of NeP (PN-NeP). A second aim was to identify if QoL varied based upon etiology of PN. We analyzed neuropathy severity (Toronto Clinical Neuropathy Score (TCSS)), pain quantity and quality (Visual Analogue Scale (VAS) pain score, Brief Pain Inventory (BPI)), QoL and health status measures (EuroQol Instrument 5 Domains (EQ-5D), Medical Outcomes Sleep Study Scale (MOSSS), Hospital Anxiety and Depression Scale (HADS), Short Form 36 Health Survey (SF-36)) and Health Assessment Questionnaire (HAQ) to determine impact of NeP. Although both cohorts were epidemiologically similar and had similar severity of PN, PN+NeP patients had considerably greater impairment for QoL, sleep efficacy, and features of anxiety and depression, leading to substantially greater health care resources utilization when compared to PN-NeP patients. The magnitude of NeP severity was the only explaining variable for increased impact upon QoL measures and diminishing overall wellbeing. Our results confirm that NeP is a primary indicator for worsening QoL and diminished overall wellbeing in PN patients. The etiology of PN did not influence levels of NeP-related compromise of QoL. Further studies are needed to determine optimal methods for management of PN+NeP patients subjected to a significant physiological, psychological and functional burden., (Copyright © 2011 European Federation of International Association for the Study of Pain Chapters. Published by Elsevier Ltd. All rights reserved.)

Purpose: The prevalence of restless legs syndrome (RLS) ranging from 6.6% to 83% has been reported in different case series. The pathophysiology of RLS in uremia is still unclear. The aim of this study was to assess the frequency of RLS in the hemodialysis patients and to explore depression and associated detrimental impact on quality of life., Methods: The diagnosis of RLS was made using the criteria of the International Restless Legs Study Group. The polyneuropathy was diagnosed according to clinical signs and symptoms. Each subject completed three questionnaires: Beck Depression Inventory, Short Form-36 Quality of Life Scale, and the Epworth Sleepiness Scale (ESS). Biochemical parameters including hemoglobin, serum ferritin, and Kt/V index were collected. The data of the patients with and without RLS or clinical polyneuropathy (PNP) were compared., Results: There were 41 male and 40 female patients. RLS, PNP, and high ESS scores were seen in 12%, 47%, and 7% of hemodialysis patients, respectively. Beck depression inventory scores were high in patients with RLS when compared to those without (28.9 ± 9, p = 0.007). General health score in the RLS patients, physical functioning score in the PNP patients were significantly lower (GH, 20.7; p = 0.036; PF, 10.5; p < 0.005). We did not observe any association between hemoglobin, ferritin levels, and Kt/V index in the RLS patients. However, Kt/V index in the PNP patients were low., Conclusions: The frequency of RLS in hemodialysis patients was lower than that reported from similar studies in other countries. The presence of RLS and PNP in hemodialyzed patients negatively affects quality of life, and RLS contributes to occurrence of depression.

Purpose: To evaluate the quality of life (QOL) in patients with cryptogenic polyneuropathy., Method: Two validated instruments (SF-36 and EQ-5D) were sent to 86 patients with a 72% response rate (44 men, 18 women). As reference, 2721 individuals (1292 men, 1429 women; 59% response rate) from the general population responded to the same QOL instruments., Results: Compared to the general population, QOL was significantly more affected in patients with polyneuropathy concerning motor functions, with 42% of the patients reporting problems with walking, 7% having difficulties with washing and dressing, and 31% having problems with usual activities (work, study, household work, and family or leisure activities). The EQ-5D results showed that 85% of the patients were suffering from pain compared to 56% of the general population. Mental health was preserved among patients with polyneuropathy. Mobility was declining with increasing age in patients, but was not affected by disease duration., Conclusions: Our study showed that patients with cryptogenic polyneuropathy have a lower QOL compared to the general population, although mental health scores did not differ between the groups. This information may be helpful when explaining the disease and its impact on newly diagnosed patients., (© 2011 Informa UK, Ltd.)

Background: It is well known that patients with uremia, as well as patients with diabetes mellitus, develop polyneuropathy., Objectives: The signs of polyneuropathy in diabetic and nondiabetic patients on continuous ambulatory peritoneal dialysis (CAPD) and their relation with age, duration of dialysis, biochemical parameters, dialysis adequacy, and health-related quality of life (HRQOL) were analyzed in the present study., Patients and Methods: 65 CAPD patients (37 men, age 29-85 years, duration on dialysis 3 months to 14 years) were divided into two groups: group 1 was comprised of 20 diabetic patients (mean age 50.1+/-13.2 years); group 2 was comprised of 45 nondiabetic patients (mean age 62.3+/-9.7 years). Biochemical parameters, dialysis adequacy, and clinical signs were determined. Motor conduction velocity on the peroneal and tibial nerves and sensitive conduction velocity on the sural nerve were measured. The Kidney Disease Quality of Life Short Form (KDQOL-SF) was used to measure the CAPD patients' self-assessment of functioning and well-being using 4 component scores: physical component summary (PCS), mental component summary (MCS), kidney disease target issues, and patient satisfaction., Results: Subjective symptoms were more intense in the diabetic patients and correlated with changes in peroneal and tibial distal motor latency (DML). Diabetic patients were significantly younger, had lower creatinine and higher glucose levels, and all analyzed pathological neurophysiological parameters were higher. Nondiabetic patients had prolonged latency of the F-wave on the peroneal nerve and the tibial nerve and reduced sensitive conduction velocity on the sural nerve. Significant correlations were found between the analyzed neurophysiological parameters and duration of dialysis and diabetes, glucose concentration, and dialysis adequacy in diabetic patients, and between neurophysiological parameters and age and dialysis adequacy in nondiabetic patients. Analysis of the 4 component scores of the KDQOL-SF revealed that diabetic patients had significantly better scores for PCS and MCS, which can be explained by their younger age. Patient satisfaction was worse in diabetic patients and correlated with duration of diabetes. In addition, significant correlations were established between PCS, MCS, and tibial DML (late neuropathic changes) in diabetic patients, and between MCS and tibial F-wave (early neuropathic changes) in nondiabetic patients., Conclusion: Polyneuropathy was significantly worse in diabetic than in nondiabetic patients on CAPD. DML on the tibial nerve correlated with glucose concentration, dialysis adequacy, PCS, and MCS in diabetic patients, whereas in nondiabetic patients, dialysis adequacy and azotemia correlated with F-waves on the peroneal nerve and the tibial nerve but MCS only with F-wave on the tibial nerve.

Painful polyneuropathy is a common neuropathic pain condition. The present study describes health-related quality of life (HRQL) in a sample of patients with painful polyneuropathy of different origin and the possible predictive role of HRQL for analgesic effect. Ninety-three patients with a diagnosis of painful polyneuropathy were included in the analysis. Data were obtained from three randomised, placebo-controlled cross-over studies testing the effect of different drugs on polyneuropathic pain (St. John's wort, venlafaxine/imipramine and valproic acid). Patients completed a HRQL questionnaire (SF-36) after a drug-free baseline period and at the end of each treatment period. At baseline, all eight SF-36 scores were lower than in the normal population. No significant differences were found between SF-36 scales during placebo and treatment with valproic acid and St. John's wort. Those two drugs had not shown a pain relieving effect in former analysis. The SF-36 scale of bodily pain (BP) was improved by venlafaxine treatment (p=0.023). General health (GH) and vitality (VT) were improved under treatment with imipramine (GH: p=0.006, VT: p=0.015). In a multivariate logistic regression analysis, baseline SF-36 scores predicted subsequent response to pharmacological treatment. Results show an impaired HRQL in painful polyneuropathy and suggest that HRQL may predict response to analgesic treatment.

"Quality of life" (QOL) measurement reflects the impact of a disease on the daily life of a patient, and this can be used as an outcome measure in clinical trials. QOL measurements are rarely used in patients with neuromuscular disease. The aim of this study was to determine whether QOL is reduced in chronic polyneuropathy, whether there is a relationship between QOL and objective measures of disease severity, and whether measuring QOL is a useful addition to the assessment of severity of polyneuropathy. We measured QOL in 90 patients with chronic axonal polyneuropathy (33 with hereditary motor and sensory neuropathy type II and 57 with chronic idiopathic axonal polyneuropathy) using the RAND 36-item Health Survey questionnaire (RAND-36). We compared the results with the QOL of a reference population, with summed motor and sensory scores, and with the Rankin scale for handicap. Patients had worse scores than the reference population on seven of eight areas of the RAND-36. Patients with both low motor and low sensory scores rated lower in physical and emotional areas than less impaired patients. A low Rankin score was related only to physical domains. We conclude that in patients with chronic axonal polyneuropathy the severity of disease can be assessed with a general QOL instrument, and that this provides additional information, particularly on areas related to emotional and social functioning.