Fatigue in chronic inflammatory demyelinating polyneuropathy.

INTRODUCTION: In this study we aimed to better understand fatigue in chronic inflammatory demyelinating polyneuropathy (CIDP) as it relates to disease activity status. METHODS: Patients with probable or definite CIDP were stratified into active CIDP or CIDP in remission. Assessments of fatigue, physical impairment, disability, sleepiness, sleep quality, and depression were collected. RESULTS: Of the 85 patients included in the study, 46 (54%) had active disease, whereas 39 (46%) were in remission. Fatigue was substantial in both groups, but was more severe in the active group. Use of sedating medications was a major contributor to fatigue. Sleep quality was poor in both groups, whereas depression more commonly affected those with active CIDP. Inflammatory Neuropathy Cause and Treatment disability, poor sleep quality, and higher level of depression had the greatest effect on fatigue severity. DISCUSSION: Fatigue is common in CIDP regardless of the disease activity state. Minimizing sedating medications, improving sleep quality, and managing depression may improve CIDP-associated fatigue.

Clinical correlates of fatigue in chronic inflammatory demyelinating polyneuropathy.

INTRODUCTION: Fatigue is a recognized manifestation of immune-mediated neuropathies, but its causes and implications are unclear. In this study, we explored the correlates of fatigue among a number of clinical parameters in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) to better understand potential contributing factors and resulting consequences. METHODS: Twenty-six clinically stable patients with CIDP underwent assessments of motor and sensory function, disability, quality of life, and depression and anxiety. Experienced fatigue was assessed by using the Rasch-built Fatigue Severity Scale and the Checklist of Individual Strength. RESULTS: The two fatigue scales provided evidence of significant intercorrelation. Only depression scores and grip strength predicted experienced fatigue levels in multivariate regression analyses. Fatigue scores inversely correlated with strength, sensory, disability, and quality of life scores. DISCUSSION: Experienced fatigue is likely multifactorial in CIDP, with both physical and neuropsychiatric components. Fatigue is associated with lower functional and quality-of-life outcomes in CIDP.

Quality of life in chronic inflammatory demyelinating polyneuropathy patients treated with subcutaneous immunoglobulin.

BACKGROUND: Subcutaneous immunoglobulin (SCIG) is effective treatment of chronic inflammatory demyelinating polyneuropathy (CIDP). Quality of life (QoL) increases following switch from intravenous administration to SCIG, but its correlation with clinical functioning is sparsely studied. AIMS OF THE STUDY: The aim of this study is to evaluate the correlation between QoL and clinical functioning in CIDP patients treated with SCIG. METHODS: Danish patients with CIDP with a disease duration <10 years and currently treated with SCIG were eligible for inclusion. QoL was assessed with EQ-5D-5L and disability by the Overall Disability Sum Score (ODSS) and Rasch-built Overall Disability Scale (RODS). Gait performance was evaluated by a 40-meter-walk test (40-MWT) and a 6-spot-step test (6-SST) along with assessment of muscle strength (Medical Research Council score [MRC]). Correlations between QoL and the measured scores were calculated. RESULTS: Of 92 eligible patients, 44 were included. QoL on the visual analogue scale (VAS) was 65% (range: 15-90) of the level of healthy controls (P = .03) and correlated to impaired gait function by 40-MWT and 6-SST. QoL correlated to RODS and ODSS, whereas there was no correlation with the MRC score. CONCLUSIONS: In SCIG treated CIDP patients QoL is reduced and correlates to gait performance and disability.

Evaluation of Quality of Life in Patients With Chronic Inflammatory Demyelinating Polyneuropathy in Iran.

OBJECTIVES: In addition to the physical disability in chronic inflammatory demyelinating polyneuropathy (CIDP), various aspects of quality of life (QoL) are affected by the disease. Our goal was to evaluate the QoL in Iranian patients with CIDP and to study the association of clinical and demographic factors with QoL parameters. METHODS: Twenty-six Iranian patients with CIDP were asked to complete a standardized Persian version of SF-36, and the association of demographic and functional parameters with QoL parameters was assessed. RESULTS: Sex, living place, and income had no significant effect on QoL. Mean physical composite score was 37.7 ± 9.4, and mean mental composite score was 37.2 ± 15.2 that was lower than similar studies. There was a positive correlation between QoL and muscle strength of different muscle groups [hand (r = 0.41, P = 0.04); hip (r = 0.44, P = 0.02); and foot (r = 0.41, P = 0.04); total manual muscle testing (r = 0.46, P = 0.02)] and a significant negative correlation between the duration of illness and mean physical composite score (r = -0.61, P = 0.00). CONCLUSIONS: Our patients achieved low scores in both the physical and mental aspects of QoL compared with other studies. We found a significant correlation between the physical domain of the SF-36 score and muscle strength of the hand, hip, and foot muscle group and with total manual muscle testing, suggesting that physical disability is the most crucial factor affecting QoL. Also, there was a significant negative association between the duration of the disease and the physical domain.

Functioning and quality of life in patients with neuropathy associated with anti-MAG antibodies.

Although anti-myelin-associated glycoprotein (MAG) antibody neuropathy is reported as a slowly progressive disease, it can lead to significant disability and impairment of health-related quality of life (HR-QoL) and social participation. The aim of this cross-sectional study was to evaluate the functioning and HR-QoL determinants in 67 patients with anti-MAG neuropathy in terms of the International Classification of Functioning, Disability, and Health (ICF). Evaluations included: Medical Research Council (MRC) sum score, Sensory Modality Sum score (SMS), Berg balance scale (BBS), Fatigue Severity Scale (FSS), Visual Analogue Scale (VAS) for pain, 9-Hole Peg Test (9-HPT), 6-min Walk Distance (6MWD), Impact on Participation and Autonomy (IPA) and the physical component score (PCS) and mental component score (MCS) of the short-form-36 health status scale (SF-36) HR-QoL measure. In the regression models, 6MWD was the most reliable predictor of PCS, explaining the 52% of its variance, while the strongest determinants of 6MWD were BBS and FSS, explaining the 41% of its variance. Consistently, VAS and BBS were good predictor of PCS, explaining together 54% of its variance. FSS was the most reliable determinant of MCS, explaining 25% of its variance. SMS and MRC were not QoL determinants. The results of our study suggest that 6MWD and FSS might be considered as potential meaningful outcome measures in future clinical trials. Furthermore, neurorehabilitation interventions aimed at improving balance and walking performance, fatigue management, and specific pain relief therapy should be considered to ameliorate participation in social life and HR-QoL in anti-MAG neuropathy patients.

Costs of illness in chronic inflammatory demyelinating polyneuropathy in Germany.

INTRODUCTION: Cost of illness studies are essential to estimate societal costs of chronic inflammatory demyelinating polyneuropathy (CIDP) and identify cost-driving factors. METHODS: In total, 108 patients were recruited from 3 specialized neuroimmunological clinics. Costs were calculated for a 3-month period, including direct and indirect costs. The following outcomes were assessed: inflammatory neuropathy cause and treatment disability scale, Mini-Mental State Examination, Beck Depression Inventory, Charlson comorbidity index, EuroQol-5D, World Health Organization quality of life instrument, and socioeconomic status. Univariate and multivariate analyses were applied to identify cost-driving factors. RESULTS: Total quarterly costs were €11,333. Direct costs contributed to 83% of total costs (€9,423), whereas indirect costs accounted for 17% (€1,910) of total costs. The cost of intravenous immunoglobulin (IVIg) was the main determinant of total costs (67%). Reduced health-related quality of life and depressive symptoms were identified as independent predictors of higher total costs. DISCUSSION: CIDP is associated with high societal costs, mainly resulting from the cost of IVIg treatment. Muscle Nerve 58: 681-687, 2018.

Employment status of patients with chronic inflammatory demyelinating polyradiculoneuropathy.

It has been previously shown that patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are unemployed or retired have worse quality of life. The aim of this study was to assess predictors of early retirement in CIDP. One hundred five patients with CIDP were included. Following measures were used: questionnaire on employment status, Medical Research Council Sum Score, INCAT disability score, Beck Depression Inventory, and Krupp's Fatigue Severity Scale. At the moment of testing, 2% of patients were students, 15% were employed, 9% were unemployed due to CIDP, 9% were unemployed but not due to CIDP, 28% were retired early due to disability caused by CIDP, and finally 37% were in old-age pension. Mean age when patients retired due to CIDP was 50 ± 8 years. Mean time from CIDP onset to retirement was 2.7 ± 2.3 years. Older age at onset, lower education, and more severe weakness at the time of diagnosis were significant predictors of early retirement due to CIDP. Retired patients were 12 times more likely to suffer from depression, compared to employed patients (OR = 12.2, 95% CI = 1.41-100, P < 0.01), and eight times more likely to have fatigue (OR = 8.2, 95% CI = 1.89-35.82, P < 0.01). Older patients with lower education and more severe weakness at the time of diagnosis were most likely retired due to CIDP. Early retirement was associated with depression and fatigue. Therefore, maintaining employment should be an important aim in the management of CIDP patients.

Home versus hospital immunoglobulin treatment for autoimmune neuropathies: A cost minimization analysis.

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.

Quality of life in inflammatory neuropathies: the IN-QoL.

BACKGROUND: No consensus exists which quality of life (QoL) measure should be used in patients with inflammatory neuropathies. Moreover, most QoL measures are ordinal-based scales with their known deficiencies. OBJECTIVES: To establish a new disease-specific interval-based QoL questionnaire in inflammatory neuropathies (IN-QoL) using the Rasch model and evaluate its scientific properties (validity, reliability and responsiveness). METHODS: 264 patients with inflammatory neuropathies completed six commonly used QoL questionnaires. The obtained data were stacked and subjected to Rasch analysis. Responsiveness was determined by using the concept of minimum clinically important differences related to varying individually obtained SEs (responsiveness definition: MCID-SE≥1.96 after 1-year follow-up compared with baseline). RESULTS: The IN-QoL fulfilled all Rasch's model requirements with high internal reliability values (patient separation index of 0.94), except being multidimensional. Additional factor analysis resulted in two (functional and mental) subsets that were unidimensional on their own. The IN-QoL showed good correlation with the EuroQol-health quality visual analogue scale (EQ-VAS) (Spearman's rho 0.72). It demonstrated acceptable responsiveness in patients with Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), as did the EQ-VAS. In patients with monoclonal gammopathy-related neuropathy and multifocal motor neuropathy, hardly any changes were seen over time. CONCLUSION: The IN-QoL questionnaire fulfils modern clinimetric requirements and correlates strongly with a patient's self-assessment of their own quality of health, while also showing responsiveness in patients with GBS and CIDP. We propose using the IN-QoL and the EQ-VAS for assessing the QoL of patients with inflammatory neuropathies in future studies.

Quality of life predictors in patients with chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic disease which can lead to many functional impairments, and like most other chronic disorders it might significantly affect quality of life (QoL). Information about QoL in patients with CIDP from developing countries is still lacking. We, therefore, sought to complete these data mosaic by investigating QoL in patients with CIDP from Serbia and surrounding countries. Our study comprised 106 patients diagnosed with CIDP. QoL was investigated using the Serbian version of the SF-36 questionnaire. The Medical Research Council 0-5 point scale, INCAT motor and sensory scores, Krupp's Fatigue Severity Scale, and Beck Depression Inventory were also used. Factors that significantly correlated with SF-36 total score in univariate analysis were included in the multiple linear regression analysis. Physical domains of the SF-36 were more affected than mental, and the overall score was 56.6 ± 25.4. Significant predictors of worse SF-36 score in our patients with CIDP were severe fatigue (ß = - 0.331, p < 0.01), higher INCAT motor score (ß = - 0.301, p < 0.01), depression (ß = - 0.281, p < 0.01), being unemployed/retired (ß = - 0.188, p < 0.05), and shorter duration of CIDP (ß = + 0.133, p < 0.01). QoL was reduced in CIDP patients, especially in physical domains. Patients with presence of fatigue and depression, with more severe motor disability, unemployed/retired ones, and those with shorter duration of the disease need special attention of clinicians since they could be at higher risk to have worse QoL.

Fatigue, Pain, Anxiety and Depression in Guillain-Barré Syndrome and Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

BACKGROUND: In the clinical evaluation of patients with Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), scant attention is paid to symptoms such as fatigue, pain and anxiety/depression. We aimed at addressing seminal studies that focused on the burden of these symptoms and their impact on quality of life (QoL) in these conditions. SUMMARY: Fatigue, pain, and anxiety/depression are increasingly being recognized in patients with GBS and CIDP, although their pathophysiological provenance remains unknown. Fatigue and pain are significant in terms of prevalence and intensity, may be a presenting symptom, and can persist for years after apparent functional recovery, suggesting residual injury. Anxiety/depression has also been examined although studies are limited. Despite their negative impact on QoL, the long-term dynamics of these symptoms in patients with GBS and particularly CIDP receiving therapy in routine clinical practice have not been systematically evaluated. Such observations formed the basis for the ongoing (GAMEDIS) studies evaluating the effect of Gamunex on fatigue and depression in patients with CIDP, of which some preliminary data are presented. KEY MESSAGES: Strength and sensory deficits are the main areas of focus in patients with GBS and CIDP, but they do not explain the total reduction in QoL, suggesting the possible role of other complaints. A more comprehensive approach to patient care demands that factors such as pain, fatigue and anxiety/depression receive greater attention. The non-interventional GAMEDIS studies are expected to provide valuable insight into the long-term effectiveness of Gamunex in everyday practice.

CIDP diagnostic pitfalls and perception of treatment benefit.

OBJECTIVE: We aimed to explore the diagnosis and misdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) and to identify pitfalls that erroneously lead to a misdiagnosis. METHODS: A retrospective study of 59 consecutive patients referred with a diagnosis of CIDP was performed. Patients were classified as having or not having CIDP according to European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria. Diagnostic and treatment data were compared in the 2 groups. RESULTS: Forty-seven percent of patients referred with a diagnosis of CIDP failed to meet minimal CIDP diagnostic requirements. All misdiagnosed patients who satisfied EFNS/PNS clinical criteria would be considered atypical as defined by the EFNS/PNS. CSF cytoalbuminologic dissociation was present in 50% of those without CIDP, although protein elevations were generally mild. Nerve conduction studies in patients without CIDP were heterogeneous, but generally showed demyelinating features better explained by a process other than CIDP. Patients frequently reported improvements after being treated with immunotherapy, even if the CIDP diagnosis was incorrect. CONCLUSIONS: CIDP misdiagnosis is common. Over-reliance on subjective patient-reported perception of treatment benefit, liberal electrophysiologic interpretation of demyelination, and placing an overstated importance on mild or moderate cytoalbuminologic dissociation are common diagnostic errors. Utilization of clear and objective indicators of treatment efficacy might improve our ability to make informed treatment decisions.

Health-related quality of life in chronic inflammatory neuropathies: a systematic review.

Chronic inflammatory neuropathies represent a heterogeneous group of disorders which affect patients' functional status and quality of life. We conducted a systematic review of the scientific literature on the effects of both disease and treatment interventions on health-related quality of life (HRQoL) in this patient population. The available data are limited, as few studies have systematically considered HRQoL in patients with inflammatory neuropathies. Moreover, in treatment trials, HRQoL measures have exclusively been used as secondary outcome measures. There is some evidence suggesting that baseline pre-treatment HRQoL reports are lower in patients with chronic inflammatory neuropathy than in age and gender-matched controls. Following treatment interventions, improvements in self-reported measures were consistently documented in the physical domain of HRQoL, which in turn correlated with improvements in traditional strength and functional scales. The impact of available treatments on the quality of life of patients with inflammatory neuropathies remains largely under-investigated. Interestingly, recent, although limited evidence from generic HRQoL measures may partly or completely contradict the results found with the primary, traditional outcome measures used (rituximab for anti-MAG neuropathy; immunoglobulins versus corticosteroids for chronic inflammatory demyelinating polyneuropathy). Similarly, HRQoL measures may suggest superiority, rather than equivalence, of certain drug administration methods (subcutaneous over intravenous immunoglobulins). Further research is needed to assess HRQOL in patients with untreated chronic inflammatory neuropathies in comparison to normative values, as well as precisely quantify treatment benefit. The role of both generic and disease-specific HRQoL measures in the evaluation of patients with chronic inflammatory neuropathies is also worthy of further consideration.

Chronic inflammatory demyelinating polyneuropathy: quality of life, sociodemographic profile and physical complaints.

UNLABELLED: Whereas an evaluation of quality of life and possible impacts on the mental state of a patient may help to evaluate the evolution of chronic inflammatory demyelinating polyneuropathy (CIDP), the aim of this study was to study the psychological profile of patients, and evaluate quality of life associated with the disease. METHOD: 41 patients were evaluated using a Mini-Mental State Examination (MMSE) and a Short-Form Health Survey (SF-36). RESULTS: The mean age of the patients was 50.6 years, 63.4% men. Of the participants, 65.9% had other health problems, 39% reported needing help with activities of daily living, 49% slept less than 8 hours per night, and 34.1% complained of some memory deficit. The average MMSE score was 26. Impairment of functional capacity and pain were the more important altered health states. CONCLUSION: CIDP has important social and economic impacts, owing to functional impairments that can lead to professional and personal limitations.

Chronic inflammatory demyelinating polyneuropathy: quality of life, sociodemographic profile and physical complaints / Polineuropatia inflamatoria desmielinizante cronica: qualidade de vida, perfil sociodemografico e queixas fisicas

Whereas an evaluation of quality of life and possible impacts on the mental state of a patient may help to evaluate the evolution of chronic inflammatory demyelinating polyneuropathy (CIDP), the aim of this study was to study the psychological profile of patients, and evaluate quality of life associated with the disease. Method 41 patients were evaluated using a Mini-Mental State Examination (MMSE) and a Short-Form Health Survey (SF-36). Results The mean age of the patients was 50.6 years, 63.4% men. Of the participants, 65.9% had other health problems, 39% reported needing help with activities of daily living, 49% slept less than 8 hours per night, and 34.1% complained of some memory deficit. The average MMSE score was 26. Impairment of functional capacity and pain were the more important altered health states. Conclusion CIDP has important social and economic impacts, owing to functional impairments that can lead to professional and personal limitations. .

A avaliação da qualidade de vida (QV) e dos possíveis impactos dos déficits funcionais sobre o estado mental de pacientes com polirradiculoneuropatia inflamatória desmielinizante crônica (PIDC) pode contribuir para a melhor compreensão de aspectos evolutivos da doença. A presente investigação teve como objetivo estudar as atividades da vida diária depacientes com PIDC e avaliar a sua QV. Método Foram avaliados 41 pacientes através do Mini Exame do Estado Mental (MEEM) e do inventário de saúde SF-36®. Resultados A média de idade dos participantes foi 50,6 anos, 63,4% homens. Problemas adicionais de saúde foram referidos por 65,9%: 39% relataram necessitar de ajuda para atividades de vida diária, 49% dormiam menos de 8 horas por noite e 34,1% referiam alguma dificuldade de memória. A média do MEEM foi 26. Através do SF-36 foi verificado maior prejuízo na capacidade funcional; a referência a dor foi proeminente. Conclusão A PIDC pode ter importante impacto social e econômico em decorrência dos prejuízos funcionais primários e secundários que podem levar ao afastamento do trabalho. .