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.

Manual Feeding Device Experiences of People With a Neurodisability.

OBJECTIVE: Neurological bilateral upper limb weakness can result in self-feeding difficulties and reliance on care providers. Mealtimes become time consuming and frustrating. In this exploratory inquiry, we examined the experiences of users of a feeding device. METHOD: Semistructured interviews were either conducted by telephone or administered via email to explore quality of life, changes to independence, benefits and limitations, and psychological impact of the equipment. RESULTS: Thematic analysis gave rise to five themes: independence and positivity, emotions, impact on family and social life, equipment functionality, and motivation. CONCLUSION: This exploratory inquiry has contributed new qualitative evidence to the knowledge and understanding of users' experiences of a manual feeding device. Users reported that the need for assistance was reduced and that their quality of life, independence, and freedom improved. Time and resources savings for the family, care providers, and staff appeared to result in a more equal relationship between user and care provider.

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.

Extended Occupational Therapy Reintegration Strategies for a Woman With Guillain-Barré Syndrome: Case Report.

This case report describes a unique long-term functional recovery process to promote successful community reintegration for a woman with Guillain-Barré syndrome (GBS), a rare autoimmune disease. Her main symptoms were very limited mobility and depressive symptoms due to the unknown cause of and cure for the illness. Holistic occupational strategies helped the client stabilize her emotional state, create a safe home environment, improve a communication method, increase physical activity, and promote social participation. Participation in a fall prevention clinical trial lowered her risk of falling; at 9 mo, she reached 75% of the maximum Social Integration score; at 13 mo, she reached near-normal level for activities of daily living (ADLs) and her fastest time for the Timed Up and Go test; and at 2 yr, she achieved a 100% score in instrumental ADLs. For community integration of clients with GBS, a comprehensive strategic self-management approach should be prescribed for long-term recovery.

Chronic Bickerstaff's encephalitis with cognitive impairment, a reality?

BACKGROUND: Bickerstaff's encephalitis (BE) is an acute post-infectious demyelinating disease with albuminocytological dissociation. A chronic form has rarely been described previously. CASE PRESENTATION: A 44-year-old man was hospitalized for drowsiness, cognitive complaint limb weakness, ataxia and sensory disturbance after diarrhea. Neuropsychological evaluation showed slowing, memory and executive function impairment, while analysis of the CSF showed albuminocytological dissociation. Immunologic tests showed positive anti-ganglioside antibodies (anti-GM1 IgM, anti-GD1a IgG and anti-GD1b IgM). Brain MRI was normal but SPECT showed bilateral temporal and frontal hypoperfusion. Outcome under immunoglobulin treatment (IVIG) was favorable with an initial improvement but was marked by worsening after a few weeks. Consequently, the patient was treated with IVIG every 2 months due to the recurrence of symptoms after 6 weeks. CONCLUSION: This case raises the question of the existence of a chronic form of BE with cognitive impairment, in the same way as chronic inflammatory demyelinating polyneuropathy is considered to be a chronic form of Guillain-Barré syndrome.

Health-related quality of life in Guillain-Barré syndrome patients: a systematic review.

Guillain-Barré syndrome (GBS) encompasses a broad spectrum of health-related quality of life (HRQL) determinants, including mobility, fatigue, pain, and depression. We systematically reviewed the literature on functional outcome domains in which GBS patients experience limitations in the short and long terms and evaluated determinants of HRQL in GBS patients. MEDLINE and EMBASE were systematically searched by two independent reviewers for articles covering HRQL data of GBS patients. Of 730 abstracts screened, 17 articles covering data of 14 studies matched the selection criteria. The included articles showed that many GBS patients experienced physical limitations, even years after the acute phase of the disease, while results were inconsistent for perceived levels of pain, fatigue, and general mental well-being. Only three papers covered HRQL assessments at more than one time point, generally showing large improvements in HRQL in the first year after GBS onset, but not thereafter. We appraised the methodological quality of included studies using a 13-item checklist; none of the articles fulfilled all items and only seven articles presented data on correlations between HRQL and determinants. In conclusion, the majority of studies on HRQL in GBS patients are cross-sectional and of low methodological quality. This paper provides guidance for much needed high-quality studies on patterns of patient-perceived recovery after GBS onset.

Post-traumatic stress symptoms in Guillain-Barré syndrome patients after prolonged mechanical ventilation in ICU: a preliminary report.

Thirty percent of Guillain-Barré syndrome (GBS) patients require mechanical ventilation (MV) in intensive care unit (ICU). Post-traumatic stress disorder (PTSD) is found in ICU survivors, and the traumatic aspects of intubation and MV have been previously reported as risk factors for PTSD after ICU. Our objective was to determine long-term PTSD or post-traumatic stress symptoms (PTSS) in GBS patients after prolonged MV in ICU. We assessed GBS patients who had MV for more than 2 months. PTSD was assessed using Horowitz Impact of Event Scale (IES), IES-Revisited (IES-R), and the Post-traumatic CheckList Scale; functional outcome using Rankin and Barthel scales; quality of life (QoL) using Nottingham Health Profile (NHP) and 36-Item Short Form Health Survey (SF-36) and depression using Hospital Anxiety and Depression Scale (HAD) and Beck questionnaire. Thirteen patients could be identified and analyzed. They had only mild disability. They were neither anxious nor depressed with an anxiety HAD at 5 (4-11.5), a depression HAD at 1 (0-3.5) and a Beck at 1 (0-5). QoL was mildly decreased in our population with a NHP at 78.5 (12.8-178.8) and mild decreased SF-36. Compared with the French population, the SF-36 sub-categories were, however, not statistically different. Twenty-two percentage of our 13 patients had PTSD and PTSS with a Horowitz IES at 12 (2-29), and an IES-R at 16 (2-34.5). Although severe GBS patients requiring prolonged MV had good functional recovery and no difference in QoL, they had a high incidence of PTSS.

Long-term outcome of Guillain-Barré syndrome in children.

The objective of this study is to determine the long-term outcome and consequences of Guillain-Barré syndrome (GBS) in children. This is an observational cross-sectional cohort study of children diagnosed with GBS (0-18 years old) at the Sophia Children's Hospital in Rotterdam from 1987 to 2009. All patients were invited for a structured interview, questionnaires, and full neurologic exam to record their current clinical condition focused on complaints and symptoms, neurological deficits, disabilities, behavior, and quality of life. Thirty-seven patients participated, 23 were now adults, with a median age of 20 years (range 4-39 years) and a median follow-up time of 11 years (range 1-22 years). Residual complaints were reported by 24 (65%) patients, including paresthesias (38%), unsteadiness of gait in the dark (37%), painful hands or feet (24%), and severe fatigue (22%). Four patients had severe neurological deficits, including facial diplegia and limb weakness. Two patients had had a recurrence of GBS. In 10 patients (26%), GBS had a negative impact on their school career. Questionnaires identified a wide range of behavioral problems. Quality of life was below normal on the subscale vitality, and above normal on the subscales social functioning and positive emotions in the adult group. Most children show good recovery of neurological deficits after GBS, but many have persisting long-term residual complaints and symptoms that may lead to psychosocial problems interfering with participation in daily life.

Patients' experiences and perceptions of Guillain-Barré syndrome: A systematic review and meta-synthesis of qualitative research.

BACKGROUND: Guillain-Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy, with an incidence of 1-2/100,000 per year. Its severity is variable, ranging from very mild cases with brief weakness to severe paralysis, leading to inability to breathe independently, or even death. Currently there is limited evidence exploring the experiences of GBS patients. The aim of this study was to review patients' experiences and perceptions of GBS and its variants at diagnosis, discharge and during recovery, by conducting a systematic review and thematic meta-synthesis of qualitative studies of patients' experiences of GBS (and its variants). METHODS: We searched twelve electronic databases, supplemented with internet searches and forward and backward citation tracking from the included studies and review articles. Data were synthesised thematically following the Thomas and Harden approach. The CASP Qualitative Checklist was used to assess the quality of the included studies of this review. RESULTS: Our search strategy identified a total of 5,282 citations and after removing duplicates and excluding citations based on title and abstract, and full-text screening, five studies were included in the review and meta-synthesis; all included studies were considered of acceptable quality. Through constant discussions and an iterative approach, we developed six analytical themes following a patient's journey from suspecting that they had a health problem, through to being hospitalised, experiencing ongoing difficulties, slowly recovering from GBS, adjusting to their new circumstances, and re-evaluating their lives. CONCLUSIONS: Despite the variety of experiences, it was evident from all included studies that being diagnosed with and surviving GBS was a life-changing experience for all participants. TRIAL REGISTRATION: Protocol was registered (CRD42019122199) on the International Prospective Register of Systematic Reviews (http://www.crd.york.ac.uk/PROSPERO).

Psychosocial dysfunction in the first year after Guillain-Barré syndrome.

In this investigation we study the impact of Guillain-Barré syndrome (GBS) on psychological distress, depressive symptoms, and health status of patients during the first year after GBS. At 3, 6, and 12 months, patients were given the General Health Questionnaire, the Sickness Impact Profile, and the Center for Epidemiologic Studies Depression Scale. Eighty-five patients participated. Psychological distress and depressive symptoms were present but improved between 3 and 6 months. At 12 months the psychosocial health status was still impaired. Patients who perceived their physical residua to be moderately to seriously disruptive and patients with muscle ache and cramps had worse scores on all scales. It can be concluded that most of the improvement occurred in the first 6 months. Psychosocial health status, however, was still impaired at 1 year, but depressive symptoms played no role. Treatment of muscle ache and cramps, and the disruptive effect of physical residua should be seriously considered.

Disability and quality of life in Guillain-Barré syndrome - Longitudinal study.

Longitudinal health-related quality of life (QoL) data in Guillain-Barré (GBS) patients are still scarce. We, therefore, investigated health- related QoL in GBS patients from Serbia and surrounding countries during a six-month follow-up period, and analyzed its association with patients' disability. Our study comprised 74 adult patients diagnosed with GBS from May 2017 until May 2018 in seven tertiary healthcare centers. Health-related QoL was investigated using the SF-36 questionnaire, and compared with functional disability assessed by the GBS disability scale (GDS). Tests were performed at day 14, day 28, month 3 and month 6 from disease onset. GDS and SF-36 scores improved over time (p < 0.01). GDS scores were different at all four time points, while SF-36 did not differ between day 14 and day 28. Pooled SF-36 scores (especially physical ones) correlated with pooled GDS scores, except for Bodily Pain and Role Emotional scores. We found that GDS score at day 14 was an independent predictor of GDS score at month 6 (ß = +0.52, p < 0.01), while SF-36 score at day 14 was an independent predictor of SF-36 score at month 6 (ß = +0.51, p < 0.01). Neurologists should look not only on disability but also on QoL in GBS patients, since these two measures provide us with important complementary items of information.

Evaluation of Post Guillain-Barre Syndrome (GBS) Patients: A Cross Sectional Study.

Guillain-Barre syndrome (GBS) is related with significant morbidity and also mortality. Little is known about the long term outcome of GBS patients who survived. The objective of this study is to determine the lasting outcome and consequences of GBS patients. This is a cross-sectional study of patients who diagnosed GBS and managed at the Intensive Care Unit of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from January 2004 to December 2017. All survived patients were invited for a structured interview, questionnaires, and full neurologic exam to record their current clinical condition focused on complaints and symptoms, neurological deficits, disabilities, behaviour, and quality of life. Thirty-eight patients participated, with a median age of 20 years (range 4-39 years) and a median interviewed time of 7 years (range 1-13 years). Residual complaints were reported by 24(63%) patients, including paresthesias (10.5%), unsteadiness of gait (37%), painful hands or feet (29%), and severe fatigue (13%). Questionnaires identified a wide range of behavioural problems. Most Patients showed good recovery of neurological deficits after GBS, but many have persisting long-term residual complaints and symptoms that may lead to psychosocial problems interfering with participation in daily life.

The long-term functional status in patients with Guillain-Barré syndrome.

BACKGROUND AND PURPOSE: The purpose of this study was to analyse the long-term impact of Guillain-Barré syndrome (GBS) on quality of life, and the relationship between clinical variables at disease onset and symptoms at follow-up to general health status. METHODS: Forty-two GBS patients were examined at median 6 years after disease onset and were compared with 50 healthy controls. The fatigue severity scale (FSS), visual analogue scale (VAS) for pain, disability rating index (DRI) and medical outcome study 36-item short-form health status scale (SF-36) were applied. Variables at onset and symptoms at follow-up were correlated with outcome measurements in GBS. RESULTS: VAS [2.9 (SD 3.3) vs. 1.5 (SD 1.9); P = 0.01] and DRI [2.5 (SD 2.1) vs. 1.0 (SD 1.5); P < 0.001] were significantly higher in patients with GBS, compared with healthy controls. Decreased physical functioning and general health were found on SF-36. Differences between GBS patients with shorter (<6 years) and longer (> or =6 years) follow-up after onset were not found. CONCLUSIONS: Relatively independent from various variables at onset, patients with GBS seem to have a reduced quality of life and functioning, and the distress seems to have become persistent after the first few years with improvement following the acute disease.

Falling ill with Guillain-Barré syndrome: patients' experiences during the initial phase.

OBJECTIVE: Research describing the personal experiences of Guillain-Barré syndrome (GBS) is limited, but is important for identifying the patients' need of support. The aim of this study was to describe experiences of falling ill with GBS, with the focus on the onset of disease, the diagnosis and the illness progress during hospital care. METHODS: The study included 35 persons, 20-78 years old. They were interviewed 2 years after the onset of GBS. The interviews were analysed using qualitative content analysis. RESULTS: The onset was described as either an incomprehensible, prolonged, increasing deterioration with puzzling sensations or as a frightening, rapid onset with a sudden loss of body control. The majority of the persons relied heavily on the reassurance of a positive prognosis, and expressed immense confidence in being able to recover. During the early phase at the hospital, a rapid and steady course of improvement inspired hope in many persons. In contrast, even in this early phase of hospital care some individuals expressed doubts of a slow recovery. Feelings of fear and insecurity were evident when losing body functions, thus causing helplessness. Sensations of pain, numbness and lost body image increased their vulnerability. Half of the ventilator-treated persons expressed vivid memories of scary hallucinations. CONCLUSION: The onset is characterized by an incomprehensible bodily deterioration or a frightening, rapid paralysis. In the initial phase, there is hope for recovery, which for many individuals is reinforced by a steady recovery. In contrast, early psychosocial support may be necessary for some persons with an alarmingly slow recovery.

Neuropsychological/-psychiatric deficits in immune-mediated neuropathies.

Albeit common, neuropsychological/psychiatric deficits in patients with autoimmune-mediated neuropathies have not received much attention; however, they can impose a considerable clinical problem. Especially in intensive-care treated Guillain Barré syndrome (GBS) severe psychosis may occur which may go unrecognised due to the severity of the neurological motor deficits. Fatigue may result in a major restriction of quality of life long after the acute phase of GBS and is also a major debilitating factor in chronic inflammatory neuropathies. Symptomatic treatment remains largely empirical.

Analysing the favourable effects of physical exercise: relationships between physical fitness, fatigue and functioning in Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy.

OBJECTIVE: To elucidate the effects of physical exercise in severely fatigued patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, and to clarify the mutual relationships between 5 domains studied in these patients: physical fitness, fatigue, objectively measured actual mobility, perceived physical functioning, and perceived mental functioning. DESIGN: Case series. SUBJECTS/PATIENTS: Twenty patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. METHODS: The patients undertook a 12-week physical exercise program. Relationships between domains were studied in the change scores, and additionally in the baseline data of patients. The percentage of significant relationships between each pair of domains was determined. RESULTS: In the change scores, a small percentage of significant relationships was found between the physical fitness domain and the other 4 domains (2/30, 7%). A higher percentage of significant relationships was found between the domains perceived mental functioning and actual mobility (44%), perceived mental functioning and perceived physical functioning (44%), and between fatigue and perceived physical functioning (33%). Generally, similar patterns were found in the baseline data. CONCLUSION: Changes in fatigue, actual mobility and perceived functioning seem not to be influenced by changes in physical fitness. This study stresses the presence and importance of additional effects of a physical training program, not directly related to increasing fitness.

Getting closer to patients: the INCAT Overall Disability Sum Score relates better to patients' own clinical judgement in immune-mediated polyneuropathies.

OBJECTIVE: To determine which widely used disability measure in Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) shows the strongest association with patients' rating scores. METHODS: Five disability scales and the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36) were assessed serially in 20 patients with newly diagnosed GBS (n = 7) or CIDP (n = 13). Also at each visit, the patient's condition was self-assessed as being worse, unchanged or better. Longitudinal regressions were carried out to determine the association between disability scales (independent variables) and SF-36 and patients' rating scores (dependent variables). RESULTS: Higher associations with the SF-36 were found in the Overall Disability Sum Score (ODSS) than other disability measures. A higher correlation with ODSS changes was also found in the rating scores of the patients. CONCLUSION: In addition to literature findings, higher associations were found between Inflammatory Neuropathy Cause and Treatment Group ODSS and outcome assessed from patients' perceptions in immune-related polyneuropathies than in other commonly used disability scales.