What should a person with relapsing-remitting multiple sclerosis know? - Focus group and survey data of a risk knowledge questionnaire (RIKNO 2.0).

BACKGROUND: Risk knowledge is relevant to make informed decisions in multiple sclerosis (MS). The risk knowledge questionnaire for relapsing-remitting MS (RIKNO 1.0) was developed and piloted in Germany. OBJECTIVE: To produce a revised RIKNO 2.0 questionnaire using mixed methodology in a European setting. METHODS: The questionnaire was translated in seven languages. MS patient and health professional (HP) expert feedback was obtained from Germany, Italy, Estonia, Serbia, and the UK. A German web-based survey of RIKNO 2.0 compared the tool with the MS Knowledge Questionnaire (MSKQ), each one used with two versions (with/without a "don't know" DN option). RESULTS: While RIKNO 2.0 was considered difficult, it was rated as highly educational. One item was reframed, and two new items were added. The web-based German survey (n = 708 completers) showed that the DN version did not increase participation rate and did not produce significantly higher scores. Internal consistency (Cronbach alpha) without SN response was 0.73. RIKNO 2.0 scores showed normality distribution irrespective of the answering format. Item difficulty was high ranging from 0.07 to 0.79. Less than 50% of questions were answered correctly (mean 8.9) compared to 80.4% in the MSKQ (mean 20.1). Higher numeracy competency and education were significantly, albeit weakly, associated to higher scores for both RIKNO 2.0 and MSKQ. CONCLUSION: Including "don't know" options in knowledge questionnaires does not increase percentage of correct replies. RIKNO 2.0 is a complex questionnaire to be used in an educational context and studies on patient information. The tool is now available in seven languages.

Risk Knowledge in Relapsing Multiple Sclerosis (RIKNO 1.0)--Development of an Outcome Instrument for Educational Interventions.

BACKGROUND: Adequate risk knowledge of patients is a prerequisite for shared decision making but few attempts have been made to develop assessment tools. Multiple Sclerosis (MS) is a chronic inflammatory disease of young adults with an increasing number of partially effective immunotherapies and therefore a paradigmatic disease to study patient involvement. OBJECTIVE/METHODS: Based on an item bank of MS risk knowledge items and patient feedback including perceived relevance we developed a risk knowledge questionnaire for relapsing remitting (RR) MS (RIKNO 1.0) which was a primary outcome measure in a patient education trial (192 early RRMS patients). RESULTS: Fourteen of the RIKNO 1.0 multiple-choice items were selected based on patient perceived relevance and item difficulty indices, and five on expert opinion. Mean item difficulty was 0.58, ranging from 0.14 to 0.79. Mean RIKNO 1.0 score increased after the educational intervention from 10.6 to 12.4 (p = 0.0003). Selected items were particularly difficult (e.g. those on absolute risk reductions of having a second relapse) and were answered correctly in only 30% of the patients, even after the intervention. CONCLUSION: Despite its high difficulty, RIKNO 1.0 is a responsive instrument to assess risk knowledge in RRMS patients participating in educational interventions.

[A case of anti-NMDA-receptor encephalitis]. / Anti-NMDA-éntsefalit - klinicheskii sluchai

A clinical case of encephalitis caused by antibodies to NMDA-receptors is presented. This rare pathology is characterized by severe cognitive impairment and needs careful differential diagnosis.

Cognitive dysfunction during anti-NMDA-receptor encephalitis is present in early phase of the disease.

Anti-NMDA-receptor encephalitis is an autoimmune disorder with a well-defined set of clinical features including psychiatric changes (anxiety, agitation, bizarre behaviour, delusional or paranoid thoughts), epileptic seizures and cognitive disturbance followed by movement disorders including orofacial dyskinesias, alterations in the level of consciousness and dysautonomia. Although the cognitive changes are not always very clear at presentation, they can persist after recovery from the acute and often prolonged illness. However, there are few studies describing neuropsychiatric changes in depth, both in the early course of the disease and in long-term follow-up.

The prevalence of depression in hereditary spastic paraplegia.

OBJECTIVE: To evaluate the prevalence of depression and sensitivity and specificity of the single-item interview 'Are you depressed?' for people with hereditary spastic paraplegia in Estonia. DESIGN: Single-item interview 'Are you depressed?' was used as a screening question for depression; all participants then completed the Beck Depression Inventory. SETTING: People with hereditary spastic paraplegia identified from the epidemiological database who agreed to participate in the study. MAIN MEASURES: Beck Depression Inventory, clinical interview. RESULTS: The epidemiological database consisted of 59 patients with clinically confirmed diagnosis of hereditary spastic paraplegia. Forty-eight of these consented to participate in the study. The Beck Depression Inventory score was higher than cut-off point in 58% (28/48) and lower in 42% (20/48). Of the study group, 44% (21/48) had mild, 13% (6/48) moderate and one person revealed severe depression. There was a statistically significant correlation between Beck Depression Inventory score and level of mobility; no other significant correlations with other measures were detected. Of the participants, 54% (26/48) had subjective complaints about depression and answered 'Yes' to the single-item interview 'Are you depressed?'. The sensitivity of the one-item interview in the hereditary spastic paraplegia group was 75% and specificity 75%. CONCLUSIONS: Our results show that mild depression is prevalent among people with hereditary spastic paraplegia. Although the single question may be helpful, it cannot be relied upon entirely when assessing a person for depression.

Clean intermittent self-catheterization in persons with multiple sclerosis: the influence of cognitive dysfunction.

BACKGROUND: Bladder problems are very common in persons with multiple sclerosis (PwMS). OBJECTIVE: The aim of this study was to investigate the ability of PwMS to learn clean intermittent self-catheterization (CISC). METHODS: The physical disability of 23 PwMS was evaluated with the Expanded Disability Status Scale (EDSS), and cognitive status was evaluated with the Brief Repeatable Battery of Neuropsychological Tests (BRB-N). CISC was taught by the same continence advisor who was blinded to the cognitive test results. The ability to learn CISC was evaluated immediately after sessions and 3 months later. Twenty-three consecutive PwMS participated in the study. RESULTS: In all, 87% (20/23) of the PwMS successfully finished CISC training. The number of lessons needed to acquire CISC skills differed significantly depending on the EDSS (Spearman r=0.682, P=0.0003), but the total cognitive decline subscore did not influence the ability to learn CISC. Only 13% (3/23) of the PwMS failed to learn CISC. The ability to learn CISC depended on the number of lessons needed to acquire CISC (r=-0.499, P=0.0313) and the EDSS score (r=-0.433, P=0.0390) but not on the course of the disease (r=0.125, P=0.5696) or on cognitive decline (r=-0.311, P=0.1480). After 3 months of follow-up, 30% (6/20) of the PwMS had ceased performing CISC. A follow-up indicated no statistically significant correlations among any of the subscores of the cognitive test battery, the EDSS score, the course of the disease, and the time required to learn CISC and effective bladder management. CONCLUSIONS: Our study thus confirmed that most (87%) PwMS were able to learn CISC in spite of cognitive dysfunction and therefore to improve their quality of life.

One question as a screening instrument for depression in people with multiple sclerosis.

OBJECTIVE: To evaluate how effectively one question 'Are you depressed?' works as a screening tool for depression in people with multiple sclerosis. DESIGN: The results from a single question were compared with formal clinical diagnosis and the classification from a standard questionnaire. SETTING: Estonian Multiple Sclerosis Centre, from October 2001 to April 2002. SUBJECTS: One hundred and thirty-four consecutive inpatients with multiple sclerosis. INTERVENTION: During two weeks of inpatient stay the mood disorder was analysed. On the basis of clinical interview and Beck Depression Inventory the diagnosis of depression was confirmed. MAIN MEASURES: Beck Depression Inventory and structured clinical interview. RESULTS: Fifty-seven per cent (77/134) of people with multiple sclerosis answered 'Yes' to the question 'Are you depressed?'. The diagnosis of depression was confirmed in 94% (72/77) of people with multiple sclerosis and not confirmed in 6% (5/77). Hence, the screening test sensitivity was 91%. Forty-three per cent (57/134) answered 'No'; 70% (40/57) did not have depression. In this case the sensitivity was 54%. In this group 30% (17/57) were actually depressed according to tests and clinical impression. The age, sex, duration of disease, cognitive abilities and physical disability did not influence consistency of the answers with test results and clinical opinion. CONCLUSIONS: One-question interview is a useful tool for screening for depression in people with multiple sclerosis as it confirms existing depression (sensitivity 91%), but the results should be treated with caution if the person denies mood problems.

IgG immune response to tumor-associated carbohydrate antigens (TF, Tn, alphaGal) in patients with breast cancer: impact of neoadjuvant chemotherapy and relation to the survival.

AIM: To study the humoral immune response to tumor-associated carbohydrate epitopes (TF, Tn and alphaGal) in patients with breast cancer and healthy donors, the putative impact of the chemotherapy and to evaluate if the level of antibody to these epitopes might be beneficial or detrimental for the patients with breast cancer. MATERIALS AND METHODS: The humoral immune response to TF, Tn and alphaGal was studied in 133 patients with breast cancer, including the patients at stage II-III (n = 44) before and after neoadjuvant chemotherapy (10 patients received cyclophosphamide/methotrexate/fluorouracyl (CMF) chemotherapy regimens, 34 patients received cyclophosphamide/doxorubicin/fluorouracil (CAF)), and in controls (healthy donors and patients with fibroadenoma). Fully synthetic carbohydrate hapten-polyacrylamide conjugates were used as antigens in ELISA for anti-carbohydrate antibody determination. The correlation analysis between the level of anti-carbohydrate antibodies and the stage of cancer, histological grade, expression of TF and alphaGal epitopes in tumor tissue, patient's survival was performed. RESULTS: The level of anti-carbohydrate antibodies varied between individuals with no significant correlation between IgG immune response to the three epitopes. Lower levels of antibodies were observed at advanced stages of cancer. Neoadjuvant chemotherapy stimulated antibody production to Tn and alphaGal epitopes (increase > 50%) in about one third of patients. Immunosuppression, decrease in antibody levels, was observed only in 4.5-13.6% of cases. High levels of TF-antigen specific IgG antibody before surgery were associated with a better survival time of stage II breast cancer patients. CONCLUSION: The widely used regimens of neoadjuvant chemotherapy (such as CMF, CAF) can stimulate the immune response to tumor-associated carbohydrate epitopes in some patients. The high levels of anti-TF antibody before surgery are associated with a better survival of stage II breast cancer patients. This may indicate that the selection of immunopotentiating regimens of neoadjuvant chemotherapy might be beneficial for the host.