Diffuse large B-cell lymphoma of the cauda equina

Primary central nervous system (CNS) lymphoma is a rare form of non-Hodgkin's lymphoma. The clinical presentation is variable, depending on its localisation within the nervous system. Only 1% of primary CNS lymphoma emerges in the spinal cord, and the prevalence of primary lymphoma of the cauda equina is unknown, but probably even rarer. Diagnosing primary lymphoma of the cauda equina is difficult, since it can mimic other more common disorders such as a herniated disc, especially in its early stages. Here we present two cases of primary cauda equina lymphoma in which diagnostic work up took a long time, as the final diagnosis was only reached after a nerve root biopsy

Lithium lacks effect on survival in amyotrophic lateral sclerosis: a phase IIb randomised sequential trial.

Item does not contain fulltextOBJECTIVES: To determine the safety and efficacy of lithium for the treatment of amyotrophic lateral sclerosis (ALS) in a randomised, placebo controlled, double blind, sequential trial. METHODS: Between November 2008 and June 2011, 133 patients were randomised to receive lithium carbonate (target blood level 0.4-0.8 mEq/l) or placebo as add-on treatment with riluzole. The primary endpoint was survival, defined as death, tracheostomal ventilation or non-invasive ventilation for more than 16 h/day. Secondary outcome measures consisted of the revised ALS Functional Rating Scale and forced vital capacity. Analysis was by intention to treat and according to a sequential trial design. RESULTS: 61 patients reached a primary endpoint, 33 of 66 in the lithium group and 28 of 67 patients in the placebo group. Lithium did not significantly affect survival (cumulative survival probability of 0.73 in the lithium group (95% CI 0.63 to 0.86) vs 0.75 in the placebo group (95% CI 0.65 to 0.87) at 12 months and 0.62 in the lithium group (95% CI 0.50 to 0.76) vs 0.67 in the placebo group (95% CI 0.56 to 0.81) at 16 months). Secondary outcome measures did not differ between treatment groups. No major safety concerns were encountered. CONCLUSIONS: This trial, designed to detect a modest effect of lithium, did not demonstrate any beneficial effect on either survival or functional decline in patients with ALS. TRIAL REGISTRATION NUMBER: NTR1448. Name of trial registry: Lithium trial in ALS.1 mei 201

Factors related to intentional non-initiation of bisphosphonate treatment in patients with a high fracture risk in primary care: A qualitative study

Background: Adherence to osteoporosis treatment is crucial for good treatment effects. However, adherence has been shown to be poor and a substantial part of the patients don't even initiate treatment. This study aimed to gain insight into the considerations of both osteoporosis patients and general practitioners (GP) concerning intentional non-initiation of bisphosphonate treatment. Methods: Osteoporosis patients and GPs were recruited from the SALT Osteoporosis Study and a transmural fracture liaison service, both carried out in the Netherlands. Using questionnaires, we identified non-starters and starters of bisphosphonate treatment. Semi-structured interviews were conducted to gain a detailed overview of all considerations until saturation of the data was reached. Starters were asked to reflect on the considerations that were brought forward by the non-starters. Interviews were open coded and the codes were classified into main themes and subthemes using an inductive approach. Results: 16 non-starters, 10 starters, and 13 GPs were interviewed. We identified three main themes: insufficient medical advice, attitudes towards medication use including concerns about side effects, and disease awareness. From patients' as well as GPs' perspective, insufficient or ambiguous information from the GP influenced the decision of the non-starters to not start bisphosphonates. In contrast, starters were either properly informed, or they collected information themselves. Patients' aversion towards medication, fear of side effects, and a low risk perception also contributed to not starting the medication, whereas starters were aware of their fracture risk and were confident of the outcome of the treatment. Concerns about osteoporosis treatment and its side effects were also expressed by several GPs. Some GPs appeared to have a limited understanding of the current osteoporosis guidelines and the indications for treatment. Conclusions: Many reasons we found for not starting bisphosphonate treatment were related to the patients or the GPs themselves being insufficiently informed. Attitudes of the GPs were shown to play a role in the decision of patients not to start treatment. Interventions need to be developed that are aimed at GPs, and at education of patients

Outcome measures in MMN revisited : Further improvement needed

The objectives of this study were to provide an overview of the outcome measures (OMs) applied in clinical trials in multifocal motor neuropathy (MMN) and to determine the responsiveness of a core set of selected OMs as part of the peripheral neuropathy outcome measures standardization (PeriNomS) study. The following OMs were serially applied in 26 patients with newly diagnosed or relapsing MMN, receiving intravenous immunoglobulin (assessments: T0/T3/T12 months): 14 muscle pairs MRC (Medical Research Council) scale, the Neuropathy Impairment Scale motor-subset, a self-evaluation scale, grip strength, and MMN-RODS\ua9 (Rasch-built overall disability scale). All data, except the grip strength, were subjected to Rasch analyses before determining responsiveness. For grip strength, responsiveness was examined using a combined anchor- (SF-36 question-2) and distribution-based ( 7 SD) minimum clinically important difference (MCID) techniques, determining the proportion of patients exceeding both the identified cut-offs. For the remaining scales, the magnitude of change for each patient on each scale was determined using the MCID related to the individual SE (responder definition: MCID-SE 65 1.96). Overall, a great assortment of measures has been used in MMN trials with different responsiveness definitions. For the selected OMs, responsiveness was poor and only seen in one fourth to one third of the patients, the grip strength being more responsive. Despite the efforts taken to standardize outcome assessment, further clinimetric responsiveness studies are needed in MMN

Correlation of the patient's reported outcome Inflammatory-RODS with an objective metric in immune-mediated neuropathies

BACKGROUND AND PURPOSE: There is increasing interest in using patient-reported outcome measures (PROMs) in clinical studies to capture individual changes over time. However, PROMs have also been criticized because they are entirely subjective. Our objective was to examine the relationship between a subjective PROM and an objective outcome tool in patients with Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and gammopathy-related polyneuropathy (MGUSP). METHODS: The Inflammatory Rasch-built Overall Disability Scale (I-RODS©, a multi-item scale that examines functionality) was completed by 137 patients with newly diagnosed (or relapsing) GBS (55), CIDP (59) and MGUSP (23) who were serially examined (GBS/CIDP, T0/T1/T3/T6/T12 months; MGUSP, T0/T3/T12). Possible association between the I-RODS findings and the vigorimeter scores, an objective linear instrument to assess grip strength, was examined. RESULTS: A significant correlating trend was found between the I-RODS and grip strength scores for the overall group and in each illness, independently. CONCLUSION: The objectivity of patients' subjective report on their functional state based on a strong correlation between the I-RODS and grip strength in patients with GBS, CIDP and MGUSP has been demonstrated. These findings provide further support to use the I-RODS and grip strength in future clinical studies in these conditions