Primary cerebral immunoglobulin light chain amyloidoma in a patient with multiple sclerosis.

A man in his 60s, known with multiple sclerosis, presented with seizures and paresis of the left arm and leg. Brain imaging showed a white matter lesion, right parietal, which was progressive over the last 6 years and not typical for multiple sclerosis. Brain biopsy showed a B-cell infiltrate with IgA lambda monotypic plasma cell differentiation and amyloid deposits, typed as lambda immunoglobulin light chain (AL). Bone marrow biopsy and PET/CT ruled out a systemic lymphoma. Extended history taking, blood and urine testing (including cardiac biomarkers) identified no evidence of systemic amyloidosis-induced organ dysfunction.Primary cerebral AL amyloidoma is a very rare entity where optimal treatment is difficult to assess. The patient was treated with locally applied volumetric modulated arc radiotherapy, 24 Gy, divided in 12 fractions. Afterwards, the paresis of the left arm partially resolved, and the function of the left leg improved. Seizures did not occur anymore.

Self-reported work productivity in people with multiple sclerosis and its association with mental and physical health.

PURPOSE: This study aimed to identify mental health, physical health, demographic and disease characteristics relating to work productivity in people with multiple sclerosis (MS). METHODS: In this cross-sectional study, 236 employed people with MS (median age = 42 years, 78.8% female) underwent neurological and neuropsychological assessments. Additionally, they completed questionnaires inquiring about work productivity (presenteeism: reduced productivity while working, and absenteeism: loss of productivity due to absence from work), mental and physical health, demographic and disease characteristics. Multiple linear and logistic regression analyses were performed with presenteeism and absenteeism as dependent variables, respectively. RESULTS: A model with mental and physical health factors significantly predicted presenteeism F(11,202) = 11.33, p < 0.001, R2 = 0.38; a higher cognitive (p < 0.001) and physical impact (p = 0.042) of fatigue were associated with more presenteeism. A model with only mental health factors significantly predicted absenteeism; χ2(11)=37.72, p < 0.001, with R2 = 0.27 (Nagelkerke) and R2 = 0.16 (Cox and Snell). Specifically, we observed that more symptoms of depression (p = 0.041) and a higher cognitive impact of fatigue (p = 0.011) were significantly associated with more absenteeism. CONCLUSIONS: In people with MS, both cognitive and physical impact of fatigue are positively related to presenteeism, while symptoms of depression and cognitive impact of fatigue are positively related to absenteeism.Implications for rehabilitationMultiple sclerosis (MS) affects people of working age, significantly interfering with work productivity.Higher cognitive and physical impact of fatigue were associated with more presenteeism in workers with MS.A higher cognitive impact of fatigue and more depressive symptoms were associated with absenteeism in workers with MS.Occupational and healthcare professionals should be aware of the impact of both physical and mental health on work productivity in workers with MS.

Cognitive functioning as a predictor of employment status in relapsing-remitting multiple sclerosis: a 2-year longitudinal study.

BACKGROUND: Cognitive functioning has been linked to employment outcomes in multiple sclerosis (MS) in cross-sectional studies. Longitudinal studies are however lacking and previous studies did not extensively examine executive functioning. OBJECTIVES: We examined whether baseline cognitive functioning predicts a change in employment status after 2 years, while taking into account mood, fatigue and disability level. METHODS: A total of 124 patients with relapsing-remitting MS (pwMS) and 60 healthy controls were included. They underwent neurological and neuropsychological examinations and completed online questionnaires. PwMS were divided into a stable and deteriorated employment status group (SES and DES), based on employment status 2 years after baseline. We first examined baseline differences between the SES and DES groups in cognitive functioning, mood, fatigue and disability level. A logistic regression analysis was performed, with change in employment status (SES/DES) as dependent variable. RESULTS: The DES group included 22% pwMS. Group differences were found in complex attention, executive functioning, self-reported cognitive functioning, fatigue and physical disability. More physical disability (OR = 1.90, p = 0.01) and lower executive functioning (OR = 0.30, p = 0.03) were retained as independent predictors of DES (R2 = 0.22, p ≤ 0.001). CONCLUSIONS: Baseline physical disability and executive functioning, but none of the other variables, moderately predicted a deterioration in employment status 2 years later. TRIAL REGISTRATION: This observational study is registered under NL43098.008.12: 'Voorspellers van arbeidsparticipatie bij mensen met relapsing-remitting Multiple Sclerose'. This study is registered at the Dutch CCMO register (https://www.toetsingonline.nl).

MRI pattern in asymptomatic natalizumab-associated PML.

OBJECTIVE: To investigate the MRI manifestation pattern of asymptomatic natalizumab-associated progressive multifocal leukoencephalopathy (PML) in patients with multiple sclerosis (MS). METHODS: 18 patients with MS with natalizumab-associated PML lesions on MRI were included. In 6 patients, the PML lesions were identified on MRI prospectively and in 12 patients PML lesions were identified retrospectively. MRI sequences were analysed for PML lesion distribution, appearance, grey matter/white matter involvement and possible signs of inflammation. Lesion probability maps were created to demonstrate lesion distribution pattern. RESULTS: The frontal lobe was involved in 14 patients (77.8%) and the parietal lobe in 4 patients (22.2%). Most patients presented with focal lesions (13 patients, 72.2%) involving one single lobe (12 patients, 66.7%). The cortical grey matter was affected in 15 patients (83.3%) and 13 patients (72.2%) presented with a combination of cortical grey and white matter involvement. Signs of inflammation were detected in 7 patients (38.8%). Among patients with available diffusion-weighted imaging, 6 patients (40%) did not show high-signal-intensity lesions. A classical imaging pattern including unilateral and unilobar focal lesions in the frontal lobe affecting the cortical grey matter or the cortical grey and adjacent white matter was observed in 8 patients (44.4%). CONCLUSIONS: Asymptomatic natalizumab-associated PML manifestations on MRI show a rather localised disease, frequently located in the frontal lobes, affecting the cortical grey matter and adjacent juxtacortical white matter. Awareness of this lesion pattern facilitates an earlier diagnosis of natalizumab-associated PML in an asymptomatic stage associated with a more favourable prognosis.

Cerebrospinal Venous Outflow in Multiple Sclerosis Patients versus Fatigue and/or Depression.

BACKGROUND: Endovascular treatment of impaired cerebrospinal venous outflow has been suggested to improve the overall quality of life in multiple sclerosis (MS) patients. Fatigue and depression are key factors in measuring the quality of life in MS patients. OBJECTIVE: In the present study, we investigated the correlation between anomalous venous outflow and the seriousness of fatigue and depression in MS patients and healthy controls. METHODS: Five cerebrospinal venous outflow parameters were measured in 20 MS patients and age- and sex-matched controls using extra- and transcranial Colour Doppler sonography. All patients and volunteers filled out the Fatigue Severity Scale (FSS) and Hospital Anxiety Depression Subscale (HADS). RESULTS: Nine abnormal parameters were found in 8 MS patients, whereas five abnormal parameters were found in 3 healthy controls (no significant difference). Only 1 MS patient met the criteria for chronic cerebrospinal venous insufficiency compared to 2 healthy controls. No significant differences were found in the FSS and HADS scores between patients with and without abnormal cerebrospinal venous outflow parameters. CONCLUSIONS: We found no significantly impaired cerebrospinal venous outflow in patients with MS versus sex- and age-matched controls. Furthermore, we did not find any correlation between anxiety or depression and impaired venous outflow in MS patients.

[A woman with a postoperative lumbar swelling]. / Een vrouw met een postoperatieve lumbale zwelling.

A 65-year-old woman had developed a large lumbar swelling in a period of four weeks following lumbar laminectomy. An MRI-scan revealed a large fluid collection, which had formed from the spinal canal. The diagnosis 'liquorcele', a rare complication of spine surgery, was established.

Daily negative interactions and mood among patients and partners dealing with multiple sclerosis (MS): the moderating effects of emotional support.

Negative interactions with intimate partners may have adverse consequences for well-being, especially for individuals dealing with chronic illness. However, it is not clear whether negative interactions affect both dimensions of positive and negative well-being and factors that may moderate this effect have not been well-described. The aim of the present study was to examine the association between daily received negative responses from the partner and end-of-day positive and negative mood in patients with multiple sclerosis (MS) and their intimate partners. Further, the moderating role of receiving emotional support from the partner on the same day was examined. Sixty-one MS patients and their intimate partners were approached via one MS centre and the neurology department of one hospital in the Netherlands and completed computerized diaries for 14 days. Both partners filled out diaries at the end of each day, recording received negative responses, emotional support and end-of-day positive and negative mood. In line with a domain specific model, patients or partners who reported receiving negative responses on a day had higher end-of-day negative mood, whereas received negative responses were unrelated to end-of-day positive mood. Further, for both patients and partners, the adverse effect of received negative responses on end-of day mood was moderated by receiving emotional support on the same day.

Assessing quality of life in people with multiple sclerosis.

PURPOSE: The quality of life of multiple sclerosis (MS) patients was assessed using two MS specific quality of life measures (FAMS and SF-54) and a short generic measure (EuroQol) in order to make recommendations for routine assessment. Factors contributing to quality of life within this population were also considered. METHOD: Questionnaires were either sent by post or completed at interview. Ninety-six MS patients known to a consultant in rehabilitation medicine completed the questionnaires. RESULTS: Patients scored very low on subscales related to physical health. Correlations were high between the FAMS and SF-54 but low with the EuroQoL. Stepwise multiple linear regressions showed mood measures to account for the most variance on all quality of life composite scores. The MS patients within the study had much lower SF-36 quality of life scores than the general population and other illness groups. CONCLUSIONS: The EuroQol did not measure the same aspects of quality of life as the FAMS and SF-54 and was not found to be a sensitive measure within this sample. The SF-54 suffered from floor effects on physical health subscales and therefore the FAMS was recommended.

Assessment of emotional problems in people with multiple sclerosis.

OBJECTIVE: To investigate the occurrence of emotional problems in multiple sclerosis (MS) patients. The utility of rating scales used to measure such problems was studied to make recommendations for clinical practice. DESIGN: Questionnaires were both sent by post and completed with an assistant psychologist at a home visit. SUBJECTS: Ninety-six MS patients in contact with a rehabilitation consultant. MEASURES: Patients were assessed using the Guy's Neurological Disability Scale (GNDS), Extended Activities of Daily Living Scale (EADL), Hospital Anxiety and Depression Scale (HAD), Beck Anxiety and Depression Scales (BAI, BDI), Clinical Outcomes in Routine Evaluation Measure (CORE) and the Brief Symptom Inventory (BSI). RESULTS: Rates of anxiety and depression ranged from 16% to 48% according to the measures used. All mood scales were highly significantly correlated with each other. Disability as measured by the GNDS, but not the EADL, was significantly correlated with all mood measures. Kappa values showed poor correspondence in the classification of cases. Receiver operating characteristic curves indicated an optimum cut-off point of 7/8 on the HAD and 2/3 on the General Health Questionnaire (GHQ-12). CONCLUSIONS: Rates of emotional problems were low given the highly disabled population studied. The HAD was relatively insensitive in comparison with the BAI and BDI. The GHQ-12 was sensitive and therefore recommended as a short screening measure. An alternative short screen, 'Emotional GNDS', was proposed.