The interactive web-based program MSmonitor for self-management and multidisciplinary care in multiple sclerosis:utilization and valuation by patients

Background: MSmonitor is an interactive web-based program for self-management and integrated, multidisciplinary care in multiple sclerosis. Methods: To assess the utilization and valuation by persons with multiple sclerosis, we held an online survey among those who had used the program for at least 1 year. We evaluated the utilization and meaningfulness of the program's elements, perceived use of data by neurologists and nurses, and appreciation of care, self-management, and satisfaction. Results: Fifty-five persons completed the questionnaire (estimated response rate 40%). The Multiple Sclerosis Impact Profile (MSIP), Medication and Adherence Inventory, Activities Diary, and electronic consultation (e-consult) were used by 40%, 55%, 47%, and 44% of respondents and were considered meaningful by 83%, 81%, 54%, and 88%, respectively. During out-patient consultations, nurses reportedly used the MSmonitor data three to six times more frequently than neurologists. As to nursing care, more symptoms were dealt with (according to 54% of respondents), symptoms were better discussed (69%), and the overall quality of care had improved (60%) since the use of the program. As to neurological care, these figures were 24%, 31%, and 27%, respectively. In 46% of the respondents, the insight into their symptoms and disabilities had increased since the use of the program; the MSIP, Activities Diary, and e-consult had contributed most to this improvement. The overall satisfaction with the program was 3.5 out of 5, and 73% of the respondents would recommend the program to other persons with multiple sclerosis. Conclusion: A survey among persons with multiple sclerosis using the MSmonitor program showed that the MSIP, Medication and Adherence Inventory, Activities Diary, and e-consult were frequently used and that the MSIP, Medication and Adherence Inventory, and e-consult were appreciated the most. Moreover, the quality of nursing care, but not so neurological care, had improved, which may relate to nurses making more frequent use of the MSmonitor data than neurologists

Congenital Bimelic Hypertrophy of the Hands

Congenital hypertrophy of the hand is a rare phenomenon. The condition is characterized by hypertrophy of muscles, varies in severity and has been reported under different names. Some patients also have aberrant muscles. Electromyography is unremarkable, and the signal intensity on MRI and ultrasound is normal. The etiology is unknown and does not seem to be confined to a peripheral nerve, part of the plexus or nerve root. The condition is assumed to be congenital. We report a 28-year-old male with asymmetric hypertrophy of both hands and give a review of the 4 other cases known so far

ORIGINAL ARTICLE - Peripheral neuropathy in merosin-negative congenital muscular dystrophy

Peripheral neuropathy in patients with merosin-negative congenital muscular dystrophy (MN-CMD) has been sporadically investigated and has been considered to be motor and demyelinating in nature on the basis of nerve conduction studies. We performed neurophysiologic studies in 12 children with MN-CMD to establish the spectrum and evolution of peripheral nervous system involvement. In our patients, nerve conduction studies for both motor and sensory nerves were near normal in the children younger than six months and abnormal in the older children. The older children had the relatively slowest nerve conduction velocities suggesting a progressive, age-related dysmyelinating neuropathy. We hypothesize that the findings are due to a myelination arrest as a result of insufficient synthesis and maintenance of the peripheral myelin sheath. (J Pediatr Neurol 2004; 2(4): 213-218)

Local Treatment with Triamcinolone Acetonide and Bevacizumab for Ocular Symptoms in a Patient with POEMS Syndrome

Purpose: To report our experience in managing a case of bilateral optic disc oedema and unilateral cystoid macular oedema with a posterior subtenon (PST) injection of triamcinolone acetonide and intravitreal bevacizumab (IVB) in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. Case Report: A 50-year-old healthy woman complaining of bilateral blurred vision for several days was referred to the ophthalmologist. An eye examination showed a bilateral optic disc swelling and small preretinal haemorrhages. In addition, a peripheral neuropathy was found. Laboratory tests showed elevated levels of M-protein type IgA of 1.4 g/l and a vascular endothelial growth factor (VEGF) concentration of 6.1 ng/ml. The cerebrospinal fluid protein content was 131 mg/dl. An abdominal CT scan revealed hepatosplenomegaly. The diagnosis of POEMS syndrome was made based on the polyneuropathy, hepatosplenomegaly, IgA lambda monoclonal proteinemia, and optic disc swelling. As vision declined, 40 mg PST injection of triamcinolone was given bilaterally. Ten weeks thereafter, 2 IVB injections of 2.5 mg were administered to the left eye, with a 4-week interval. Because of an incomplete recovery of her visual functions, diarrhoea and general malaise, melphalan, followed by autologous peripheral blood stem cell transplantation, was initiated. After systemic treatment, she had no visual complaints anymore, the optic disc oedema had resolved, and the VEGF serum concentration had normalized. Discussion: Local treatment with IVB and PST injection of triamcinolone can be an option for ocular symptoms in POEMS syndrome. However, given the incomplete recovery of the ocular abnormalities and the other symptoms, systemic treatment remains standard

Does self-efficacy affect cognitive performance in persons with Clinically Isolated Syndrome and early Relapsing Remitting multiple sclerosis?

In persons with multiple sclerosis (MS) a lowered self-efficacy negatively affects physical activities. Against this background we studied the relationship between self-efficacy and cognitive performance in the early stages of MS. Thirty-three patients with Clinically Isolated Syndrome (CIS) and early Relapsing Remitting MS (eRRMS) were assessed for self-efficacy (MSSES-18), cognition (CDR System), fatigue (MFIS-5), depressive symptoms (BDI), disease impact (MSIS-29), and disability (EDSS). Correlative analyses were performed between self-efficacy and cognitive scores, and stepwise regression analyses identified predictors of cognition and self-efficacy. Good correlations existed between total self-efficacy and Power of Attention (r = 0.65; P < 0.001), Reaction Time Variability (r = 0.57; P < 0.001), and Speed of Memory (r = 0.53; P < 0.01), and between control self-efficacy and Reaction Time Variability (r = 0.55; P < 0.01). Total self-efficacy predicted 40% of Power of Attention, 34% of Reaction Time Variability, and 40% of Speed of Memory variabilities. Disease impact predicted 65% of total self-efficacy and 58% of control self-efficacy variabilities. The findings may suggest that in persons with CIS and eRRMS self-efficacy may positively affect cognitive performance and that prevention of disease activity may preserve self-efficacy

Relationship between working hours and power of attention, memory, fatigue, depression and self-efficacy one year after diagnosis of clinically isolated syndrome and relapsing remitting multiple sclerosis

The role of cognitive domain dysfunction with respect to vocational changes in persons with Clinically Isolated Syndrome (CIS) and early Relapsing Remitting Multiple Sclerosis (eRRMS) is insufficiently known. We investigated thirty-three patients - 14 CIS, 19 eRRMS -, mean (standard deviation [SD]) time since diagnosis 13.5 (4.8) months and mean (SD) Expanded Disability Status Scale (EDSS) score 1.3 (1.1). Patients were assessed on the CDR System, a set of automated tests of cognitive function, which yielded scores for Power of Attention (ms), Continuity of Attention (#), Working Memory (SI), Episodic Memory (#) and Speed of Memory (ms). Work-related items and the confounding variables fatigue, depression, disease impact and self-efficacy, were assessed by self-report questionnaires. Patients had poorer Power of Attention compared to normative data (1187 [161.5] vs. 1070 [98.6]; P<0.0001) and slower Speed of Memory (4043 [830.6]) vs. 2937 [586.1]; P<0.0001). Power of Attention (Pearson r = -0.42; P<0.04), Working Memory (r = 0.42; P<0.04) and depression r = -0.41; P<0.05) correlated with number of days worked per week. Fatigue (r = -0.56; P<0.005), self-efficacy (r = 0.56; P<0.005) and disease impact (r = -0.46; P<0.05) correlated with number of hours worked per week. Persons who wished to work less had poorer Power of Attention (1247 vs. 1116 ms; P<0.02), those who wished to change job had poorer Episodic Memory (1.35 vs. 1.57; p<0.03). People who reduced working hours within 12 months after diagnosis had higher fatigue and disease impact, and lower self-efficacy. The findings of this pilot study indicate that one year after the diagnosis of CIS and RRMS Power of Attention and Speed of Memory are reduced, that Power of Attention and Memory are associated with a capability of working less hours, and that fatigue, depression and disease impact may negatively, and self-efficacy positively affect working hours

Work-related questions.

<p>Work-related questions.</p

Correlations between confounding variables and HRQoL, and working hours.

<p>Max., maximum;</p><p>*P<0.005;</p><p>**P<0.05.</p

Correlations between cognitive scores and working hours.

<p>Max., maximum; *, P<0.04; all other P values >0.125.</p