Information from patients who are unable to continue their visits to a study centre may be of major importance for the interpretation of results in multiple sclerosis (MS) clinical trials. To validate a questionnaire based on the Expanded Disability Status Scale (EDSS), patients in five different European centres were assessed independently by pairs of trained EDSS raters, first by telephone interview and a few days later by standardized neurological examination. Seventy women and 40 men with an average age of 43.7 years (range 19-74 years) were included in the study. Mean EDSS score at the last visit was 4.5 (0-9). EDSS assessment by telephone was highly correlated with the EDSS determined by physical examination (Pearson's correlation coefficient = 0.95). An intraclass correlation coefficient (ICC) of 94.8% was found for the total sample; 77.6% and 86%, respectively, for patients with EDSS < 4.5 (n = 46) and > 4.5 (n = 64). Kappa values for full agreement were 0.48; for variation by +0.5 steps and +1.0 steps, 0.79 and 0.90, respectively. Best agreement could be found in higher EDSS scores, where assessment by telephone interview might be needed most. The telephone questionnaire is a valid tool to assess EDSS score in cases where the patient is unable to continue visiting a study centre or in long-term follow-up of trial participants.

Objective: To evaluate Bacille Calmette-Guérin (BCG) effects after clinically isolated syndromes (CIS).Methods: In a double-blind, placebo-controlled trial, participants were randomly assigned to receive BCG or placebo and monitored monthly with brain MRI (6 scans). Both groups then entered a preplanned phase with IM interferon-b-1a for 12 months. From month 18 onward, the patients took the disease-modifying therapies (DMTs) that their neurologist considered indicated in an open-label extension phase lasting up to 60 months.Results: Of 82 randomized subjects, 73 completed the study (33 vaccinated and 40 placebo). Conclusions: Early BCG may benefit CIS and affect its long-term course.Classification of evidence: BCG, as compared to placebo, was associated with significantly reduced development of gadolinium-enhancing lesions in people with CIS for a 6-month period before starting immunomodulating therapy (Class I evidence). Neurology ® 2014;82:41-48 GLOSSARY BCG 5 Bacille Calmette-Guérin; CDMS 5 clinically definite multiple sclerosis; CI 5 confidence interval; CIS 5 clinically isolated syndrome; CSE 5 conventional spin-echo; DMT 5 disease-modifying therapy; EDSS 5 Expanded Disability Status Scale; Gd 5 gadolinium; IFN 5 interferon; MS 5 multiple sclerosis; RR 5 relative risk; T1W 5 T1-weighted; T2W 5 T2-weighted; TE 5 echo time; TR 5 repetition time.The majority of multiple sclerosis (MS) cases start with a first demyelinating episode (usually referred to as clinically isolated syndrome [CIS]) that is generally reversible. Approximately half of these cases convert to clinically definite MS (CDMS) within 2 years of the diagnosis and have substantial risk of disability, while about 10% of people with CIS remain free of further neurologic events, even in the presence of MRI compatible with MS.

Our observation that in many patients with MS-related TN a pontine demyelinating plaque and neurovascular compression coexist should prompt neurologists to seek possible neurovascular compression in patients with MS-related TN.

Background and Purpose: The etiology of stroke in the young is different from that in older patients and remains unknown in almost one third of the cases. To gain further insight into both pathogenic and etiologic determinants, we prospectively studied a large number of consecutive young adults with focal cerebral ischemia.

We investigated MRI activity in MS during the menstrual cycle in relation to physiologic sex hormone fluctuations. Eight women with relapsing-remitting MS were submitted to serial brain gadolinium-enhanced MRI examinations over a 3-month period in two alternate follicular and luteal phases of the menstrual cycle. The ratio of progesterone/17-beta-estradiol during the luteal phase was significantly associated with both number (r = 0.6, p = 0.03) and volume (r = 0.7, p = 0.009) of enhancing lesions, providing support for a role of these hormones as immunomodulatory factors in MS.

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In this review we summarize progress in research on Parkinson's disease-related pain as reported in articles published in the Journal of Neurology in the years 2011 and 2012.

To further evaluate the relationship between clinical disability and Magnetic Resonance Imaging (MRI) lesion burden, we examined 85 patients with clinically definite multiple sclerosis (54 relapsing-remitting and 31 secondary progressive). This cross-sectional study reports on the correlations between total and infratentorial lesion volume on both T1 and T2 weighted images, and overall physical disability measured by Expanded Disability Status Scale, ambulation index and individual functional systems. Assessment of the hypointense lesion load on T1 weighted images rather than the hyperintense lesion load on T2 weighted images at brain MRI was shown to be useful for differentiating relapsing-remitting from secondary progressive Multiple Sclerosis. A weak relationship between disability and total lesion volume on both T1 and T2 weighted images was found in relapsing-remitting Multiple Sclerosis. In secondary progressive Multiple Sclerosis, infratentorial lesion volume on T2 weighted images represents the only marker of disability. Finally, the presence of cerebellar, brainstem and mental impairment was significantly associated to a greater total lesion volume on MRI, while no relationship was found with other functional systems.