Inaugural tumor-like multiple sclerosis: clinical presentation and medium-term outcome in 87 patients.

BACKGROUND: Tumefactive demyelinating lesions of the central nervous system can be the initial presentation in various pathological entities [multiple sclerosis (the most common), Balo's concentric sclerosis, Schilder's disease and acute disseminated encephalomyelitis] with overlapping clinical presentation. The aim of our study was to better characterize these patients. METHODS: Eighty-seven patients (62 women and 25 men) from different MS centers in France were studied retrospectively. Inclusion criteria were (1) a first clinical event (2) MRI showing one or more large demyelinating lesions (20 mm or more in diameter) with mass-like features. Patients with a previous demyelinating event (i.e. confirmed multiple sclerosis) were excluded. RESULTS: Mean age at onset was 26 years. The most common initial symptoms (67% of the patients) were hemiparesis or hemiplegia. Aphasia, headache and cognitive disturbances (i.e. atypical symptoms for demyelinating diseases) were observed in 15, 18 and 15% of patients, respectively. The mean largest diameter of the tumefactive lesions was 26.9 mm, with gadolinium enhancement in 66 patients (81%). Twenty-one patients (24%) had a single tumefactive lesion. During follow-up (median time 5.7 years) 4 patients died, 70 patients improved or remained stable and 12 worsened. 86% of patients received initial corticosteroid treatment, and 73% received disease-modifying therapy subsequently. EDSS at the end of the follow-up was 2.4 ± 2.6 (mean ± SD). CONCLUSION: This study provides further evidence that the clinical course of MS presenting with large focal tumor-like lesions does not differ from that of classical relapsing-remitting MS, once the noisy first relapsing occurred.

The Global Adherence Project (GAP): a multicenter observational study on adherence to disease-modifying therapies in patients with relapsing-remitting multiple sclerosis.

BACKGROUND: most disease-modifying therapies (DMTs) for multiple sclerosis (MS) are self-injectable medications that must be taken on an ongoing basis to reduce disease activity. Thus, adherence to therapy becomes an important challenge that must be addressed to maximize benefits of therapy. This study evaluated rates of adherence to prescribed treatment and explored factors affecting adherence amongst patients with relapsing-remitting MS. METHODS: this was an observational, multicenter, multinational, phase 4 study. Patients and physicians received paper questionnaires regarding adherence to DMTs approved at the time of the study, including intramuscular interferon beta-1a (IFNß-1a), subcutaneous IFNß-1a, IFNß-1b, and glatiramer acetate. Quality of life and cognition data also were collected. Multivariate analysis was conducted to identify factors associated with adherence to long-term DMTs. RESULTS: two thousand six hundred and forty-eight patients were studied, revealing an average treatment duration of 31 months. Seventy-five percent of patients (n = 1923) were adherent to therapy. The most common reasons for non-adherence were forgetting to administer the injection (50.2%) and other injection-related reasons (32.0%). Adherent patients reported better quality of life (P < 0.05) and fewer neuropsychological issues (P < 0.001) than non-adherent patients. Adherent patients had significantly shorter duration of disease (P < 0.001) and shorter duration of therapy (P = 0.005) than non-adherent patients. Women were more likely than men to adhere to treatment. CONCLUSION: identifying factors that affect adherence to prescribed treatments is the first step in improving adherence of patients with MS to therapy, thereby helping maximize the benefits of long-term DMTs.

Prospective clinical and electrophysiological follow-up on a multiple sclerosis population treated with interferon beta-1 a: a pilot study.

OBJECTIVE: To analyse transcranial magnetic stimulation (TMS) variables in a prospective six-month follow-up pilot study on patients suffering from relapsing-remitting multiple sclerosis (RRMS), satisfying inclusion criteria for interferon (IFN) beta-1a treatment. BACKGROUND: So far, no predictive factors are available as to the course of RRMS treated with IFN beta-1 a. DESIGN/METHODS: Fifteen RRMS patients were studied before (month 0 (M0)) and after IFN beta-1a onset (M3, M6). The parameters analysed were motor functional score (mFS), Expanded Disability Status Scale (EDSS), and TMS variables - central motor conduction time (CMCT) and amplitude ratio (AR). RESULTS: Four of the six patients with no motor signs at inclusion, subsequently showed signs of pyramidal dysfunction. All had abnormal M0_TMS variables. The number of M0_TMS abnormalities per patient was greatest in the group that showed mFS worsening, and was significantly correlated with M6_EDSS. The M0_CMCT was significantly correlated with M6_EDSS. During follow-up, the number of patients with abnormal TMS variables decreased from 12/15 to 4/15, and the total number of abnormalities decreased from 33.3 to 16.7%. CONCLUSIONS: TMS variables might be predictive of disease progression. The improvement observed here in the TMS variables may reflect an improvement in MS patients undergoing IFN beta treatment.

[Progressive spastic paraplegia as a presentation of oculodentodigital syndrome]. / Syndrome oculodentodigital révélé par une paraparésie spastique progressive.

Oculodentodigital syndrome (ODD) is a rare congenital disorder which associates eyes and facial abnormalities, defects in teeth enamel and type III syndactyly. The causal genetic defect in this syndrome is still unknown. Some patients with ODD syndrome also manifest spastic paraparesis. In most cases, inheritance is autosomal dominant with variable clinical expression. Herein, we report on a patient with ODD syndrome, who was referred for evaluation of spastic paraplegia. Our observations show that ODD syndrome can be recognized in late adulthood and revealed by spastic paraplegia.

[Hashimoto's encephalitis and sleep disorders]. / Encéphalite d'Hashimoto et troubles du sommeil.

Hashimoto's encephalitis is a rare cause of encephalitis which is improved by corticosteroid treatment. We report the case of a 42-year-old woman who developed progressive dementia associated with episodes of recurrent discorders of consciousness which rapidly improved with corticosteroids. During these episodes, no sleep activity was recorded on the holter EEG. These discorders were reversible with treatment and a normal EEG sleep pattern reappeared. At physical examination, Hashimoto's encephalitis can mimic Creutzfeld-Jakob disease. Systematic sleep-EEG recordings can be helpful for diagnosis of sleep disorders related Hashimoto's encephalitis. This case illustrates the importance of searching for antithyroid antibodies in patients with unexplained encephalitis.

A longitudinal study of callosal atrophy and interhemispheric dysfunction in relapsing-remitting multiple sclerosis.

OBJECTIVES: To determine if callosal atrophy and interhemispheric dysfunction can be detected in the early stages of relapsing-remitting multiple sclerosis (MS) and to evaluate their progression in relation to the disability and evolution of lesions seen on magnetic resonance imaging during a 5-year period. METHODS: We compared 30 patients who had clinically definite early-onset replasing-remitting MS and mild disability with control subjects. Regional and segmental callosal size and extent of white matter abnormalities on magnetic resonance imaging, as well as performance on tasks exploring interhemispheric transfer of motor, auditory, and sensory information were assessed. Patients with MS were evaluated at baseline and after 5 years. Physical disability was determined at both times using the Expanded Disability Status Scale score. RESULTS: Patients with MS were seen with significant callosal atrophy and functional impairment of interhemispheric transfer at baseline that worsened during the 5-year study. A significant correlation was found between the magnitude of disability and the severity of morphological and functional callosal involvement at baseline. This association persisted at year 5. Baseline clinical characteristics such as age and prestudy relapse rate were unrelated to callosal size or interhemispheric performance. However, the number of baseline T2-weighted lesions was correlated with callosal involvement and this relation persisted at year 5. CONCLUSION: Patients who had relapsing-remitting MS in the early stages of the disease and mild disability had significant callosal involvement that progressed over time. The relationship between disability, T2-weighted lesions load, and degree of morphological and functional callosal impairment confirm the potential value of using callosal dysfunction as a surrogate marker of disease progression in MS.

Alcoholic epilepsy: a unified and dynamic classification.

The interactions between alcohol and the CNS are complex and there are experimental data suggesting that chronic and acute effects are different and often opposite. An intriguing hypothesis is that repeated alcohol withdrawal seizures (AWS) may render the brain more excitable, leading to an epileptogenic state reminiscent of the 'kindling' model. To gain insight into this question we compared alcoholic patients with seizures related to episodes of AW (AWS) and patients with seizures unrelated to episodes of AW (UAWS). There were several significant differences between the AWS and UAWS groups. Age at admission for seizure was younger in the AWS groups (p < 0.005), seizure number was higher among patients with a history of seizures before admission in the UAWS group (p < 0.05). Neurologic signs were more frequent in the UAWS group (p < 0.05). Duration of intoxication was longer in the UAWS group and brain atrophy demonstrated by CT scan was more common in the UAWS group than the AWS group. Based on these findings, we propose a dynamic classification in which patients presenting seizures unrelated to any cause other than alcohol are classified in several successive stages of 'alcoholic epilepsy', the first being characterized by AWS and the last by persistent chronic seizures.

Goals and preparation method for ground operations for the manned space flight Altair.

The mission's success fully depends on the Payload Operations conducted during the space flight. The Ground Team has to be trained to assist the Space Crew, to replan the cosmonaut's activities when contingencies occur onboard and to change or cancel Payload activities when required. In order to act efficiently during the mission, the Ground Team must be prepared in advance of the flight and able to operate special tools for tracking the mission's progress. anticipating problems and taking decisions in realtime. This document sets out the approach for conducting such a preparation for Ground Operation. It will be focused on the Altair mission performed in July 1993 onboard the Russian Mir space station.

Biomedical program of the ALTAIR french russian flight onboard the MIR station.

One year after the achievement of the 2 weeks ANTARES french-russian mission in the MIR station in July 1992, a 22 days ALTAIR mission with a french cosmonaut has been performed in July 1993, making use of the scientific payload remaining on board. Taking benefit of the analysis of the previous mission, the experimental protocols were adapted to refine scientific objectives and gave to the scientists the opportunity to enhance quantitatively and qualitatively their results. The french biomedical program, conducted in close scientific cooperation with IMBP and associated laboratories, was composed of 8 experiments out of which 2 were new with regards to the ANTARES program. In the field of cardio-vascular physiology and fluid regulation, the experiments: ORTHOSTATISME, DIURESE have been renewed and complemented by the TISSU experiment (proposed by a german scientist) and a real-time tele-assistance program using US echography technic and ground support from the french CADMOS support control center located in Toulouse. With respect to neurosciences objectives, to the experiments VIMINAL (cognitive processes) and ILLUSIONS (study of proprioceptives cues), was added the SYNERGIES experiment to analyse the postural adjustments during movement. The IMMUNOLOGIE experiment carried on and the radiobiological experiment BIODOSE ended. Adding the results of the 2 missions ANTARES and ALTAIR, and the data obtained in between onboard with russian cosmonauts, the scientists have received a wealth of physiological data and gained reproducibility and confidence in their results.