The aim of the present study was to determine the sensitivity and the profile of motor evoked potentials (MEP) in patients with clinically isolated syndrome (CIS) suggestive of multiple sclerosis (MS). We measured the central motor conduction time (CMCT), amplitude ratio (AR), and surface ratio (SR) in tibialis anterior and first dorsal interosseous muscles in 22 patients with CIS. In 12 patients, the triple stimulation technique (TST) was also performed. AR was abnormal in 50% of patients, CMCT in 18% of patients, and TST in 25% of patients. AR had the highest sub-clinical sensitivity and the best positive predictive value. In the absence of clinical pyramidal signs, an early AR decrease seems to result from demyelination inducing excessive temporal dispersion of the MEP, while in territories with clinical pyramidal signs, it seems to result from conduction failure, which suggests that clinical pyramidal signs may be attributable to conduction failure. This study demonstrates that MEP, especially the AR, is sensitive to motor pathway dysfunction right from the early stages of MS.

L’anorexie mentale est un trouble psychiatrique grave, pour lequel on ne dispose que de peu de stratégies thérapeutiques validées. Le style sociocognitif particulier de ces sujets a été décrit dès les années 1960 : un style concret avec des difficultés d’abstraction. Des évaluations neuropsychologiques permettent désormais de mieux le définir, mettant en évidence une attention excessive aux détails, une flexibilité mentale défaillante, ainsi qu’une perception et une expression émotionnelles altérées, en dépit de performances globales comparables à celles de la population générale. La neuro-imagerie apporte également des arguments dans le sens d’anomalies morphologiques et fonctionnelles de structures corticales et sous-corticales, impliquant en particulier les circuits fronto-striatothalamiques. Au vu de ces données récentes, qui demandent à être davantage étudiées, ce profil pose la question d’une analogie avec les troubles neurodéveloppementaux, et la remédiation cognitive déjà proposée dans certains d’entre eux apparaît alors comme une voie thérapeutique innovante.Anorexia nervosa is a serious psychiatric disorder, for which very few validated therapeutic strategies exist. The specific sociocognitive style of anorexic patients has already been described in the 1960s: it involves a concrete style with abstraction difficulties. Current neuropsychological tests have contributed to a more precise definition of these difficulties.Contrary to common beliefs, these patients’ intellectual performances are not superior to those of the general population. However, detailed comparisons of profiles on the Weschler Scales suggest difficulties in synthesizing information and better abilities in concrete problem solving.The dominant hypothesis concerning the attentional dimension is the existence of a weakness in central coherence, resulting in superior detail processing and a weakness in global integration. This trend appears to be stable even after the normalization of nutritional status.The impairment of set-shifting abilities leads to rigidity, expressed by inflexibility and perseveration, both in reasoning and behaviour. This reduced cognitive flexibility appears to persist after recovery, and may constitute a familial trait. In addition, this likely endophenotype seems to be independent from obsessional traits.Alexithymia is frequently described in anorexic individuals. It is the verbal description of feelings which seems to be particularly impaired. It may explain underlying difficulties in empathy. Indeed, these subjects have lower scores on emotional tests drawn from the theory of mind. These cognitive abnormalities are well documented in pervasive developmental disorders.Evidence from neuroimaging suggests abnormalities in cortical and subcortical structures, involving the temporal and orbito-frontal lobes. Various functional hypotheses are formulated, involving fronto-striatothalamic circuits, amygdala or insula.Pervasive developmental disorders are over-represented among anorexic subjects in comparison to the general population. Conversely, restrictive and selective eating disorders are more frequent among individuals presenting an autistic spectrum disorder.In view of the common cognitive and neuroanatomical data that are found in anorexia nervosa and neurodevelopmental disorders, we adhere to the hypothesis that anorexia nervosa may be similar to a neurodevelopmental disorder. Clinical observations suggest that this hypothesis may be especially relevant in the early forms of anorexia nervosa. These cognitive data confirm the potential relevance of new therapeutic modalities such as cognitive remediation. Initial results from its application to anorexia nervosa seem promising.A review of the recent literature highlights the possible existence of a developmental impairment of cortical and subcortical structures, associated with specific abnormalities in cognitive development such as a weakness in central coherence, reduced set-shifting ability and poor social skills. On this basis, cognitive remediation may be a promising therapeutic innovation.

L'évaluation de la plainte mnésique repose généralement sur l'utilisation de questionnaires d'auto-évaluation qui quantifient seulement les dysfonctionnements de la mémoire ou d'autres activités cognitives tels qu'ils sont perçus par le sujet. Or la plainte mnésique exprimée spontanément par la personne se distingue d'une simple perception d'un éventuel dysfonctionnement, témoigne d'un inconfort et traduit un ressenti. Le but de cette étude est de proposer un outil permettant de quantifier spécifiquement cette dimension de la plainte mnésique. Cent cinq sujets témoins, répartis en 4 classes d'âge (50 - 90 ans) et 3 classes de niveau culturel, sans demande précise concernant leur mémoire, ont participé à l'étude de normalisation de l'Echelle d'Intensité de la Plainte Mnésique (EIPM). Cette échelle comprend dix questions réparties en trois rubriques : Historique des modifications de la mémoire, Répercussions dans la vie quotidienne, Médicalisation/Nosophobie. Chaque réponse a été notée sur une échelle de 0 à 3, donnant un score total maximum de 30. Un score partiel a été calculé pour chaque rubrique. L'EIPM a été aussi appliquée à 83 sujets autonomes, reçus en consultation mémoire (MMS ≥24). Les données du bilan neuropsychologique extensif ont permis de distinguer deux groupes : l'un de 42 patients présentant un MCI amnésique et l'autre de 41 sujets présentant une plainte mnésique isolée (PMI). Les résultats obtenus chez les témoins montrent que la plainte est d'autant plus exprimée que le niveau culturel est élevé, sans répercussion sur la vie quotidienne ou démarche de médicalisation. Le score total moyen de plainte chez les témoins est toujours inférieur à 6. La comparaison des résultats des consultants et de ceux des témoins, met en évidence un score total et des scores partiels à l'EIPM significativement plus élevés pour les consultants. On observe également des différences de distribution des scores totaux de plainte entre le groupe contrôle d'une part et les deux autres groupes (MCI et PMI) qui ont un profil similaire. Cette étude met en évidence la grande sensibilité à la plainte mnésique de l'EIPM et fournit des normes de référence pour l'utilisation de cette échelle en consultation mémoire.

Context. - Progressive supranuclear palsy (PSP) is classically characterized by supranuclear ophthalmoplegia, paroxysmal imbalance with backward falling, axial dystonia, rigidity, pseudobulbar palsy and cognitive dysfunction. However, incomplete or atypical clinical presentation has been previously reported, but in all these cases, the patients had at least one of the main clinical features of the disease (ophthalmoplegia, parkinsonian syndrome or cognitive dysfunction).Case report. - A 60-year-old woman presented with nocturnal agitation and choreiform movements. A few months later she developed severe swallowing disorders, caused by achalasia of the upper esophageal sphincter, and responsible for recurrent acute respiratory distress and pneumonia, prevailing to tracheotomy and gastrostomy. She died suddenly two years after the onset of the symptoms.Results. - Postmortem examination of brain revealed a tauopathy, with deposition of abnormal phosphorylated tau in threads and in coiled-shaped as well as globose tangles in the brainstem, subthalamic nuclei and hippocampus. Nuclei of the medulla, including the vagus/solitarius complex and the region of the nucleus ambiguous were especially rich in tau positive inclusions. Ultrastructural analysis of globoid-shaped tangles in the brainstem revealed the presence of straight and paired helicoidal filaments compatible with a PSP.Conclusions. - This case contributes to improve knowledge of the clinical phenotypic range of PSP. in this case, the neuropathological lesions accounted for most of the symptoms. However, the early death of the patient was probably related to the particular distribution of the neuropathological lesions. This case suggests that the initial neuropathological changes in PSP is located in the dorsal brainstem. (C) 2008 Elsevier Masson SAS. Tous droits reserves.

Introduction. The most frequent acute and sub-acute complications of chronic alcoholism are delirium tremens, hepatic encephalopathy and Gayet-Wernicke encephalopathy. Morel laminar sclerosis is a rare and less known complication, often reported with Marchiafava-Bignami disease. Case report. A 57-year-old alcoholic man presented delirium after surgery. Anterograde and retrograde amnesia as well as wrong recognitions appeared progressively and one generalized seizure occurred. He then developed mutism and became bedridden. Magnetic resonance imaging (MRI) showed high-intensity bilateral ternporoparietal signals from white matter on T2-weighted images and high-intensity signals from the parietal cortex on T1-weighted images. The patient died four months after the onset of the delirium. Postmortem examination of the brain showed cortical laminar necrosis with Alzheimer Type II gliosis but without demyelinisation of the corpus callosum. Conclusion. Cortical laminar necrosis with chronic ethylism is usually called Morel's laminar sclerosis. Nevertheless, histology is not typical of this diagnosis, because of necrosis especially of the second (and not the third) layer of the cortex, and because of the absence of lesion of the corpus callosum. MRI data are of interest here because they were rarely reported in cases of Morel's laminar sclerosis.

Proton magneticresonance spectroscopic imaging(1H-MRSI) was used to studymetabolic abnormalities inside thegray matter (GM) during or distantto white matter (WM)inflammatory processes reflectedby T1 gadolinium-enhancing lesionsin patients at the very earlystage of multiple sclerosis (MS).The spectroscopic examinationwas performed in the axial planeusing a home-designed acquisition-weighted, hamming shape,2D-SE pulse sequence(TE = 135 ms; TR = 1,600 ms).Bilateral thalami and the medialoccipital cortex were explored in35 patients (15 with and 20 withoutT1-Gd enhancing lesions) withclinically isolated syndromesuggestive of MS and in 30 controls.The mean duration since thefirst presenting symptom was 9.1(±6.7) months. The two groups ofpatients (with or withoutT1 Gd-enhancing lesions) did notdiffer in terms of time elapsedsince the first clinical onset andT2 lesion load. The spatial contaminationof surrounding WMtissues was obtained in each GMregion by determining the tissuecomponent in the ROI from GMand WM probability mapssmoothed with the point spreadfunction of the MRSI acquisition.Contribution of WM signal wasimportant (60%) inside thalamiwhile the region centered on themedial occipital cortex was wellrepresentative of GM metabolism(>70%). Comparisons of relativemetabolite levels (ratios of eachmetabolite over the sum of allmetabolites) between all patientsand controls showed significantdecrease in relative N-acetylaspartate (NAA) levels, increase inrelative choline-containing compounds(Cho) levels and nochange in relative creatine/phosphocreatinelevels inside the threeROIs. Decrease in relative NAAlevels and increase in relative Cholevels were found in patients withinflammatory activity, while nometabolic alterations were presentin patients without T1 Gd-enhancinglesions. These resultssuggest that abnormalities in GMmetabolism observed in patientsat the very early stage of MS aremainly related to neuronal dysfunctionoccurring during acuteinflammatory processes.

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-1a.Design/methods Fifteen RRMS patients were studied before (month 0 (MO)) 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.

Cognitive impairment in patients with multiple sclerosis (MS) is a common occurrence and is generally fairly circumscribed. The prevalence of the cognitive deficits usually encountered could vary with the clinical course of the disease. To investigate whether the presence of cognitive impairment may occur in the very early stage of MS, we assessed the cognitive status of a group of 40 patients presenting with a recently diagnosed clinically isolated syndrome suggestive of MS (CISSMS), in comparison with 30 age-, sex-, and educational level-matched healthy control subjects. An extensive battery of neuropsychological tests was used to explore verbal and non-verbal memory, attention, concentration, speed of information processing, language and abstract reasoning. Patients with CISSMS had a significant, frequent (57%), and circumscribed cognitive impairment, focused on memory, speed of information processing, attention and executive functions.

The paced auditory serial addition test (PASAT) is routinely used to evaluate the cognitive part of the multiple sclerosis functional composite (MSFC) score, the new reference index of patient disability. PASAT is sensitive to subtle cognitive impairment related to MS, although the cognitive components of this test still remain unclear. In order to better characterize brain systems involved during this complex task, functional magnetic resonance imaging (fMRI) experiments were conducted during PASAT in a population of ten normal subjects. The paradigm consisted of a series of 61 single-digit numbers delivered every 3 s. After each number, subjects were asked to overt vocalize the result of the addition of the two last numbers heard. A control task consisting of the repetition of the same series of single-digit numbers was used. Statistical group analysis was performed using the random effect procedure (SPM 99). Cortical activation was observed in the left prefontal cortex, the supplementary motor area, the lateral premotor cortex, the cingulate gyrus, the left parietal lobe, the left superior temporal gyrus, the left temporal pole, and visual associative areas. fMRI activations underlying PASAT were consistent with an involvement of verbal working memory and the semantic memory retrieval network which could be related to arithmetic fact retrieval. This study on normal subjects could provide a base for the understanding of the potential abnormal cortical activation in MS patients performing this test for a cognitive evaluation.

INTRODUCTION:Chickenpox is considered as a high risk factor for developing stroke in childhood, but descriptions in adult are exceptional (only three cases reported, to our knowledge).CASE REPORT:A 37-year-old man presented with a chickenpox eruption, followed by a right parietal and a left occipital infarcts, associated with multiple lacunae. There was no coagulation disorder, no hypertension or cardiovascular disorder. Cerebral angiography showed an irregular narrowing of the right internal parietal artery and vascular defects in right parietal and left occipital areas. The diagnosis of VZV-related vasculitis was evoked. White cell count, serology and VZV PCR were negative in the cerebrospinal fluid. Clinical improvement was observed after treatment by corticosteroids and aciclovir.CONCLUSION:Chickenpox is a rare cause of cerebral vasculitis. Involvement of both medium and small vessels was present here, contrary to other adult case reports in the literature. Hematogenous dissemination of the virus responsible for cerebral vasculitis seems to be the most probable pathophysiological mechanism.

INTRODUCTION Thrombolytic treatment in the early stage of ischemic cerebral attacks requires rapid confirmation of the diagnosis and topographic localization. Unusual clinical features can lead to misdiagnosis with the risk of delaying optimal therapeutic management. OBSERVATION We report the cases of two patients who experienced acute tetraparesis without any associated encephalic sign, consistent with the diagnosis of spinal cord injury. Cervical magnetic resonance imaging (MRI) was normal. Conversely, cerebral MRI displayed in both cases bilateral hemispheric infarction. Two ischemic lesions were revealed in the territory of both anterior cerebral arteries in the first patient, while the second patient had a bilateral infarction in the posterior arms of both internal capsules. CONCLUSION In case of tetraparesis, emergency spinal cord MRI should be performed to rule out neurosurgical etiologies and ischemia. If negative, cerebral MRI should be performed at the same time to look for early cerebral infarction in both hemispheres and determine the indication for thrombolysis.

L’association neurofibromatose de type 1 (NF-1) et sclérose en plaques (SEP) n’a été que très rarement rapportée. Nous décrivons le cas d’une patiente de 40 ans, porteuse d’une NF-1 familiale révélée dans l’enfance par des taches café-au-lait et des neurofibromes cutanés. Elle présenta successivement dès l’âge de 35 ans, une névrite optique rétrobulbaire inaugurale, des épisodes déficitaires sensitivomoteurs corticosensibles, puis un syndrome vestibulaire, une atteinte pyramidale et une ataxie d’aggravation progressive avec gêne croissante à la marche. L’altération des potentiels évoqués multimodaux, les résultats de l’analyse du liquide céphalorachidien et de l’IRM cérébrale permirent de retenir le diagnostic de SEP définie, d’évolution rémittente puis secondairement progressive. Les principales données épidémiologiques, cliniques et paracliniques de l’association SEP/NF-1 sont présentées. Les rapports entre ces 2 pathologies sont discutés et les mécanismes physiopathologiques pouvant rendre compte de cette association envisagés.

PURPOSE:To determine whether voxel-based analysis of magnetization transfer ratio (MTR) maps can provide evidence of a coherent pattern of gray matter (GM) macroscopic and microscopic tissue damage in patients at the earliest stage of multiple sclerosis (MS).MATERIALS AND METHODS:We acquired GM MTR maps in 18 patients with clinically isolated syndrome suggestive of MS (CISSMS), and 18 sex- and age-matched healthy controls. We evaluated the clinical status of the patients using the MS functional composite score and the expanded disability status scale. A two-sample t-test (P <0.0001, k=20, uncorrected for height threshold) was used to compare GM MTR maps from patients and controls on a voxel-by-voxel basis. We then extracted data from regions with t-values above the statistical threshold to verify the significance of differences using a nonparametric Mann-Whitney U-test.RESULTS:A between-groups comparison of GM maps revealed large abnormalities in the basal ganglia, including the bilateral thalamus, bilateral lenticular nucleus, bilateral head of caudate, and protuberance, and smaller abnormalities in the right insula, right BA 4, and left BA 40. The MTR measured in the left caudate and right insula was inversely correlated with duration following the first clinical event.CONCLUSION:These results suggest that although MS is a multifocal demyelinating disease that affects white matter (WM), a pattern of tissue damage is present inside the GM involving predominantly basal ganglia at the earliest stage of the disease.

Association between neurofibromatosis type 1 (NF-1) and multiple sclerosis (MS) has been very rarely described. We report the case of a 40-year-old woman presenting familial NF-1 who had cafe au lait spots and cutaneous neurofibromatosis since childhood. Five years earlier, she experienced a first episode of unilateral optic neuritis, recurrent sensory and motor disturbances, then gait ataxia and pyramidal tract dysfunction with progressive walking impairment. Altered evoked potentials, CSF analysis and cerebral MRI findings were consistent with the diagnosis of MS (secondary progressive form after relapsing-remitting phase). We review major demographic, clinical and laboratory data of MS associated with NF-1 and discuss about the potential pathophisiological mechanisms implied.