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Memory impairment, especially verbal episodic memory (VEM), represents a common ground for cognitive complaint in patients with multiple sclerosis (MS). Beyond the difficulty caused in daily life, these deficits may impact on occupational activities. Neuropsychological assessment of these patients has to include VEM tests, to describe the level of dysfunction of the different processes contributing to VEM and, if required, to guide adapted cognitive rehabilitation. The objective of the present paper is to propose a critique review of the literature on VEM abilities in MS. This review will present the conceptual references and the psychometric characteristics of the main VEM tests applied in MS (isolated tests or included within more general batteries developed specifically for MS). In a second phase, we propose an inventory of work on MS presented as a function of the cognitive processes involved. This approach provides an approach to the limitations of each conception and possible terminological ambiguities. Contributions to knowledge of MS memory impairments will be clarified, as well as the impact of the disease characteristics (MS forms, disease duration, EDSS)., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Introduction: Interest in studying swallowing disorders in patients with altered consciousness has increased over the past decade. Swallowing deficit is frequently encountered in severe brain-injured patients., State of Art: Results of studies have highlighted different factors such as the delay between the injury and the treatment and the level of consciousness of these patients, as well as the presence or not of tracheotomy, which will determine the feasibility of resuming oral feeding. Nowadays, very few valid and sensitive scales can be used to assess swallowing deficit in patients with disorders of consciousness. The Facial Oral Tract Therapy (FOTT) scale is an inter-professional multidisciplinary approach offering a structured way to evaluate and treat patients with swallowing disorders. In contrast with other scales, patients do not have to follow verbal instructions for the FOTT., Perspectives: This paper presents a review of existing literature on the assessment and management of swallowing disorders in patients with altered state of consciousness, and a description of the FOTT method., Conclusion: The FOTT seems to be an interesting assessment and rehabilitation tool for patients with disorders of consciousness. However, clinical studies are needed to confirm the validity and sensitivity of this technique., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Biofeedback is a complementary non-pharmacological and non-surgical therapeutic developed over the last thirty years in the management of drug-resistant epilepsy. Biofeedback allows learning cognitive and behavioral strategies via a psychophysiological feedback loop. Firstly, this paper describes the different types of biofeedback protocols used for the treatment of drug-refractory epilepsy and their physiological justifications. Secondly, this paper analyzes the evidence of effectiveness, from a medical point of view, on reducing the numbers of seizures, and from a neurophysiological point of view, on the changing brain activity. Electroencephalography (EEG) biofeedback (neurofeedback) protocol on sensorimotor rhythms (SMR) has been investigated in many studies, the main limitation being small sample sizes and lack of control groups. The newer neurofeedback protocol on slow cortical potential (SCP) and galvanic skin response (GSR) biofeedback protocols have been used in a smaller number of studies. But, these studies are more rigorous with larger sized samples, matched control groups, and attempts to control the placebo effect. These protocols also open the way for innovative neurophysiological researches and may predict a renewal of biofeedback techniques. Biofeedback would have legitimacy in the field of clinical drug-resistant epilepsy at the interface between therapeutic and clinical neurophysiology., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

In 2012, interest remains high in the field of dysimmune neuropathies, chiefly concerning Guillain-Barré syndrome (GBS). The pathophysiological mechanisms are now better known but electrophysiological criteria should be updated. The risk of GBS in H1N1 vaccination is now well evaluated. Nerve ultrasonography provides new prospects for diagnosis and follow-up of dysimmune neuropathies but cannot substitute for electrophysiology. This paper aims to present some noteworthy articles published in 2012 in the field of dysimmune neuropathies., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

The link between stress and migraine is complex. In its recent conception, stress is viewed as a transactional process between an individual and his-her environment in which the individual makes a response to an internal or external constraint. This paper reviews the evidence in favor of a relationship between stress and migraine. Many studies show that 50 to 80% of patients report stress as a precipitating factor for their migraine headaches. Many authors have suggested that acute stress can provoke biological modifications lowering the threshold of the individual's susceptibility to a migraine attack. It has also been shown that the incidence of migraine is higher when stress scores are higher in the previous year. This suggests that as well as being a precipitating factor of crisis, stress could also be a precipitating factor of illness in susceptible individuals. Moreover, stress can trigger migraine chronification. This has been shown in many retrospective studies and in one prospective study. Hyperalgesia and central sensitivity to pain induced by chronic stress can partly explain this phenomenon. Many retrospective studies also show that adverse events during childhood, like sexual and physical abuse, are more frequent in migraineurs than non-migraineurs. Nevertheless, there is no prospective study allowing considering a causal link between childhood abuse and migraine in adulthood. Another point that will be tackled is the comorbidity between stress related psychiatric disorders, such as post-traumatic stress disorder, and migraine. Here again, many studies conducted in huge samples from the general population are convincing. All that leads to propose stress management therapies to migraineurs. Randomized control trials and meta-analyses have shown that relaxation therapies, biofeedback and stress management cognitive behavioral therapies are effective in migraine prophylaxis, above all in children. The use of these therapies is of particular interest in association with pharmacological treatments in patients with frequent crises. However, the majority of the studies have poor methodological standards. Nevertheless, stress management therapies are proposed as prophylactic treatment in the French recommendations for migraine management., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Introduction: Because of the improvement of cancer prognosis, long-term damages of treatments become a medical and public health problem. Among the iatrogenic complications, neurological impairment is crucial to consider since motor disability and pain have a considerable impact on quality of life of long cancer survivors. However, radiation-induced neuropathies have not been the focus of great attention. The objective of this paper is to provide an updated review about the radiation-induced lesions of the peripheral nerve system., State of the Art: Radiation-induced neuropathies are characterized by their heterogeneity in both symptoms and disease course. Signs and symptoms depend on the affected structures of the peripheral nerve system (nerve roots, nerve plexus or nerve trunks). Early-onset complications are often transient and late complications are usually progressive and associated with a poor prognosis. The most frequent and well known is delayed radiation-induced brachial plexopathy, which may follow breast cancer irradiation. Radiation-induced lumbosacral radiculoplexopathy is characterized by pure or predominant lower motor neuron signs. They can be misdiagnosed, confused with amyotrophic lateral sclerosis (ALS) or with leptomeningeal metastases since nodular MRI enhancement of the nerve roots of the cauda equina and increased cerebrospinal fluid protein content can be observed. In the absence of specific markers of the link with radiotherapy, the diagnosis of post-radiation neuropathy may be difficult. Recently, a posteriori conformal radiotherapy with 3D dosimetric reconstitution has been developed to link a precise anatomical site to unexpected excess irradiation., Perspectives and Conclusion: The importance of early diagnosis of radiation-induced neuropathies is underscored by the emergence of new disease-modifying treatments. Although the pathophysiology is not fully understood, it is already possible to target radiation-induced fibrosis but also associated factors such as ischemia, oxidative stress and inflammation. A phase III trial evaluating the association of pentoxifylline, tocopherol and clodronate (PENTOCLO, NCT01291433) in radiation-induced neuropathies is now recruiting., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Parkinson's disease is mainly considered as a motor disorder defined by a motor triad. However, various non-motor manifestations may be encountered in Parkinson's disease, including hyposmia, pain, fatigue, sleep disorders, cognitive and behavioral disorders. The pathophysiology of these signs is complex, not univocal and remains poorly understood. Functional imaging techniques either by positron emission tomography, single photon emission tomography or functional magnetic resonance imaging provide an invaluable opportunity to better understand the pathophysiology of these signs. In this paper, we present a review of the recent advances provided by functional imaging in this area., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

The subthalamic nucleus deep-brain stimulation Parkinson's disease patient model seems to represent a unique opportunity for studying the functional role of the basal ganglia and notably the subthalamic nucleus in human emotional processing. Indeed, in addition to constituting a therapeutic advance for severely disabled Parkinson's disease patients, deep brain stimulation is a technique, which selectively modulates the activity of focal structures targeted by surgery. There is growing evidence of a link between emotional impairments and deep-brain stimulation of the subthalamic nucleus. In this context, according to the definition of emotional processing exposed in the companion paper available in this issue, the aim of the present review will consist in providing a synopsis of the studies that investigated the emotional disturbances observed in subthalamic nucleus deep brain stimulation Parkinson's disease patients. This review leads to the conclusion that several emotional components would be disrupted after subthalamic nucleus deep brain stimulation in Parkinson's disease: subjective feeling, neurophysiological activation, and motor expression. Finally, after a description of the limitations of this study model, we discuss the functional role of the subthalamic nucleus (and the striato-thalamo-cortical circuits in which it is involved) in emotional processing. It seems reasonable to conclude that the striato-thalamo-cortical circuits are indeed involved in emotional processing and that the subthalamic nucleus plays a central in role the human emotional architecture., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Parkinson's disease provides a useful model for studying the neural substrates of emotional processing. The striato-thalamo-cortical circuits, like the mesolimbic dopamine system that modulates their function, are thought to be involved in emotional processing. As Parkinson's disease is histopathologically characterized by the selective, progressive and chronic degeneration of the nigrostriatal and mesocorticolimbic dopamine systems, it can therefore serve as a model for assessing the functional role of these circuits in humans. In the present review, after a definition of emotional processing from a multicomponential perspective, a synopsis of the emotional disturbances observed in Parkinson's disease is proposed. Note that the studies on the affective consequences of subthalamic nucleus deep brain stimulation in Parkinson's disease were excluded from this review because the subject of a companion paper in this issue. This review leads to the conclusion that several emotional components would be disrupted in Parkinson's disease: subjective feeling, neurophysiological activation, and motor expression. We then discuss the functional roles of the striato-thalamo-cortical and mesolimbic circuits, ending with the conclusion that both these pathways are indeed involved in emotional processing., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Introduction: The contribution of stroke units to improve morbidity, mortality and recovery in stroke victims is clearly demonstrated. However, acute management of language disorders in these specialized units is controversial and little standardization is seen for the management of swallowing disorders., State of the Art: The recently validated French scale for rapid screening for language disorders (LAST) in acute stroke patients should enable optimal detection and early management. A standardized protocol should be used to screen for and manage swallowing disorders. This protocol should include daily evaluations, individually tailored rehabilitation sessions, adaptation of food textures, patient education for adequate eating position, team training, and information for families., Perspectives and Conclusions: These protocols imply co-operation and coordination between the medical and allied profession teams and the daily presence of a speech and language therapist. This presence is crucial for patients in stroke units to achieve the full benefits of the management scheme proposed in this paper., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

The purpose of this paper is to highlight the difficulties of applying neuroepidemiological methods in low income countries or developing countries, which are mostly tropical countries, taking advantage of the experience of the Institute of Neuroepidemiology and Tropical Neurology, which was created in Limoges in 1982. These difficulties could be related to several aspects: methodological, logistical, political or economical, linked to ethical issues, even difficulties to publish the studies. However, concept and neuroepidemiological methods should stay the same worldwide, even if their translation into practice could sometimes raise some problems in developing countries. Study protocol should be more detailed. Some specific epidemiological methods could be useful. Collection of data should be standardized. True cooperation at every level is needed for these researches to be valid., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Many papers have been published in the field of immune-mediated neuropathies in 2010. Various topics have been covered: diagnostic criteria and clinical forms, pain and its risk factors, clinical evaluation and new immunological markers. Additionally, as nerve biopsy is still useful for evaluating patients with peripheral neuropathy, French and international guidelines have been published. This paper aims to summarize recent discoveries in the field of immune-mediated neuropathies., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Cognitive impairment is now well-known in multiple sclerosis (MS). However, few rehabilitation interventions are proposed or really efficient., Objectives: To present a review of cognitive rehabilitation intervention research conducted in people with multiple sclerosis (MS), regarding different findings about episodic memory, working memory, attention and executive function disorders in MS., Data Sources: A search of Medline (yield 20 papers) and of PsychInfo (yield 1 article), using combinations of the following terms: cognitive rehabilitation, multiple sclerosis, cognitive therapy, neuropsychological rehabilitation, in the title or in the abstract, from 1960 to March 2010, excluding animal studies., Results: Episodic memory rehabilitation studies appear to be promising. Programs on working memory, attention and executive functions are in the very early phases., Conclusions: Results are encouraging and allow specific recommendations for future research about: (1) inclusion criteria, often not defined, (2) a specific baseline adapted to the program of rehabilitation, (3) a control measure regarding program efficiency and (4) a role for the psychologist (presence and advice during the program)., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Carotid-cavernous fistulas are abnormal communications between the carotid system and the cavernous sinus. Depending on their direct or indirect nature, they have different clinical manifestations and management. Are discussed in this paper: first the anatomy of the cavernous sinus, then clinical signs, diagnosis, management and differential diagnosis of carotid-cavernous fistulas., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Inflammatory optic neuritis (ON) represents a frequent clinical situation in neurology and ophthalmology. When MRI and CSF analysis are normal, ON is considered idiopathic with a suspected viral etiology. However, in several cases either a recurrence or a myelitis may occur. In the first case, it is relapsing inflammatory optic neuritis (RION) and in the second case it is neuromyelitis optica (NMO). Nevertheless, predictive criteria of a recurrence or an extension of the disease to spinal cord remains unknown, excepted for anti-NMO IgG antibodies which are probably highly specific for a future evolution to NMO. In the present paper, the authors successively present the two clinical situations (RION and NMO) and attempt to summarize diagnostic and prognostic criteria., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Genetics and exposure to toxins constitute the main determinants in the onset of Parkinson's disease (PD). At least, 13 loci and nine genes involved in familial and sporadic forms have been described. A significant association between occupational exposure to pesticides (especially insecticides) and PD has been confirmed recently with rare cases even being recognized as occupational disease. We develop in this paper a practical approach for such situations where a common genetic or toxic origin is suggested. Such an approach can be applied very broadly using case by case study then further analysis in a specialized center of reference in the field of genetics or occupational diseases and the environment. A pedigree needs to be drawn to evaluate a potential genetic factor with, if possible, the examination of various family members. Depending on the mode of inheritance, age of disease onset and phenotypic expression, genetic analysis will be carried out (mainly the study of parkin gene for recessive transmission and LRRK2 gene for dominant transmission). The evaluation of a toxic factor is more difficult because its direct involvement may not always be defined with certainty, the collection of information is more complex (product list, causal relationship, protection system used...). The course of action will identify the existence of a potential risk factor particularly in patients at risk (farmers, workers in a factory using heavy metals) by considering secondary specialized consultation with the occupational physician or pathology consultation work for possible development of a procedure for recognition of occupational disease., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

This paper, written by French amyotrophic lateral sclerosis (ALS) center experts, presents an update of recent advances in fundamental, epidemiological and clinical research in ALS based on a review of the literature between September 2008 and November 2009. Among other pathophysiological mechanisms, the role of stress of the endoplasmic reticulum and the importance of energetic metabolic disturbances have been underscored. In the field of genetics, research has been advanced through the identification of mutations of the gene FUsed in Sarcoma/Translated in LipoSarcoma (FUS/TLS) in individuals with familial and sporadic ALS. This gene is involved in the regulation of transcription, splicing and RNA transport, and has functional homology to another ALS gene, TARDBP, which suggests that a common mechanism may underlie motor neuron degeneration. A report showed that mice expressing a mutant form of human TDP-43 develop a progressive and fatal neurodegenerative disease reminiscent of both ALS and frontotemporal lobar degeneration with ubiquitin aggregates (FTLD-U), providing a new animal model that may help to better understand the pathophysiology and test new therapeutics. Beside genetic studies, several epidemiologic studies have investigated the role of environmental factors. A recent study suggests that smoking is a risk factor for developing ALS and it is hypothesized that this could occur through lipid peroxidation via formaldehyde exposure. From a neuroprotective perspective, trials with IGF-1, sodium valproate, coenzyme Q or glatiramer acetate have failed to demonstrate any beneficial effect. A study published in 2008 argued that lithium may have a neuroprotective effect in ALS mice and also in patients. However, two preclinical studies failed to replicate the neuroprotective effect of lithium in ALS mice. Therapeutic trials have been performed or are currently ongoing in Europe and North America. Their results have not yet been published., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Since the first consensus papers published early in the 2000s, a growing number of recent publications has shown that adapted physical activity is not only safe in the context of myopathy but also potentially effective as a therapeutic tool. After a short recall of the different exercise modalities, the mechanical strain they induce and the expected muscular benefits, the present paper reviews the different studies related to exercise therapy in myopathic patients and provides a critical analysis of the topic. Myopathies are rare diseases with many different etiologies and a large number of training modalities which could be useful for the different muscular challenges have been proposed. We have chosen to focus on several specific training modalities and to discuss the results from the most recent papers. The purpose of this review is to, firstly, update physical training guidelines for patients with myopathy and, secondly, highlight some common pitfalls associated with this strategy. This is particularly important for medical and allied professionals involved in prescribing and managing exercise therapy protocols., (Copyright (c) 2009 Elsevier Masson SAS. All rights reserved.)

Normal pressure hydrocephalus must be considered when gait disturbance, cognitive impairment and sphincter disorders are associated. Symptoms of normal pressure hydrocephalus, especially gait disturbance, are potentially curable by surgery. Our paper provides a summary review of gait disorders associated with normal pressure hydrocephalus detailing their characteristics and the best assessment methods. Although the pathogenic mechanisms underlying normal pressure hydrocephalus remain poorly understood, advances in imaging have enabled considerable progress in our fundamental knowledge of the condition. Tapping the cerebrospinal fluid by lumbar puncture or external lumbar drainage remains the diagnostic test and is predictive of a favorable response to surgical treatment. Clinical severity scores validated for walking and for sphincter dysfunction and cognitive disorders provide the best means of assessing each patient's response to treatment., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)

From one year of age, gait and balance disorders are frequent complaints in neurology. During the first year following the acquisition of independent walking, most of the gait, such as transition from digitigrade to plantigrade locomotion, parameters are well-established in children. Nevertheless, bipedal locomotion means solving a large number of balance problems. Locomotor balance needs many years to mature in the course of ontogenesis. From various gait analysis, it was possible to establish a repertoire of locomotor strategies used in typical and pathological development. The last part of this paper is devoted to the possible responses that can be proposed for gait and balance disorders occurring during childhood., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

Astasia-abasia is defined as the inability to stand and to walk, despite sparing of motor function underlying the required balance and gestures. Initially, astasia-abasia was considered a psychogenic gait disorder, but later on, the description of "high-order" gait disorders mimicking this pure functional deficit led authors to refer to "astasia-abasia" as a pure descriptive term, without a presupposed etiological or anatomical substrate. In this paper, the main clinical characteristics of both psychogenic and non-psychogenic astasia-abasia are presented and discussed., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)

Introduction: The relationship between visual perception and visual mental imagery are at the center of a lively theoretical debate between those postulating common neurocognitive processes between perception and imagery and those who emphasize the differences between these two entities. Neuropsychology can make an important contribution to this debate, by assessing associations and dissociations between perceptual and imaginal deficits in patients with brain damage. However, currently there is no standardized test battery available for such assessments., Material and Methods: Here we present a battery of paper-and-pencil tests assessing different domains of visual mental imagery and visual perception abilities: object form and color, animals, orthographic material, numbers, faces, and space. We also explored the effects of age, educational level and gender on performance on a group of 103 participants free of neurological damage., Results: The battery includes two parts: one composed of 14 tests assessing mental imagery and the second part composed of eight tests assessing the abilities of visual perception. We calculated the correlations between the tests, and found that, with the exception of orthographic material, there were generally poor correlations between imagery and perceptual tests., Conclusion: This result seems inconsistent with hypotheses postulating a strict correspondence between perceptual and imagery abilities.

Background: Over the past 20 years, the neurology specialty has changed because of the explosion of information and new treatment modalities that became available, and has consequently become more complex and diversified. The satisfaction of residents concerning their training and the competencies that they acquire has never been thoroughly assessed in France., Methods: We conducted a national survey in order to assess (1) the methods for training and validation; (2) the level of knowledge that residents perceived to have acquired in different domains; and (3) their satisfaction towards training and their wishes., Results: One hundred and eight residents replied to the survey. The main sources of training were local teaching (74%), personal work (61%), scientific (57%) and didactic (54%) papers. Residents seemed unable to acquire knowledge on all domains of the curriculum established at a national level, particularly for neurophysiology, neuropsychology, comatose state and sleep disorders, oncology and psychiatry. Even postgraduate year four residents were not fully competent with several technical tools, particularly neurophysiological tests. Fifty eight percent of residents were satisfied with their training, but 16% were not and 26% were half-hearted. Overall the residents were in favor of more standardization in their training, national-level certification, and would be keen on having access to clinics and the use of a follow-up monitoring chart., Conclusion: Our results suggest that it would be useful to better identify the competencies that every neurologist should acquire and to use appropriate tools to reach these objectives.

This paper from a group of French experts in amyotrophic lateral sclerosis (ALS) presents an update of recent advances in fundamental, epidemiological and clinical research in ALS. Recent development in the pathogenesis of ALS suggests that motor neuron degeneration is a multifactorial and noncell autonomous process. Research has been advanced through the identification of the TAR-DNA-binding protein (TDP-43) as a common neuropathological marker of ALS and frontotemporal lobar degeneration with ubiquitin-positive inclusions. Recently, mutations in the TDP-43 gene have been described in individuals with familial and sporadic ALS. Fundamental research in ALS is expected to lead to the disclosure of new diagnostic markers and therapeutic targets. A small trial has suggested that lithium carbonate may slow ALS progression but larger trials will be needed to confirm these results.

Myelitis secondary to systemic disease is a rare condition but clinical involvement is frequently severe. In case of a well-known systemic disease before myelitis diagnosis is relatively easy to perform. On contrary, when the systemic disease is not known previously the management is more difficult. This is of importance for therapeutical considerations. In the present paper we review the topic of acute myelitis secondary to systemic diseases.

The most frequent ophthalmologic sign in multiple sclerosis (MS) is optic neuritis (ON), but internuclear ophthalmoplegia, nystagmus, and ocular motor nerve palsies are also observed. This paper describes the most important signs suggesting an inflammatory origin of ON, most particularly MS. We detail new laboratory exams such as optic coherence tomography (OCT), which could be of potential interest for MS patients. Finally, we summarize the prognosis factors for disability in MS after a first episode of ON.

Introduction: Post-ictal psychosis syndrome (PIP) belongs to the group of epileptic psychoses which, according to the most commonly used classification, is to be distinguished from ictal psychoses, on one hand, and from inter-ictal psychoses, on the other., Objectives: The present paper aims to review recent data concerning the clinical, therapeutic and pathophysiological aspects of PIP., Methods: We report four cases of PPI, which involved four patients hospitalized at the Salpêtrière hospital between 2001 and 2005, and discuss these cases in light of the relevant literature., Results: The PIP fit generally occurs in patients suffering from intractable temporal lobe epilepsy which started several years before. The psychiatric disorders suddenly take hold after a series of complex partial seizures with frequent secondarily generalized tonic clonic seizures. During the episode, EEG recordings do not show any epileptic activity. Psychiatric symptoms consist of persecutory delusive ideas, verbal and visual hallucinations, agitation, and aggressiveness. Mood disorders are variable from one patient to another and exhibit intraindividual fluctuation. In most patients given antipsychotic drugs, the short-term outcome of PIP is favorable . In the long-term, even if recurrence is the main long-term risk, progression to severe mood disorders or to poor prognosis inter-ictal psychosis is possible. Accordingly, the clinician must be aware of this syndrome in order to correctly diagnose PIP since effective treatment with antipsychotic drugs is available., Conclusion: The short-term prognosis of PPI is usually favorable but this syndrome can potentially develop in the long-term to more severe psychiatric disorders. It is, therefore, important to recognize PIP syndromes which respond more readily to pharmacological treatments than other types of psychoses.

Introduction: Several extra-intestinal manifestations of inflammatory bowel disease have been reported, although reports of neurologic disorders are rare., Case Reports: We report two cases of peripheral and central neurological manifestations of inflammatory bowel disease. The first case is a 33-year-old woman who developed acute inflammatory demyelinating motor polyneuropathy in association with Crohn's disease. Her symptoms improved with immunomodulatory therapies. Our second case is a 46-year-old woman, previously diagnosed with ulcerative colitis that subsequently developed ischemic stroke concomitantly to severe exacerbation of digestive symptoms. Her radial and humeral pulses were unequal bilaterally. Associated Takayashu's arteritis was suspected. The cardiovascular and immunological assessments were negative. Complementary investigations were foreseen but the patient died early of acute abundant rectal bleeding., Conclusion: This paper focuses essentially on the probable manifestations of inflammatory bowel disease in the nervous system, particularly peripheral neuropathies and stroke. We discuss different pathophysiologic mechanisms incriminated, notably thromboembolism and immunological abnormalities.

The organization of episodic and semantic memory is currently debated, and especially the rule of the hippocampus in the functioning of these two systems. Since theories derived from the observation of the famous patient HM, that highlighted the involvement of this structure in these two systems, numerous studies questioned the implication of the hippocampus in learning a new semantic knowledge. Among these studies, we found Vargha-Kadem's cases of developmental amnesia. In spite of their clear hippocampal atrophy and a massive impairment of episodic memory, these children were able to acquire de novo new semantic knowledge. In the present paper, we describe a new case of developmental amnesia characteristic of this syndrome. In conclusion, the whole published data question the implication of the hippocampus in every semantic learning and suggest the existence of a neocortical network, slower and that needs more exposures to semantic stimuli than the hippocampal one, which can supply a massive hippocampal impairment.

During these last 30 years, cognitive rehabilitation has accomplished dramatic improvement. In this paper, we review progress in four main domains: the development of pragmatic and ecological approaches in neuropsychological rehabilitation; the development of computerised rehabilitation; rehabilitation of executive functions; cognitive rehabilitation in degenerative dementia. Finally, we present a single-case study, recently published elsewhere, showing the effectiveness of rehabilitation of verbal working memory in a patient with left hemisphere stroke. In addition, future issues for rehabilitation research are presented. The development of bottom-up rehabilitation strategies as well as the use of inter-hemispheric interactions appears as future promising tracks for clinical rehabilitation.

With age, the brain undergoes both structural and functional alterations. Overall, the literature consistently reports global brain atrophy in normal adults, generally more pronounced in frontal areas. As a result of different methodologies and inclusion criteria, other brain areas have been the matter of conflicting findings, notably the hippocampus. Regarding resting-state PET studies, they have consistently highlighted a metabolic deterioration of the frontal and anterior cingulated cortices. By contrast, relatively few investigations have sought to identify those areas that remain intact with aging, or undergo the least deterioration. We report a study designed to establish a comprehensive profile of both structural and functional changes in the aging brain, using up-to-date voxel-based methodology in 45 optimally healthy subjects aged 20-83 years. One of the main findings is that the lesser structural deterioration of the anterior hippocampal region, together with the lesser functional alteration of the posterior cingulate cortex, appear to mark the parting of the ways between normal aging and Alzheimer's disease, which is characterized by early and prominent deterioration of both structures. This paper also deals with studies set out to establish the relationship between changes in episodic memory retrieval in normal aging on the one hand and gray matter volume and 18FDG uptake on the other hand. Frontal areas dysfunction is involved in memory decline in older subjects, at least in some conditions, a finding which clearly contrasts with that found in Alzheimer's disease where the hippocampus plays a key role. Finally, compensatory mechanisms are reviewed through activation studies which often show supplementary activations in old subjects compared to young as well as in Alzheimer's disease patients compared to healthy elderly subjects. Paradoxically, those mechanisms seem to be underpinned, at least partially, by frontal areas in both populations, but researches are needed to better identify which subregions are involved.

Multiple sclerosis (MS) is a chronic progressive disease which is the leading cause, after road traffic accidents, of handicap in young subjects. The large range of symptoms associated with MS lead to continuing decline in mood and quality of life. Despite therapeutic advances, functional impairments have significant consequences. Neurorehabilitation can be highly contributive in this disease with the goals of increasing independence and quality-of-life and improving functional capacities. Individualized programs elaborated by a multidisciplinary team of experts are the key to success of rehabilitation. Assessment is difficult because of the underlying conflict between the philosophies of rehabilitation and evidence-based medicine. The aim of this paper is to provide an overview of MS rehabilitation. Physical exercise is safe and should be encouraged for people with MS. Some studies have shown that supervised exercises have a beneficial effect on MS disability and quality of life. Inpatient rehabilitation for MS yields short-term benefits in function, mobility and quality of life; periodic hospitalization may be needed. In the future, rehabilitation professionals will have to learn how to anticipate patient needs and lay the groundwork for services and equipment in advance. Rehabilitation is one of the treatments of MS patients and should be viewed as an ongoing process to maintain and restore maximum function and quality of life.

Oscillopsia is an illusion of an unstable visual world. It is associated with poor visual acuity and is a disabling and stressful symptom reported by numerous patients with neurological disorders. The goal of this paper is to review the physiology of the systems subserving stable vision, the various pathophysiological mechanisms of oscillopsia and the different treatments available. Visual stability is conditioned by two factors. First, images of the seen world projected onto the retina have to be stable, a sine qua non condition for foveal discriminative function. Vestibulo-ocular and optokinetic reflexes act to stabilize the retinal images during head displacements; ocular fixation tends to limit the occurrence of micro ocular movements during gazing; a specific system also acts to maintain the eyes stable during eccentric gaze. Second, although we voluntary move our gaze (body, head and eye displacements), the visual world is normally perceived as stable, a phenomenon known as space constancy. Indeed, complex cognitive processes compensate for the two sensory consequences of gaze displacement, namely an oppositely-directed retinal drift and a change in the relationship between retinal and spatial (or subject-centered) coordinates of the visual scene. In patients, oscillopsia most often results from abnormal eye movements which cause excessive motion of images on the retina, such as nystagmus or saccadic intrusions or from an impaired vestibulo-ocular reflex. Understanding the exact mechanisms of impaired eye stability may lead to the different treatment options that have been documented in recent years. Oscillopsia could also result from an impairment of spatial constancy mechanisms that in normal condition compensate for gaze displacements, but clinical data in this case are scarce. However, we suggest that some visuo-perceptive deficits consecutive to temporo-parietal lesions resemble oscillopsia and could result from a deficit in elaborating spatial constancy.

Brain death diagnosis is based upon several clinical and paraclinical criteria that have been legally defined. There is a medico-legal protocol when brain death diagnosis is made in order to allow organ removal for a possible transplantation. In France, EEG or cerebral arteriography must legally be used to confirm a clinical brain death suspicion. There is a specific procedure to perform an EEG to confirm the diagnosis of brain death. However all the criteria have been made using conventional paper EEG, while numerized is now used. The comparison of EEG recording using both analogical and numerised acquisition allow us to report several recommendations to use EEG for brain death diagnosis.

Faces represent a crucial vector of interhuman communication. The message transmitted by the face has multiple features. Recognition of each feature can be impaired independently or in combination with others. In order to understand the behavioral consequences of such impairments, which can be a major social handicap, we first must specify the neural networks involved in face recognition. We propose in this first part to present the systems involved in face recognition, in particular the question of identity and prosopagnosia. Different neural networks are indeed implicated in the recognition of invariant facial features such as identity, gender, ethnicity, and recognition of variant features like facial expression and eye gaze. This paper is illustrated by some of our scalp and intracranial electrophysiological studies performed in humans allowing us to describe some aspects of face recognition dynamics combining an excellent spatial and temporal resolution. Intracranial recordings were performed in drug refractory epileptical patients implanted with depth electrodes. These studies demonstrate that numerous deep brain and cortical structures participate early and sometimes in a sustained manner in face recognition.

Introduction: Multiple sclerosis (MS) is a major cause of neurological disability among young adults. The cognitive disorders are the second cause of alteration of quality of life after physical handicap and are often responsible for loss of social-occupational adaptability. The prevalence of cognitive disorders is 40 to 65%. The alteration of executive functions predominates whereas instrumental functions are generally preserved. The assessment of these disorders is often underestimated by the usual battery of neuropsychological tests. However, the link between psychometric results and executive difficulties of daily life is uncertain., Objectives: To evaluate the sensitivity of an ecological test compared to standard psychometric tests in assessment of executive disorders in MS., Methods: Twenty subjects with clinically definite MS were matched for age, sex and pre-morbid intellectual level with control subjects. A battery of neuropsychological and ecological tests was applied to all subjects. The performances on these tests formed a global score of executive function (SFE). The "paper and pencil" multiple errands test was used as the ecological test to examine planning and goal-oriented behavior. We also assessed fatigue and depression with the Fatigue Severity Scale and the Beck Depression Inventory., Results: There was no significant differences between MS patients and controls in neuropsychological executive tests, except for verbal fluencies (p=0.01). The performances were significantly decreased in the MS group for the multiple errands test (p=0.01). 75% of MS subjects have a pathological score for this test. There was a significant link between the performances with this test and SFE (p=0.009)., Conclusions: Executive disorders are underestimated in MS. However, we suggest that an ecological approach is more reliable than standard neuropsychological tests to estimate the cognitive difficulties in daily life in MS subjects. The results of our study favor further research to ascertain the usefulness of ecological assessment in MS.

Memory disorders observed in Alzheimer's disease gave rise, from the eighties, to a detailed analysis into the framework of cognitive neuropsychology which aimed at describing the deficits of very specific processes. Beyond their clinical interest, these studies contributed to the modelisation of human memory thanks to the characterization of different memory systems and their relationships. The first part of this paper gives an overview of the memory deficits in Alzheimer's disease and insists on particular cognitive phenomena. Hence, several examples are developed in the domains of semantic memory (such as hyperpriming and hypopriming effects) and autobiographical memory. Recent results highlight the existence of severe autobiographical amnesia observed in all neurodegenerative diseases, though with contrasting profiles: Ribot's gradient in Alzheimer's disease (showing that remote memories are better preserved than recent ones), reverse gradient in semantic dementia and no clear gradient in the frontal variant of frontotemporal dementia. The second part of this article presents advances in cognitive neuroscience searching to disclose the cerebral substrates of these cognitive deficits in Alzheimer's disease. The studies using functional imaging techniques are the most informative regarding this problematic. While showing the dysfunctions of an extended network, they emphasize the selectivity of cerebral damages that are at the root of very specific cognitive dysfunctions, coming close in that way to the conceptions of cognitive neuropsychology. These neuroimaging studies unravel the existence of compensatory mechanisms, which until recently were clearly missing in the literature on neurodegenerative diseases. These different researches lead to a wide conception of human memory, not just limited to simple instrumental processes (encoding, storage, retrieval), but necessarily covering models of identity and continuity of the subject, which interact in a dynamic way with eminently changing memory representations.

Bulbar involvement is usual in patients with amyotrophic lateral sclerosis (ALS), leading to a progressive loss of speech, phonation and swallowing abilities. The goal of the speech therapy is to maintain, as long as possible, those abilities and to devise alternative communication strategies when oral communication is ineffective. This paper presents guidelines for speech therapy during disease progression.

Although noninvasive ventilation may improve survival in patients with amyotrophic lateral sclerosis (ALS), ineffective airway clearance is an important cause of therapeutic failure. We report in this paper the main studies which have assessed assisted cough techniques in patients with ALS. Manually assisted cough (in particular with previous air stacking) and mechanical insufflation/exsufflation may significantly increase cough peak flow. Characteristics, limitations and long-term benefits of these techniques are also discussed.

Amyotrophic lateral sclerosis (ALS) is a progressive degeneration of upper and lower motor neurons. In the absence of any validated biological marker, the diagnosis of ALS depends upon recognition of characteristic symptoms and signs together with supportive electrophysiological findings. The diagnosis of ALS is easy to recognize in its fully developed form but during the early stages both false positive and false negative diagnoses are common. In clinical practice, diagnostic difficulties mostly arise with patients who present either with only upper motor neuron, or with only lower motor neuron signs. It may be difficult to distinguish ALS with clinically predominant lower motor neuron involvement from alternative diagnoses including spinal atrophies of adult onset, Kennedy's disease, inclusion body myositis and motor neuropathies with conduction blocks. The diagnosis of ALS related syndromes (progressive muscular atrophy, primary lateral sclerosis and progressive bulbar palsy) requires the elimination of alternate diagnoses. This paper reviews the main characteristics of diseases mimicking ALS and the atypical subsets of ALS.

The hallmark of progression in patients with amyotrophic lateral sclerosis (ALS) is the development of progressive weakness and muscular wasting. Strength testing has been used to monitor the course of the disease and to test the efficacy of new drugs. This paper review the methods used to quantify the weakness and compare their accuracy and reproducibility.

In amyotrophic lateral sclerosis (ALS) announcement of the diagnosis is an important step in the process of comprehensive care. The patterns of psychological reactions following bad news must be considered with precaution. The neurologist must take into consideration the specific aspects of announcing familial ALS. This paper reviews the modalities of announcement of the diagnosis and course in ALS patients.

Respiratory muscle weakness represents the major cause of mortality in patients with amyotrophic lateral sclerosis (ALS). As a result, ventilatory assistance is an important part of disease management. Nowadays, noninvasive ventilation (NIV) has become the first choice modality for most patients and represents an alternative to tracheostomy intermittent positive-pressure ventilation. Although, some consensus guidelines have been proposed to initiate NIV in patients with restrictive chronic respiratory failure, these criteria are discussed regarding ALS. While the current consensus recommends that NIV may be used in symptomatic patients with hypercapnia or forced vital capacity<50p.cent of predicted value, early use of NIV is proposed in the literature and reported in this paper.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving primarily motor neurons in the cerebral cortex, brainstem and spinal cord. In the absence of any biological marker, the diagnosis of ALS is based on clinical analysis, combined with the results of electromyography. Consensus diagnosis criteria (El Escorial criteria) have been developed to define workable and internationally acceptable guidelines for the diagnosis of ALS. A combination of lower and upper motor neuron signs with evidence of spread within a region or to other regions is required. The diagnosis of ALS has been categorized into various levels of certainty depending on the presence of upper motor neuron and lower motor neuron signs together in the same topographical anatomic region (brainstem and cervical, thoracic or lumbosacral spinal cord). Clinical types and patterns of ALS have been defined. The emerging concept of "ALS plus" is characterized by the presence of atypical clinical features, e.g. extrapyramidal signs or dementia, in association with the classical phenotype of ALS. This paper reviews the classical and atypical clinical features of ALS.

Amyotrophic lateral sclerosis (ALS) is characterized by significant clinical variability. Different subsets are classically individualized: bulbar onset and limb onset ALS, sporadic and familial ALS, ALS-plus syndromes (characterized by the presence of atypical clinical features, e.g. extrapyramidal signs or dementia, in association with the classical phenotype of ALS) and Western Pacific ALS. In addition, ALS-related syndromes include progressive muscular atrophy, primary lateral sclerosis and progressive bulbar palsy. The recognition of ALS subsets and ALS-related syndromes is important in clinical practice since the prognosis may vary depending on the clinical presentation. The prognosis of bulbar-onset ALS is poor compared with the spinal-onset type. Primary lateral sclerosis, defined by pure upper motor neuron findings, has a more benign course than classical ALS. It has also important implications for therapeutic trials to ensure the homogeneity of clinical material since inclusion of atypical forms with different prognoses can skew the outcome analysis. This paper reviews the clinical characteristics of the ALS subsets and ALS-related syndromes.

Multiple sclerosis (MS) has been described for more than a century, but its cause remains unknown. Numerous reports have been written concerning borderline types of the disease. In the present paper we present the pseudo-tumoral variants of MS (so called Balo's, Marburg's and Schilder's forms), demographic variants (young and elderly onset of MS), related disorders (neuromyelitis optica and acute demyelinating encephalomyelitis). We also discuss the differential diagnosis with other auto-immune diseases.

Over the last years, technical advances in neuroimaging have allowed drastic improvements in the assessment of acute ischemic cerebral events. Beyond conventional morphological analysis, diffusion-weighted and perfusion-weighted MRI now enable routine functional assessment of brain tissue; spectroscopy and diffusion tensor imaging still remains in the domain of clinical research. During acute ischemia events, diffusion-weighted MRI can detect the movements of water molecules and cytotoxic edema related to cell injury enabling rapid diagnosis and early assessment of cerebral ischemia. In conjunction with perfusion imaging, which detects hypoperfusion areas, diffusion-weighted MRI provides a means to identify areas of penumbra ischemia. More recent multislice computed tomographic (CT) scans with multimodal analysis are also very competitive for assessment of cerebral ischemia (non-enhanced CT, CT angiography and perfusion CT). The purpose of this paper is to describe the CT and MRI patterns during the different stages of cerebral infarcts.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) comprises a group of dysimmune neuropathies easily diagnosed in more than half of the patients. Diagnosis is based on clinical, electrophysiological and biological clues. In some patients, diagnosis is unclear because of the debated value of the available clues. In such circumstances, dysimmune neuropathies may not be diagnosed, leading to insufficient treatment. This is an important category of patients because immunomodulatory drugs have proven efficacy. The CIDP spectrum includes a relatively wide range of diseases. Besides the easily recognized classic forms, there are many clinical variants, sometimes with a paucisymptomatic presentation leading to uncertain diagnosis. The French CIDP study group has established guidelines for diagnostic strategy in CIDP patients. The first part of this paper is devoted to the clinical aspects of the disease, classical forms and variants. In the second part, the results of electrophysiological studies are reported. In a third chapter, complementary examinations useful for diagnosis are discussed. The fourth chapter deals with the diagnostic strategy, discussed in relation to the different situations which may be encountered in clinical practice. details the technical modalities of appropriate electrophysiological studies and presents normal results together with those indicating demyelinating neuropathy. Nerve biopsy technique and results are given in appendix II.