Réorganisation du cortex sensorimoteur dans le cadre de la paralysie cérébrale unilatérale : apports des neurosciences

Cerebral palsy (CP) is a non-progressive injury to the developing central nervous system and defines as permanent disorders of the development of movement and posture, causing activity limitation. This neurodevelopmental disorder may lead to spastic unilateral cerebral palsy after early unilateral brain lesions. Physical and rehabilitation medicine has a particular interest in the study of organization and reorganization of the sensorimotor cortex following early brain injury. From neuroscience standpoint, early brain lesions have been shown to induce substantial neural reorganization owing to the higher plasticity in the developing brain. Unilateral injuries either to the motor cortex or the corticospinal tract can lead to different patterns of reorganization of the sensorimotor cortex. Many patients develop ipsilateral corticospinal pathways to control the paretic hand with the non-lesioned hemisphere. This type of reorganization is often observed following unilateral periventricular brain lesions, which damage the corticospinal tracts in the periventricular white matter. In this group of patients, the primary motor cortex has been found to be represented in the non-lesioned precentral gyrus ipsilateral to the paretic side. Inversely, in patients with perinatal unilateral middle cerebral artery stroke, primary motor cortex remains organised in the lesioned precentral gyrus contralateral to the paretic hand. However, regardless of these inter- or intrahemispheric motor representations, the primary somatosensory cortex representation remains in the lesioned hemisphere in both groups. These two types of corticospinal reorganization could influence the efficacy of rehabilitation.     

Stimulation cérébrale profonde et troubles de la marche dans la maladie de Parkinson

Introduction

Gait disorders and freezing of gait (FOG) are seen in most patients with advanced Parkinson disease. Response to levodopa and deep brain stimulation is variable across patients.

State of art

Thalamic stimulation is ineffective on gait and can even worsen balance when bilaterally applied. Pallidal stimulation moderately improves gait disorders and FOG although this effect tends to wane after three to five years. Stimulation of the subthalamic nucleus (STN) improves levodopa-responsive gait disorders and FOG. However, some patients worsen after STN stimulation and others are better improved under levodopa than under STN stimulation. Synergistic effects of the two treatments have been reported. As for pallidal stimulation, there is a failure of long-term STN stimulation to improve gait, probably due to the involvement of non-dopaminergic pathways as the disease progresses. Levodopa-resistant gait disorders and FOG do not usually benefit from STN stimulation. In the rare cases of levodopa-induced FOG, STN stimulation may be indirectly effective, as it enables reduction or arrest of the levodopa treatment.

Perspectives

Pedunculopontine nucleus stimulation has recently been performed in small groups of patients with disabling gait disorders and FOG. Although encouraging, the first results need to be confirmed by controlled studies involving larger series of patients.

Conclusions

Overall, gait disorders remain a motor PD symptom that is little improved, or only temporarily, by current pharmacological and surgical treatments. Patient management is complex.     

Apnée du sommeil et paralysie cérébrale à l’âge adulte. À propos de trois observations

Respiratory disorders are frequent in cerebral palsy with restrictive and also obstructive disorders. Obstructive sleep apnea has been described in childhood but there are few data about this problem in adults. Usually clinical symptoms are snoring, apneas, nycturia, sleepiness, awakenings, headache, cognitive disorders, and depressive syndrome. Obesity is a strong risk factor. Moreover, prevalence increases with age in adults. We report three cases of adults with cerebral palsy suffering from obstructive sleep apnea. Particularities of this syndrome are discussed in cerebral palsy. Diagnosis may be difficult because of the existing impairment, which can mask specific clinical signs. According to the risk of impairment worsening and of cardiovascular disease, obstructive sleep apnea must be evoked in case of any doubt. Individuals who are suspected of having obstructive sleep apnea should undergo polysomnography to confirm the presence and severity of sleep disordered breathing.     

Thrombolyse in situ dans la thrombose veineuse cérébrale

Introduction

Cerebral venous thrombosis (CVT) is uncommon with a variable clinical presentation and an unpredictable outcome. Heparin is used for first-line treatment in association with symptomatic and etiologic management. Despite adequate anticoagulation, the condition may deteriorate in some patients warranting the use of local thrombolysis (LT) known for good efficacy and safety. But there are few cases and trials upon which to base guidelines for the use of LT.

Methods

A retrospective review of the medical and radiological records of patients with CVT was managed in the Caen hospital over a six-year period. We compared clinical factors of poor prognosis and radiological findings according to treatment delivered.

Results

Thirty-six patients are treated for CVT. LT was performed in eight of them; dose-adjusted intravenous heparin was the only treatment in the 28 others. Good outcome was achieved in two thirds of the patients with functional sinus patency in all cases. Based on an analysis of the radiological data of the 36 patients, we propose a summary of radiological risk factors associated with a worsening condition despite adequate anticoagulation.

Discussion/Conclusion

Based on our experience and a review of the literature which includes 98 previous cases, LT appears to be a relative effective end safe procedure even in the presence of a hemorrhagic infarct. The treatment by LT should be considered in patients who present clinical criteria of gravity and radiological risk factors associated with failure of heparin treatment. The usefulness of LT remains to be determined in a randomized trial comparing heparin alone and heparin associated with LT.     

Aspergillose cérébrale chez le sujet immunocompétent. À propos de trois cas

Introduction

Cerebral aspergillosis is a severe disease most commonly suspected in immunocompromised patients.

Case report

We report herein three cases of cerebral aspergillosis in immunocompetent adults. Sinus involvement was noted in two cases, but there was no extracerebral involvement in the third case. Mycology samples provided the diagnosis in two cases. In the third case, cerebral imaging visualized a tumor; the patient underwent surgery and the pathology exam of the surgical specimen established the diagnosis. All patients were given antifungal treatment and achieved a good outcome.

Conclusion

Cerebral aspergillosis is highly uncommon in immunocompetent patients. In addition to immunodepression, the notion of pulmonary or ENT involvement may be suggestive. In the brain, aspergillosis mainly involves the basal nuclei and the thalamus.     

Actualités et perspectives dans la prise en charge de l’hémiplégie cérébrale infantile en médecine physique et de réadaptation

Hemiplegic (or spastic unilateral) cerebral palsy accounts for about 30% of all cases of cerebral palsy. With a population prevalence of 0.6 per 1000 living births, it is the most common form of cerebral palsy among children born at term and the second most common type among preterm infants. Precocity of care at observation of the first symptoms allows preventing orthopaedic disorders and furthermore the installation of pathological patterns. Before any therapeutic intervention, it is necessary to evaluate the lower and upper limb function by means of reliable, valid and reproducible tools. This helps define precise and mutual objectives for child and family as well as therapists, therefore allowing an objective evaluation of the results. This principle applies as much in fundamental techniques (physiotherapy, occupational therapy, psychomotricity, orthesis…) as in more recent developments such as botulinum toxin, exercise programs, constraint-induced therapy, motor mental imagery or virtual reality. These recent therapies present, at various degrees, scientific evidence of their efficiency in the improvement of limb function and are now an integral part of the usual care of children with hemiplegic cerebral palsy.     

Évaluation du recrutement des muscles fessiers dans les exercices de rééducation chez le jeune paralysé cérébral

Gluteus muscles weakness, especially Gluteus Maximus and Gluteus Medius ones are frequently reported in Cerebral Palsy (CP) walking children. Thus, their reinforcement in rehabilitation is often justified. Many physiotherapy exercises solicit their recruitment, but their efficiency was not yet reported. Ten different exercises are assessed and compared using surface electromyography (EMGs). The activity of Gluteus Maximus and Gluteus Medius muscles was recorded in 18 CP and eight asymptomatic children while realizing those exercises. The levels of muscular activation were compared between each exercise, and the existence of patterns was sought in the group of CP children. CP children measures were also compared to those recorded for valid children. This study gives classification of the exercises with respect to their efficiency for the recruitment of Gluteus Medius and Gluteus Maximus. The activity of these muscles in the various phases of the exercises is stated. Finally selection criterions between the exercises are proposed according to rehabilitation objectives of the pelvic belt.     

Biomarqueurs cérébraux fonctionnels non invasifs de la performance motrice : supervision cérébrale pour la rééducation motrice en infirmité motrice cérébrale

For several years, studies have investigated non-invasive functional brain biomarkers derived from EEG signals in healthy people. Such biomarkers allow assessment of cortical dynamics in relation to the level of motor performance. Thus, similar biomarkers could be specifically engineered for persons with Cerebral palsy (CP) in order to provide the therapist new rehabilitation tools such as brain monitoring systems. This article presents a brief state-of-the-art research line related to these biomarkers, their current limitations and possible solutions, as well as their potential use for brain monitoring of individuals with CP in rehabilitation settings.     

Le syndrome de vasoconstriction cérébrale réversible

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe headaches, as well as vasoconstriction of cerebral arteries, which resolves spontaneously in one to three months. This condition has a moderate female preponderance. The mean age of onset is around 45 years. About 60% of the cases are secondary, mainly occurring during postpartum and/or after exposure to vasoactive substances. The main clinical presentation includes multiple recurrent thunderclap headaches over one to three weeks. The major complications of RCVS are localized cortical subarachnoid hemorrhages (cSAH) (20-25%) and parenchymal strokes (5-10%). Complications occur with different time courses: hemorrhages (cSAH and intracerebral hemorrhages), and posterior reversible encephalopathy syndrome are early events occurring during the first week, while ischemic events including TIAs and cerebral infarcts occur significantly later, during the second week. Diagnosis requires the demonstration of the “string and beads” aspect of cerebral arteries by a cerebral angiogram (MRA, CTA or conventional) and the demonstration of the complete or marked normalisation of arteries by a repeat angiogram performed within 12 weeks of onset. Treatment is based on nimodipine that seems to reduce thunderclap headaches within 48 h. However, nimodipine has not proven any efficacy against the hemorrhagic and ischemic complications of RCVS. Relapses are possible but rare and have not been reported yet in prospective series. It seems appropriate to advise the patients to avoid sympathomimetic and serotoninergic substances.     

Les contraintes en rééducation : réflexions d’une psychologue

Motor reeducation of children with cerebral palsy comprehends some constraints, both physical and psychological. Through the observation of a number of young patients, we wish to show how they feel about these constraints, what are the motives and the aims of their participation and how their psychological development can be affected. We shall then consider what the use of these constraints implies for the health care team. Finally, the notion of “therapeutic alliance” will be presented and distinguished from “compliance”.     

Une approche pédagogique multimodale en mathématiques auprès d’élèves avec infirmité motrice cérébrale

The pedagogical approach presented provides successful solutions for cases of difficulty in treating numerical information. The remediation relies on tools to allow access to various mathematical activities. They have proven themselves effective with children with cerebral palsy where motor impairment is associated with cognitive impairment. They have also facilitated the learning of ordinary pupils identified with learning difficulties. The learning aids circumvent the instrumental difficulties to free attention for mental manipulation. To counter the many difficulties observed in handling tasks in the non-symbolic stage, the emphasis is placed on the presymbolic tasks already sufficiently organized to successfully access the symbolic tasks. Three principles ensure the success of the remediation implemented: they are based on multimodal input (gestural, visual and verbal), they ensure accurate results and allow the formation of cognitive relations within mathematics and also more generally in the development of the pupil. In providing the child with reliable tools, he finds pleasure in using his preserved cognitive abilities, thus installing confidence.     

Application de la résolution de problème dans une unité de suicidants et suicidaires : premiers résultats auprès de 53 patients hospitalisés

Suicidal behaviour is a clinical problem that has posed major challenges to efforts in its understanding and treating. There are many studies on the various mental disorders presumed to underlie suicidal behaviour. Less than 50 randomized clinical trials have been conducted evaluating treatments aimed specifically at treating individuals with high suicidal risk. There is sparse data regarding treatments for primary mental disorders, which actually reduce the risk of suicide attempts or of suicidal ideation. The authors think that focussing efforts on treatment of suicidal behaviours may improve our understanding of the mechanisms related to suicidal behaviour and mining them for ideas will lead to effective interventions. For over a year the authors introduced the problem-solving therapy in their broad program for treating suicidal inpatients. In this pilot study the authors observed that assisting patients in developing a more positive problem-solving orientation may modify customary attitudes of avoiding problems and reduce tendencies to respond in an impulsive or careless manner.     

Rééducation de la position assise de l’enfant paralysé cérébral infantile. Les moyens préventifs orthopédiques non chirurgicaux. Présentation d’un cas clinique

There are several sitting positions, which need a specific control of adjustment against gravity and a postural organization available to be learnt. The acquisition of sitting position is a very important step of child's functional development: this step is a very crucial point for clinicians who take care of rehabilitation of children affected by cerebral palsy because these children have abnormalities of functions against gravity and bad propioceptive informations. There are several and different pathological factors that could disturb the sitting position and it is very important to know these factors to prevent the orthopaedic problems and offer the most suitable chair for each child. For the most part, the children affected by cerebral palsy, especially if they belong to 3 or 4%, spend in sitting position 80% of their living time. If the child spends a lot of his time in a particular position, we must pay attention to the postural abnormalities and their orthopaedic outcomes. For this reason, it is very important to understand the importance of preventive treatment and clinic assessment oriented to identify the abnormalities. The educational and therapeutic strategies should be organized in an integrated and multidisciplinary way.     

Évolution clinique de la dépression post-accident vasculaire cérébral à Kinshasa

Objectives

To describe the evolution of the clinical profile of post-stroke depression over a period of one year and to determine factors associated with changes in post-stroke depression.

Methods

Prospective cohort study with a follow-up of 1 year including 30 consecutive eligible patients. The severity of depression was assessed with the patient health questionnaire (PHQ9).

Results

The mean age was 55.87 ± 12.67 years. Seventy percent of patients were men. The two assessments for neurological status, perceived health status and test results of attention were not statistically different. The rate of depressive symptoms was 26.67% in 2011 and 20% in 2012. Disability and apathy were significantly improved. The average for disability increased from 2.77 ± 1.19 to 2.46 ± 2.19 (P = 0.002). From 66.7% in 2011, the proportion of patients able to walk without assistance rose to 93.3% in 2012 (P = 0.03). In addition, the proportion of patients apathetic decreased from 43.3% to 13.3% (P = 0.01). Greater age, female sex, sleep disorders and post-stroke apathy remained associated with DPAVC between the two assessments, with an increase in the strength of the association for apathy.

Conclusions

The frequency of post-stroke depression is high and remains stable over time. Disability is the clinical feature that evolved more favorably. The association with apathy, present at the beginning, of the study was strengthened one year later.     

Aspect en IRM cérébrale des maladies mitochondriales. Algorithme décisionnel des maladies mitochondriales les plus fréquentes

Mitochondrial diseases are due to deficiency of the respiratory chain and are characterized by a broad clinical and genetic heterogeneity that makes diagnosis difficult. Some clinical presentations are highly suggestive of given gene mutations, allowing rapid genetic diagnosis. However, owing to the wide pattern of symptoms in mitochondrial disorders and the constantly growing number of disease genes, their genetic diagnosis is frequently difficult and genotype/phenotype correlations remain elusive. For this reason, brain MRI appears as a useful tool for genotype/phenotype correlations. Here, we report the most frequent neuroradiological signs in mitochondrial respiratory chain deficiency and we propose a diagnostic algorithm based on neuroimaging features, so as to direct molecular genetic tests in patients at risk of mitochondrial respiratory chain deficiency. This algorithm is based on the careful analysis of five areas on brain MRI: (1) basal ganglia (hyperintensities on T2 or calcifications); (2) cerebellum (hyperintensities on T2 or atrophy); (3) brainstem (hyperintensities on T2 or atrophy); (4) white matter (leukoencephalopathy); (5) cortex (sub-tentorial atrophy); (6) stroke-like episodes. We believe that the combination of brain MRI features is of value to support respiratory chain deficiency and direct molecular genetic tests.     

Infarctus cérébral et myocardique après consommation de cannabis chez une femme jeune

Introduction

Cannabis is the most consumed drug in the world particularly in young adults. Few reports have suggested a causal role of cannabis in the development of cerebral or cardiovascular events. We describe the first association of myocardial infarction and stroke after heavy cannabis consumption in a 45-year-old woman.

Observation

Stroke occurred in relation with a right carotid and middle cerebral artery thrombosis after cannabis abuse. The patient was successfully treated with intravenous rt-PA. Two days after her admission, she presented a myocardial infarction due to a coronary thrombosis. Cerebral and coronary arteries were angiographically normal. Etiological tests were negative and a toxic cause in relation with cannabis consumption was concluded.

Conclusion

Cannabis can be associated with vascular events by different mechanisms. Thrombosis may occur in cerebral and/or coronary arteries. We suggest that it might be useful to search for cannabis consumption systematically in young subjects victims of stroke and myocardial infarction.     

Intérêts et limites des échelles d’évaluation de la douleur chez l’enfant en situation de handicap

Accessing to a management of pain is now considered as peoples’ fundamental right. The pain should be evaluated before being treated. Pain is always a subjective phenomenon. Assessing pain of those who express in a singular way is a great challenge. Children and adolescents with intellectual disabilities, with cerebral palsy or with autism are now recognized as sensitive to pain as well as anyone. If some of them are able to use self-report tools, we have to apply disability-adapted scales to others. Like any assessment tools, they have advantages and limitations. We need to know them in order to make appropriate use for the care of vulnerable people.     

Le réseau cérébral par défaut : rôle cognitif et perturbations dans la pathologie

Introduction

A “resting state” or “default mode network” has been highlighted in functional neuroimaging studies as a set of brain regions showing synchronized activity at rest or in task-independent cognitive state.

State of the art

A considerable and increasing number of studies have been conducted over the last few years so as to unravel the cognitive function(s) of this brain network.

Perspectives

This review gives an overview of anatomical, physiological and phenomenological data regarding the default mode network. Different hypotheses have been proposed regarding the role of this network. Several studies have highlighted its involvement in autobiographical memory, prospection, self, attention, and theory of mind. The influence of the attention level and consciousness onto resting state brain network activity has also been discussed. Specific changes have been described in normal aging, Alzheimer's disease (AD) and multiple sclerosis (MS).

Conclusions

These studies altogether contribute to a better definition of the default mode network, in terms of implicated brain structures, subtending mechanisms, and potential cognitive roles. For instance, similarities and relationships were found between self-related brain activity and resting-state activity in regions belonging to this network, namely posterior cingulate and prefrontal areas that may reflect introspective activity experienced, more or less consciously, when the brain is not specifically engaged in a cognitive task. As a whole, the default mode network appears as a non human-specific intrinsic functional network, active all over the life from birth until aging where it is progressively modified, and sensitive to different pathologies including AD and MS. On the other hand, many points remain to be clarified concerning this network, such as the exact part of its activity dedicated to self-related cognitive processes (introspection, imaginary mental scenario based on past autobiographical experiences) and that involved in a sentinel-like attentional process designated to react to possible environmental events. Indeed, it seems that this network is functional even in case of low level of consciousness, i.e., during light sleep. Conversely, a loss of self and environment perception as in coma, deep sleep or anesthesia might modulate its connectivity along the anteroposterior axis, i.e., frontal activity disappearance associated with a parietal reinforcement of connectivity. Since studies aiming at highlighting these points are still uncommon to date, exhaustive and objective explorations are needed to better understand all these resting state processes.     

Applications et apports de la vidéo augmentée en réhabilitation pédiatrique

Background

Augmented video enables digital video image treatment by augmenting it with computer generated information, measurements and/or virtual imagery. We tested this technology (1) as a measurement tool associated with a visual gait assessment scale for children with cerebral palsy to determine whether it improved it's interobserver reliability and (2) as an external feedback tool during physiotherapy sessions.

Methods and results

(1) Two observers watched a random selection of 20 gait videos of 12 children with cerebral palsy. The videos were scored using the Observational Gait Scale, one with standard video software, the other with augmented video software (Dartfish®). Interobserver agreement for the total score went from substantial to excellent with video augmentation (κ 0.785 → 0.809). The most important increases were noted for the items that required objective angle and/or duration measurements. (2) Qualitative study of augmented video use during two successive physiotherapy sessions in 13 children with motor impairments. The software's contribution had positive assessments especially in children aged 9 and above.

Conclusions

The use of augmented video software improves the reliability of filmed gait assessments, offering an accessible and versatile alternative to 3D kinematic analysis. The analysis tools and filmed sequence manipulation options provided by augmented video also have the potential for being clinically applied in motor therapy as a potent augmented feedback tool.