Traitement des chondrosarcomes de la base du crâne

Background and purpose

Skull base chondrosarcomas are rare. Gross total removal is the treatment of choice, but can be difficult depending on the closeness of noble structures. Proton beam therapy can be associated in most cases.

Methods

Retrospective study of five cases treated in 13 years and study of the literature.

Results

Median age of patients was 34 years [28-46]. Cranial nerve palsy was the common clinical presentation. Tumor location was variable but always off midline. Treatment was surgical in all patients with a maximal resection and proton beam therapy associated for two cases. Surgical complications were rare with cranial nerve palsy as the main side effect. Outcomes were good with a median follow-up of 12.4 years [4.3-16.2].

Discussion

The review of the literature showed that chondrosarcomas of skull base are rare. The best outcome is achieved with total surgical resection. Medical imaging can only give clues to the diagnosis. Pathology is required to obtain a precise immunohistochemistry diagnosis. Multidisciplinary treatment using proton beam therapy and surgical removal enables a good local control (90-100%) at 5 years with good quality-of-life. It is difficult to determine how many cases have been published (around 220 cases in the literature) since many surgical or radiotherapy series included the same patients.     

Thrombolyse intraveineuse par rt-PA des accidents vasculaires cérébraux ischémiques : l’expérience de l’unité neurovasculaire de Nice

Introduction

Intravenous thrombolysis with rt-Pa in stroke has been approved in France since 2002. We report an evaluation of our practice. We have tried to identify predictive factors of dependence and death, and to compare our results with the data of the literature.

Patients and method

All patients treated with intravenous rt-PA within the first 270 min after the stroke onset were included. Univariate, then multivariate analyses were performed to determine the variables influencing the functional outcome at 3 months follow-up, according to a dichotomy established from the modified Rankin scale.

Results

One hundred and forty-two patients were included in this study (mean initial National Institute of Health Stroke Scale [NIHSS]: 15). Fifty percent had a Rankin score higher than 2 at 3 months follow-up. NIHSS above 12, glycemia of at least 120 mg/l, and systolic blood pressure above 160 mmHg at admission were identified as independent predictive factors of poor functional outcome. Less than 4 points decrease of NIHSS proved to be a simple and early predictor of poor functional outcome at 3 months follow-up.

Conclusions

In terms of safety and efficacy the data issuing from the daily activity of our stroke unit are comparable with those of clinical trials.     

Confirmation de l’intérêt de la biopsie cutanée dans la neuropathie des petites fibres. Résultats préliminaires

Introduction

In small-fiber neuropathy, skin biopsy reveals a reduction of intraepidermal nerve fiber density (IENFD), a feature often necessary for diagnosis. In France, this technique has not been widely used for this purpose.

Patient and method

To validate this method, we studied 13 patients with suspected small-fiber neuropathy, analyzed their nervous intra- and subepidermal network with a punch skin biopsy and compared our data with those of literature.

Results

Ten patients had pure small-fiber neuropathy and three an axonal polyneuropathy involving large-caliber nerve fibers. In the group of patients with pure small-fiber neuropathy, we found medium IENFD (11.6 ± 4.46 fibers per millimeter in the proximal thigh and 7.15 ± 3.59 fibers per millimeter in distal leg), well correlated with the electron microscopy quantitative and qualitative analysis of the unmyelinated subepidermal fibers.

Conclusion

This work demonstrated the good reproducibility of skin biopsy for analyzing the small-fibers in our cohort. These results require further confirmation in a larger cohort and validation in comparison with controls analyzed on a local level. Nevertheless, these techniques seem to be useful to assess the difficult diagnosis of small-fiber neuropathy.     

Étude longitudinale de la qualité de vie dans la sclérose en plaques : corrélation aux paramètres morphométriques de l’imagerie

Introduction

Health related quality of life (HRQOL) is often affected in multiple sclerosis (MS). Nevertheless, to our knowledge, there is no longitudinal study in the literature about the correlation between MRI parameters and HRQOL in MS patients.

Methods

We included 28 patients with clinically definite relapsing remitting MS. All patients initiated subcutaneous interferon beta-1a therapy. To assess HRQOL, we used the SEP-59 scale, the French validated translation of MSQOL-54, and the MusiQoL scale. Conventional MRI was performed every year. Lesion load (LL) and brain atrophy were automatically measured using SepINRIA, a free software developed by INRIA in Sophia Antipolis.

Results

The mean EDSS score was 1.7 and disease duration was 2.5 years. Our results revealed that HRQOL was significantly correlated to T1 and T2-LL with both SEP-59 and MusiQoL scales. T1-LL was better correlated with physical dimensions and T2-LL was better correlated with mental components. At 1-year follow-up, patients whose MRI showed either an increase of T1 LL or at least one gadolinium enhancing lesion had a worse HRQOL at the end of the study. Initial brain parenchymal fraction (BPF) measure was also correlated with the long-term follow-up HRQOL. EDSS scored at the end of the study had not significantly changed (1.3; P > 0.05).

Conclusion

Our study revealed pertinent clinicoradiological correlations between HRQOL and MRI parameters in our cohort.     

Les maladies neurologiques repérées lors de la consultation multidisciplinaire de la chute du CHRU de Lille (France)

Background

People with neurological disorders including stroke, dementia, Parkinson's disease, and polyneuropathy are known to have an increased risk of falls.

Objective

To evaluate the prevalence and nature of neurological risk factors among the patients attending the Multidisciplinary Falls Consultation of the University Hospital of Lille (France), and to analyze the characteristic features of patients termed “neurological fallers” with neurological risk factors.

Methods

The study included 266 consecutive patients who were initially assessed by a geriatrician, a neurologist and a physiatrist, and again, six months later, by the same geriatrician.

Results

Two out of three patients had neurological signs that can be regarded as neurological risk factors of falling. These neurological signs had not been diagnosed before the consultation in 85% of cases. The most common conditions were deficit of lower extremity proprioception (59% of patients) and cognitive impairment (43%). The most frequently evoked neurological diseases were dementia (40% of patients), polyneuropathy (17%) and stroke (8%). Compared with other patients, “neurological fallers” were more frequently living in a nursing home, had lower ADL and MMSE scores at baseline, had experienced more falls in the six preceding months, had a lower probability of having a timed Up-and-Go test less than 20 seconds and a single limb stance equal to 5 seconds. In the follow-up, “neurological fallers” reported hospitalizations more often.

Conclusion

The findings show that a large proportion of old persons presenting at the Multidisciplinary Falls Consultation have unrecognized neurological disorders. Comprehensive neurological examination including an evaluation of cognition is required in every elderly faller.     

Abcès cérébelleux révélant une malformation artérioveineuse pulmonaire isolée en dehors de la maladie de Rendu-Osler

Introduction

Brain abscesses occur in 5 to 13 % of patients with pulmonary arteriovenous malformation (PAVM), more often present in Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT).

Case report

A 51-year-old man with a history of transient Parinaud syndrome at 37 years complained of headache for 2 months before acute onset of a left cerebellar syndrome without fever. CT-scan and MRI of the head revealed a heterogeneous left cerebellar lesion. A brain abscess was drained and all signs resolved. CT-scan of the chest revealed a left lingual PAVM; occlusion was incomplete after coil embolization. He had no feature of HHT and no mutation in ENG and ACVRL1 genes. A second embolization was performed 5 months later, but the malformation was not occluded at 6 months.

Discussion

We report the seventh case of PAVM complicated by a cerebellar abscess. The right to left shunt in PAVM results in hypoxemia, secondary polycythemia and paradoxical embolization of infective organisms bypassing the pulmonary filter.

Conclusion

Combining different MRI techniques (in particular diffusion and proton MR spectroscopy) provides invaluable data for the diagnosis of brain abscess. Careful search for PAVM must be undertaken, particularly in adults with cryptogenic abscess, to avoid further abscess formation or stroke.     

Acceptabilité et faisabilité de la psychothérapie par les patients avec trouble de personnalité limite

Background

Borderline Personality Disorder (BPD) is a pervasive pattern of instability and impulsivity associated with serious psychosocial limitations and a significant risk of suicide (4-10 %). The prevalence of this disorder is around 1.8 % in the general population. In a public health perspective, the high financial cost arising from this disorder (around 17,000€ per year) requires optimizing their care utilization carefully. This medico-economic aspect converges with clinical observations of instability in the health network; BPD patients meet at least seven psychiatrists. Indeed, psychotherapy is less accessible, particularly in some geographic area.

Objective

Discuss strength and weakness of psychotherapy for BPD in the real world.

Method

Through a non systematic literature review, we present data on the availability and use of health services by people with BPD.

Results

The effectiveness and limitations of psychotherapeutic approaches, considered as the gold-standard therapy, are reviewed. All the psychotherapies assessed by Randomised Control Trials seem to be effective. However, not all the psychotherapies have the same level of evidence for core symptoms of BPD. Furthermore both the length of most of the psychotherapies suggested to these unstable patients and the number of participants (Dialectic Behavioral Therapy and Mentalization Based Treatment) needed to constitute the team for some of them are serious limitations. Moreover, even available, as shown by observational studies, high drop-out in studies they have a limited acceptability for BPD themselves. Some of them have less drop-out (Dialectic Behavioral Therapy) or a better therapeutic alliance (Schema Focused Psychotherapy) than other one. A presentation of alternatives to psychological approaches is proposed, particularly an example of a Quebec multidisciplinary care organization covering the range of service needs of people with BPD.

Conclusions

The heterogeneity of clinical situations grouped in the BPD is confronted with the limits of a dichotomous approach (structured psychotherapy or chaotic utilization of care), manifested by a reduced acceptability of care today. This encourages them to rethink the consistency of the service offer to these severe patients.     

Intérêt de l’analyse factorielle discriminante pour classer les syndromes parkinsoniens à partir de la tomoscintigraphie d’émission monophotonique

Introduction

The objective was to assess the value of single photon emission computerized tomography (SPECT) and factorial discriminant analysis (FDA) in the differential diagnosis of Parkinson's disease (PD), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD).

Patients and methods

Sixty-two patients with clinical diagnoses of either CBD, PSP or PD were studied using brain HmPaO-SPECT. Thirteen pairs of regions of interest (ROIs) were drawn on the slices located 50 mm and 90 mm above the canthomeatal plane. Twenty-six uptake indices and 13 asymmetry indices were determined. FDA was performed in order to determine whether or not the patients could be classified into the correct clinical group on the basis of SPECT data alone. The most discriminant parameters were used to generate two predictive scores, which were tested in a second group of 15 patients.

Results

FDA of all 39 variables correctly classified all the patients. A subset of 10 variables was used to build predictive scores, which correctly classified 90% of PD patients, 100% of PSP patients and 86% of CBD patients. When tested in the validation group of 15 patients, these predictive scores correctly classified 87% of the individuals. The frontal medial, temporoparietal and parietal regions were the most discriminant.

Conclusion

Using SPECT data alone, this study enabled us to distinguish between PD, PSP and CBD in patients with clear clinical presentations of the diseases in question. This novel, statistical approach provides reliable information. However, a prospective study dealing with de novo parkinsonian syndromes will be necessary.     

Traitements de fond de la sclérose en plaques : enseignements des études randomisées comparatives directes

Introduction

Several first-line immunomodulatory treatments are available to physicians for treating patients with relapsing-remitting multiple sclerosis, namely three interferon-β preparations and glatiramer acetate. In order to enlighten their choice of treatment, physicians need data from good quality comparative clinical trials.

Methods

This review outlines and compares the findings of the various randomized clinical trials that have assessed the efficacy and safety of immunomodulatory treatments for multiple sclerosis. Six such studies have been reported to date, a Danish study (interferon-β 1a sc versus interferon-β 1b sc), the EVIDENCE study (interferon-β 1a sc versus interferon-β 1a im), the INCOMIN study (interferon-β 1b sc versus interferon-β 1a im), the BECOME and BEYOND studies (interferon-β 1b sc versus glatiramer acetate) and the REGARD study (interferon-β 1a sc versus glatiramer acetate).

Results

These studies have demonstrated somewhat superior efficacy for interferon-β preparations administered subcutaneously several times a week compared to preparations administered intramuscularly once a week. In contrast, no difference in efficacy has been demonstrated between the two subcutaneously administered interferon-β preparations, or between either of these preparations and glatiramer acetate. Differences in the safety profile of the four immunomodulatory treatments were observed, in particular a more favorable skin tolerance with intramuscularly administered interferon-β and a better systemic adverse event profile for glatiramer acetate.

Conclusions

These various randomized comparative trials have provided objective criteria that can be used by physicians for choosing between immunomodulatory treatments.     

Atteinte multiple des nerfs crâniens : atteinte multiple des nerfs crâniens au cours du tétanos céphalique. À propos d’une observation et revue de la littérature

Introduction

Cephalic tetanus is the most serious form of localized tetanus. It associates trismus with impairment of one or more cranial nerves. It was a rare condition, whose diagnosis can raise several problems.

Case report

A 49-year-old-man presented multiple and unilateral cranial nerve involvement revealing cephalic tetanus.

Conclusion

This case illustrates the importance of considering cephalic tetanus when patients present cranial nerve palsy associated with injury.     

Méthode et résultats du test de perfusion dans l’hydrocéphalie à pression normale : revue de la littérature

Introduction

The cerebrospinal (CSF) infusion test is used to analyze the dynamics of CSF circulation. We describe the technique and report test results obtained in subjects with normal pressure hydrocephalus.

State of the art

The CSF infusion test is based on pressure monitoring during a continuous infusion of saline solution into the CSF space via a lumbar puncture. The main parameters are: intracranial pressure, resistance to CSF outflow (Rout), and compliance of the cranial enclosure estimated by the pressure-volume index. Our review of the recent literature dealing with the results of infusion test in normal pressure hydrocephalus indicates that the positive predictive value is about 80 % for Rout greater than 12 mmHg/ml per minute, i.e. shunt response is observed in 80 % of patients. The information available in the literature for assessing negative predictive value of Rout and usefulness of compliance is limited.

Perspectives and conclusion

The CSF infusion test may be helpful for the diagnosis of normal pressure hydrocephalus, as supplemental testing when the diagnosis is not certain. We propose a procedure, which includes the infusion test, for identifying shunt-responsive patients.     

Le suicide dans la maladie de Huntington : état actuel des connaissances

Objectives

In 1872, George Huntington was the first to describe a genetic disease combining three types of disorder symptoms viz: motor, cognitive - evolving to cortical dementia and psychiatric. The purpose of this paper is to provide a selective review of the major issues and findings concerning suicide in Huntington's disease. The aim was to understand why patients with Huntington's disease have a higher suicide rate than those suffering from other neuro-developmental diseases.

Materials and methods

The present review is based on a MEDLINE survey of the relevant literature. The terms used in the search were: “Suicide”, “predictive genetic testing”, “suicidal risk factors” and “suicide attempt” all in combination with “Huntington's disease”. All abstracts were read and potentially relevant articles were examined in full. Various other important cross-references were included.

Results

Most of the authors found that suicidal occurrence in Huntington patients was four times higher than that in the general population. No specific individual risk factor was found except a lack of offspring and of psychological support. This review also considered the patient's attitude towards predictive genetic testing and its results. Reactions varied from psychological relief to paradoxical suicidal resurgence.

Conclusion

This paper emphasizes the need of appropriate psychiatric care for Huntington's patients in order to try to prevent suicidal behaviour.     

Comparaison de la qualité de vie de patients atteints d’atrophie multisystématisée et de maladie de Parkinson

Purpose

Quality of life (QoL) in multiple system atrophy (MSA) is thought to be poorer than in Parkinson's disease (PD), primarily because of motor impairment, autonomic dysfunction and depression. The aim of the study was to investigate QoL in 10 patients with probable MSA (parkinsonian subtype) compared with 10 PD patients matched for motor disability on UPDRS III motor score.

Methods

All patients were ambulatory and non-demented. Mean durations of disease in MSA and PD patients were respectively 3.6 and 9.0 years. QoL was assessed using the SF-36 health-related questionnaire and a life satisfaction visual analogue scale. Patients were also evaluated for cognitive function (Mattis Dementia Rating Scale [Mattis DRS], Wisconsin Card Sorting Test [WCST], Stroop, Fluencies), depression (Beck Depression Inventory-II [BDI-II]), apathy (Modified Apathy Evaluation Scale) and were screened for non-motor symptoms (NMS Quest).

Results

The only difference in QoL between MSA and PD patients matched for motor disability was that the SF-36 vitality subscore was more impaired in MSA and negatively correlated with interference index on Stroop word colour testing. Depression and non-motor symptoms were associated with poorer QoL in both groups. Among MSA patients, cognitive impairment (Stroop interference index) and apathy also had a negative impact.

Conclusion

There was no major difference in QoL between MSA and PD patients matched for motor disability with a disease duration about 5 years longer. The SF-36 vitality subscore was more impaired in MSA and associated with interference sensitivity.     

Un cas de crampe des écrivains dystonique combinée à un syndrome du canal carpien à Ouagadougou (Burkina Faso)

Introduction

Focal hand dystonia and carpal tunnel syndrome are linked to impairment of the central and peripheral nervous systems, respectively. We report a singular case combining these two entities.

Case report

A 44-year-old right-handed male pharmacy employee whose daily activity for 18 years was to fill out and staple insurance vouchers and unload and store boxes of medicines, presented, almost concomitantly, the combination of right focal hand dystonia and homolateral carpal tunnel syndrome.

Conclusion

This observation corroborates the results of experimental studies on repetitive manual activity which would be a source of central and peripheral changes, affecting the hand somatotopy in the sensory-motor cortex and the descending control of basal ganglia, explaining dystonia, and leading to median nerve compression related to repeated microtrauma, inflammatory reactions and fibrosis of the carpal tunnel.     

Stimulation magnétique transcrânienne répétée (rTMS) et symptômes négatifs de la schizophrénie

Purpose

The author has endeavoured to present a qualitative review of current data on the interest of repetitive Transcranial Magnetic Stimulation (rTMS) in the treatment of negative symptoms of schizophrenia, its therapeutic impact and processes that underlie it.

Method

The method consisted in a review of the literature by an extensive consultation of the computerized Medline database.

Results

Despite the small number of controlled studies and the small sample sizes, rTMS appears to be an effective therapeutic method in the treatment of negative symptoms of schizophrenia and also helps understanding the patho-physiologic processes that underlie them (hypofrontality, dopamine hypothesis and responsiveness of the HPA axis).

Conclusion

Before any conclusion may be reached about the effectiveness of this new technology more studies are required using larger parameters for active treatment and optimal placebos (alpha-TMS at least 110 % of the motor threshold, focused on the hypo-active area).     

Traitement de l’hypotension spontanée du liquide cérébrospinal par perfusion épidurale de sérum salé isotonique

Introduction

Spontaneous intracranial hypotension (SIH) is an uncommon cause of secondary headache due to a cerebrospinal fluid (CSF) hypotension. Lumbar epidural blood-patch (LEBP) is the most effective treatment and can be repeated in case of relapse. There is no standard therapeutic strategy for patients free of dural tears who fail to respond to several consecutive blood-patches. We report two cases of SIH successfully treated by an epidural saline infusion after two consecutive LEBP.

Case reports

A 35-year-old woman was admitted to hospital for severe orthostatic headache. The diagnosis of SIH was retained. Two LEBP were performed but with no clinical benefit. Headache disappeared totally after an epidural saline infusion. A second woman, aged 75 years, was admitted for chronic orthostatic headaches. The CSF pressure was low. Search for a dural tear was negative. After two unsuccessful LEBPs, the patient was treated with an epidural saline infusion. Her headache resolved completely and definitely.

Discussion

It is common procedure to search for a dural tear when patients fail to respond to several consecutive LEPB. Surgical repair is however exceptional. An epidural saline infusion might be an efficient therapeutic alternative despite the small number of cases reported in the literature.     

Comparación de la capacidad diagnóstica precoz de los biomarcadores de la enfermedad de Alzheimer en resonancia magnética cerebral y líquido cefalorraquídeo

Introduction

The goals of this study were to compare the early diagnostic utility of Alzheimer disease biomarkers in the CSF with those in brain MRI in conditions found in our clinical practice, and to ascertain the diagnostic accuracy of both techniques used together.

Methods

Between 2008 and 2009, we included 30 patients with mild cognitive impairment (MCI) who were examined using 1.5 Tesla brain MRI and AD biomarker analysis in CSF. MRI studies were evaluated by 2 radiologists according to the Korf?s visual scale. CSF biomarkers were analysed using INNOTEST reagents for Aβ_1-42, total-tau and phospho-tau_181p. We evaluated clinical changes 2 years after inclusion.

Results

By 2 years after inclusion, 15 of the original 30 patients (50%) had developed AD (NINCDS-ADRA criteria). The predictive utility of AD biomarkers in CSF (RR 2.7; 95% CI, 1.1-6.7; P<.01) was greater than that of MRI (RR 1.5; 95% CI 95%, 0.7-3.4; P<.2); using both techniques together yielded a sensitivity and a negative predictive value of 100%. Normal results on both complementary tests ruled out progression to AD (100%) within 2 years of inclusion.

Conclusions

Our results show that the diagnostic accuracy of biomarkers in CSF is higher than that of biomarkers in MRI. Combined use of both techniques is highly accurate for either early diagnosis or exclusion of AD in patients with MCI.     

L’enfance des schizophrènes : revue de la littérature

Introduction

Pre-morbid antecedents in schizophrenia have been studied for some time now more particularly as potential markers of vulnerability. What are the tell-tale signs in some of the patient's childhood? The authors suggest a non-exhaustive review of the literature on this subject.

Method

The authors reviewed the literature (English and French) of prospective and retrospective studies.

Results

Many fields appear to be impaired during the childhood of some schizophrenic patients, fields such as: developmental abnormalities, speech impairments, social interactions, behaviour, cognitive functioning. The authors also noticed the presence of neurological soft signs and para-clinical abnormalities.

Discussion

The authors suggest a critical and synthetic review of existing data: what can be retained of this data? The authors also discuss the evolution of these signs and their interaction with the evolution of the disease itself.

Conclusion

Many of these signs were noticed in several children who later developed schizophrenia. For many authors, the more important these signs are, the more severe the disease will be. These pre-morbid antecedents give rise to theoretical questions and open perspectives concerning an early diagnosis of schizophrenia.     

Maladie de Parkinson : de la physiopathologie des troubles psychiques à la maîtrise du traitement dopaminergique

Introduction

Behavioral changes in Parkinson's disease are complex and their pathophysiology is not yet fully understood. The dopaminergic system seems to play a major role and most of the behavioral disorders in Parkinson's disease can be classified into either hypodopaminergic if related to the disease itself or hyperdopaminergic if related to dopaminergic treatment.

State of the art

Subthalamic stimulation, which enables withdrawal of dopaminergic medication at an advanced stage in the disease, provides a model for the study of certain nonmotor, dopamine-sensitive symptoms. Such a study has shown that apathy, which is the most frequent behavioral problem in Parkinson's disease, is part of a much broader hypodopaminergic behavioral syndrome which also includes anxiety and depression. Nonmotor fluctuations - essentially fluctuations in the patient's psychological state - are an expression of mesolimbic denervation, as shown in positron emission tomography. Drug-induced sensitization of the denervated mesolimbic system accounts for hyperdopaminergic behavioral problems that encompass impulse control disorders that can be alternatively classified as behavioral addictions. The association of impulse control disorders and addiction to the dopaminergic medication has been called dopamine dysregulation syndrome. While L-dopa is the most effective treatment for motor symptoms, dopamine agonists are more effective in improving the nonmotor levodopa-sensitive symptoms. On the other hand, L-dopa induces more motor complications and dopamine agonist more behavioral side effects. There is increasing data and awareness that patients’ quality of life appears to be dictated by hypo- and hyperdopaminergic psychological symptoms stemming from mesolimbic denervation and dopaminergic treatment rather than by motor symptoms and motor complications related to nigrostriatal denervation and dopaminergic treatment.

Perspectives

Better management requires knowledge of the clinical syndromes of hyper- and hypodopaminergic behaviors and nonmotor fluctuations, a better understanding of their underlying mechanisms and the development of new evaluation tools for these nonmotor symptoms.

Conclusions

The neurologist who strives to gain mastery of dopaminergic treatment needs to fine tune the dosage of levodopa and dopamine agonists on an individual basis, depending on the presence of motor and nonmotor signs respectively.