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1.

Introduction

Described in 1890, Uhthoff's symptom corresponds to a transient blurred vision provoked by physical exercise or an increase of temperature. It is a frequent symptom occurring during the course of multiple sclerosis.

Patients and method

We report here four cases of patients presenting isolated Uhthoff phenomenon preceding multiple sclerosis by several years.

Results

These four patients presented transient neurological symptoms induced by intensive sporting activity for 1 to 6 years before diagnosis of multiple sclerosis. These symptoms were often visual but sometimes motor or sensorial. All symptoms appeared above a certain threshold specific to each patient, after 15 to 30 minutes of intense physical exercise (bike, running or handball) and all disappeared after a few minutes to one hour rest with full recovery to baseline.

Discussion

Uhthoff's phenomenon is explained by a conduction block. It is due to axonal demyelization that provokes a reorganization of sodium channels induced by a decrease in a safety factor highly sensitive to temperature, or by release of soluble blocking substances (oxide nitric or cytokines).

Conclusion

Without being specific, this symptom is strongly suggestive of this disease. Our case reports confirm the existence of “infraclinic multiple sclerosis”.  相似文献   

2.

Introduction

Horton's disease is the most common vasculitis of elder people. Several neurological complications are reported, but pachymeningitis is exceptional.

Observation

A 71-year-old patient who presented headache, hyperesthesia of the scalp, weight loss with a biological inflammatory syndrome and meningeal thickening on MRI. The diagnosis of pachymeningitis related to Horton's disease was retained. The patient was treated by corticosteroids with a good clinical, biological and radiological course after 22 months.

Conclusion

Horton's disease is a potential diagnosis in elderly persons with pachymeningitis and inflammatory syndrome.  相似文献   

3.
4.

Background

Although cognitive disorders are well-known in multiple sclerosis (MS), even in earlier stages of the disease, their management may be overlooked. Our objective was to elaborate and evaluate the efficiency of a remedial program (PROCOG-SEP) designed for MS patients. The evidence-based program proposes exercises to both stimulate preserved functions and develop new abilities compensating for cognitive disabilities.

Design/Methods

Twenty-four patients with MS participated in 10/2-hour PROCOG-SEP sessions over a 6-month period. A neuropsychologist recorded BCcog-SEP performances before and after the PROCOG-SEP program. In addition, the same neuropsychologist conducted psychoclinical interviews to complete the before and after cognitive evaluations. The statistical analysis used the t-test performed with Excel.

Results

Compared with the initial levels, subtests of BCcog-SEP showing improvement after PROCOG-SEP were: verbal memory (SRT), visuospatial memory (10/36), verbal fluency (animal categories) and response to conflicting orders. Also, individual psychological interviews tended to be in favor of a general improvement in quality of life (more social interactions for instance).

Conclusions/Relevance

To our knowledge, the management program we have elaborated is the first designed to improve cognitive deficits in MS. These encouraging results suggest possibilities for improving cognition and thus quality-of-life in MS patients.  相似文献   

5.

Introduction

Tuberous sclerosis is an autosomal dominant inherited phakomatosis. It is associated with a wide variety of central nervous system abnormalities, but intracranial aneurysms are rare.

Case report

We report a 34-year-old patient fulfilling the diagnostic criteria of tuberous sclerosis in association with intracranial aneurysm.

Discussion

This association has been reported in only 17 other cases of tuberous sclerosis. We discuss the etiopathogenic mechanisms, preferential localizations and the various therapeutic propositions.  相似文献   

6.

Background

Aberrant brain activation during facial emotion discrimination has been described in chronic schizophrenia, while little is known about early stages of the illness. The aim of the current study was to investigate valence-specific brain activation of emotion discrimination in first-episode schizophrenia. These patients provide the advantage of lacking the effects of long-term medication and chronic illness course and can hence further enhance the understanding of underlying psychopathological mechanisms.

Methods

Using event-related fMRI, we investigated 18 first-episode schizophrenia patients and 18 matched healthy subjects during an explicit emotion discrimination task presenting happy, sad and neutral monochromatic facial expressions. A repeated measure analysis of variance (ANOVA) with the factors Group (patients, healthy subjects), Gender and Emotion (happy, sad, neutral) was performed on behavioural and functional data.

Results

Behavioural performance did not differ between groups. Valence-independent hypoactivations in patients were observed for the anterior cingulate and orbitofrontal cortex while hyperactivations emerged in the posterior cingulate and the precuneus. Emotion-specific group differences were revealed in inferior parietal and orbitofrontal brain areas and the hippocampus.

Conclusions

First-episode schizophrenia already affects areas involved in processing of both, emotions and primary facial information. Our study underlines the role of dysfunctional neural networks as the basis of disturbed social interactions in early schizophrenia.  相似文献   

7.

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.  相似文献   

8.

Objective

To assess the effectiveness of an individual program of psychoeducation in inpatients hospitalized for the first time with bipolar disorder, by comparing the acquired knowledge of patients who have received the program and that of patients who did not.

Methods

Patients who followed psychoeducation program were hospitalized in a bipolar disorder unit and control patients were hospitalized in other units of the same psychiatric department. At the end of hospitalization, personal questionnaires assessed knowledge with respect to bipolar disorder and its treatment; allowing us to calculate a “knowledge score”.

Results

The psychoeducation group had a better knowledge of bipolar disorder and of its treatment than that of the control group [39.3 (ET 3) versus 29.8 (ET 6,8); p < 0.0001], independently of other demographic and clinical characteristics.

Discussion

Structured and formalized psychoeducation appeared useful in inpatients in order to familiarize them with their disorder and their treatment.  相似文献   

9.

Introduction

Spontaneous dissection of cervical arteries is one of the major causes of stroke in young patients. The recommended treatment is curative anticoagulation. However, in selected cases, this treatment could fail or meet contraindications. In such cases, an endovascular stent-assisted angioplasty could be successful.

Case report

We report the case of a 47-year-old patient with spontaneous dissection of the carotid and vertebral arteries. Despite optimized anticoagulant therapy, cerebral perfusion was low. An endovascular stent-assisted angioplasty was performed in the intra- and extracranial segments of the right internal carotid artery. Consequently by improving the intracranial circulation, normal functioning was restored with no immediate complications and the patient remained free of complications at 16-month follow-up.

Conclusion

Dissections of cervical arteries that lead to a chronic reduction in brain perfusion might benefit from endovascular stent-assisted angioplasty.  相似文献   

10.

Background

Individual structural imaging studies in the pre-psychotic phases deliver contrasting findings and are unable to definitively characterize the neuroanatomical correlates of an increased liability to psychosis and to predict transition to psychosis.

Method

Ninenteen voxel-based morphometry (VBM) studies of subjects at enhanced risk for psychosis and healthy controls were included in an activation likelihood estimation (ALE) meta-analysis.

Results

The overall sample consisted of 701 controls and 896 high risk subjects. Subjects at high risk for psychosis showed reduced gray matter (GM) volume as compared to controls in the right superior temporal gyrus, left precuneus, left medial frontal gyrus, right middle frontal gyrus, bilateral parahippocampal/hippocampal regions and bilateral anterior cingulate. High risk subjects who later developed a psychotic episode showed baseline GM volume reductions in the right inferior frontal gyrus and in the right superior temporal gyrus.

Conclusions

GM volume reductions in temporo-parietal, bilateral prefrontal and limbic cortex are neuroanatomical correlates of an enhanced vulnerability to psychosis. Baseline GM reductions in superior temporal and inferior frontal areas are associated with later transition to psychosis.  相似文献   

11.

Background

In May 1978, was voted Law 180, forbidding any new admission in Italian mental hospitals. Many authors set it as an example to be followed in other countries; others contradict this point of view. The purpose of this paper is to analyse the Italian psychiatric revolution in retrospect thirty years later.

Method

Several articles and books written by stakeholders and by social researchers were reviewed.

Results

The occurrence of the Italian psychiatric revolution cannot be understood without considering the backward situation of mental care in Italy at the time. Changes began before 1978 but were accelerated and shaped by the law. However, some flaws of the previous system have remained over time, most of all, the North-South gap.

Discussion

Three points are specially original in Italy and need to be considered before advocating a similar law in another country: first, phasing out psychiatric hospitals went along with difficult problems, second in Italy, local human action is very important, so that implementation of such a law is a bottom-up process, and third, the history of the Italian mental care system has created in the general population a lower propensity to seek care than in other countries.  相似文献   

12.

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.  相似文献   

13.

Introduction

Neurological complications of metronidazole are rare, predominantly peripheral neuropathies, especially in patients on a long-term high-dose regimen. Cerebellar syndrome or seizures are less frequently reported. The concomitant occurrence of the three complications is exceptional.

Case report

We report herein a case with these three complications as side effects of metronidazole. For the cerebellar syndrome, the T2-weighted brain MRI showed a rounded and well-delimited zone of high signal intensity in the cerebellar dentate nuclei, extending up to the protuberance and the subthalamic nucleus, bilaterally and symmetrically.

Conclusion

Neurological complications are possible when a treatment with metronidazole is prescribed for a long duration or at high dose. In our patient, the clinical abnormalities and MRI signs regressed a few months after treatment withdrawal.  相似文献   

14.

Introduction

Neurosyphilis is rare today. It arises on average 20 years after poorly treated or untreated primary syphilis. Considering the decline in the incidence of syphilitic meningo encephalitis and the little known MRI aspects, we report the case of a patient affected by neurosyphilis occurring after primary syphilis untreated for 25 years.

Case report

A 65-year-old man presented typical clinical features including general paresis with psychiatric disorders, maniac access, and frontal syndrome as well as tabes dorsalis and Argyll-Robertson pupil. Brain MRI showed bilateral high intensity signals on the T2 weighted sequence located in mesiotemporal, insular, frontal regions.

Conclusions

Very few cases of neurosyphilis with detailed brain MRI aspects have been reported. The interest of this case report is first to recall the importance of syphilitic serology in patients with subacute psychiatric disorders and secondly to present rarely reported aspects of the brain MRI.  相似文献   

15.

Introduction

Posterior cortical atrophy (PCA) is a clinically and radiologically defined syndrome, in which predominant symptoms focus on higher visual dysfunction with progressive course and association with cortical atrophy or hypometabolism that predominates in the posterior part of the hemispheres. Homonymous hemianopia (HH) has rarely been described in this syndrome.

Methods

We report on six patients (four females, two males, aged 63 to 80) referred for visual disorder which led to demonstration of HH using perimetry testing. These patients were followed for 1 to 5 years after discovery of HH. Brain imaging with MRI or CT scan was obtained in the six cases and a SPECT scan was performed in four cases.

Results

HH was left-sided in four cases and right-sided in two cases. Associated symptoms related to higher visual dysfunction were simultagnosia, alone or as part of a full Balint's syndrome, alexia, constructional apraxia, dressing apraxia, visual form agnosia, prosopagnosia and hemispatial neglect. These symptoms were mild at onset but invariably worsened with disease progression. Dementia eventually developed in all cases. The clinical diagnosis was probable Alzheimer's disease in five cases and corticobasal degeneration in one case. Radiology showed posterior cortex atrophy in all cases as well as reduced cerebral blood flow in the same region, with an asymmetrical pattern compatible with the side of HH.

Conclusion

Elementary cortical lesions in PCA can develop mainly in the associative visual areas and even in the primary visual area, resulting in HH. HH has rarely been documented in PCA, but its prevalence would probably be higher if systematic search was conducted. Apparently isolated HH of insidious onset should suggest PCA and lead to neuropsychological testing and search for discrete atrophic changes of the posterior cortex on MRI as well as for metabolic alterations with SPECT or PET.  相似文献   

16.

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.  相似文献   

17.

Introduction

Spinal neurosarcoidosis is rare and exceptionally inaugural.

Observation

A 49-year-old African woman developed a progressive left Brown-Sequard syndrome. Magnetic resonance imaging (MRI) scans of the cervical spinal cord revealed an intramedullary lesion from C2 to T1 with intense pial enhancement after administration of contrast material associated with cervical spondylosis. The diagnostic of sarcoidosis was confirmed by liver biopsy which demonstrated noncaseating granulomas.

Conclusions

MRI features of spinal neurosarcoidosis were reviewed by the authors with focus on differential diagnosis.  相似文献   

18.

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.  相似文献   

19.

Introduction

The main objective of this review is to present a new approach to the memory deficit in Alzheimer's disease. Recent memory models suggest that information is recovered either on the basis of recollection or on the basis on familiarity. Recollection, unlike familiarity, requires the retrieval of contextual details related to the encoded information.

State of the art

This review suggests that recollection is particularly affected in Alzheimer's disease. In contrast, familiarity seems to be relatively preserved. A related deficit in metamemory is observed when recollection is required; a decrease in recollection in Alzheimer's disease could explain the pattern of metamemory problems. The deficit in recollection could be explained by a disconnection between medial temporal areas and frontal areas.

Perspectives

This novel approach to memory gives research perspectives concerning both early diagnosis and rehabilitation strategies of Alzheimer disease.

Conclusion

This overview showed deficits in conscious memory processes conceived of as recollection. These novel insights should provide new explanations for the deficits observed in Alzheimer's disease, particularly metamemory.  相似文献   

20.

Objective

To evaluate the relationship between a history of traumatic experiences and the clinical features of first-episode psychosis (FEP).

Method

We tested associations between trauma variables and duration of untreated psychosis (DUP), age of onset (AO), PANSS-rated positive and negative symptoms and depressive symptoms (Calgary Depression Scale) in a sample of 54 FEP patients.

Results

Mean DUP was 34.4 weeks, while mean AO was 24.7 years. Witnessing a seriously violent assault (49%) was associated with high positive symptoms (p = 0.002), while a significant personal experience of racism and discrimination (39%) was associated with high depressive (p = 0.042) symptoms. Previous sexual assault (44% of females) was associated with high positive (p = 0.028) and negative (p = 0.035) symptoms with a trend association with depressive symptoms (p = 0.092).

Conclusion

Our findings suggest that previous traumatic experience is associated with positive and affective symptoms in FEP.  相似文献   

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