Pachyméningite associée à une probable maladie de Horton : étude d’une observation

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.     

Anévrismes intracrâniens et sclérose tubéreuse de Bourneville : une association rare

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.     

Neurosjögren : une présentation inhabituelle

Introduction

Neurological manifestations in Sjögren's syndrome are variable. The peripheral nervous system is generally involved. We report a rare case of an unusual central neurological manifestation.

Case report

A 54-year-old woman was admitted with headache and tetraparesia. The physical examination revealed a tetrapyramidal syndrome and a bilateral parotidomegaly. The patient's general condition was nevertheless quite good. Brain MRI showed an heterogeneous pontine lesion with multiple nodular formations in the periventricular white matter. Blood tests revealed anti-SSA and anti-SSB antibodies. A labial salivary gland biopsy was grade IV in Chisholm scoring system and Schirmer's test was positive.

Conclusion

A latent Sjögren's syndrome can lead to a wide variety of focal brain MRI abnormalities and should be evoked when the etiology is not clear.     

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.     

Phénomène d’Uhthoff lié à l’effort, isolé, précédant une sclérose en plaques

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”.     

Apport d’une démarche électroclinique dans le diagnostic de l’épilepsie chez une population de sujets âgés confus

Background

Delusion symptoms often occur in old people; epilepsy is one of the main reasons behind these acute episodes. Current guidelines and recommendations from the Academy of Medicine have proposed a double clinical and electroencephalographic approach. Recently, a working group of French experts has issued an electro-clinical scale. The aim of our study was to compare the usual approach with the new one based on the electro-clinical score.

Method

All EEG requests performed since December 2008 in Bretonneau Hospital for elderly people aged over 75 years for delusion syndromes were retained for this study.

Results

One hundred and fifteen old patients from a geriatric-hospital (age 83.5 ± 6.06 years) were included in this protocol. The classical diagnostic process yielded the diagnosis of epilepsy for 50 subjects. The electro-clinical scale confirmed the diagnosis of epilepsy in 30 patients and ruled it out in 29 patients.

Conclusion

This study underscores the importance of evidence-based medicine for the diagnosis of epilepsy in old people and points out the underuse of the new technical tool, EEG-monitoring, for the management of these patients.     

Programme spécialisé pour les premiers épisodes psychotiques : analyse d’une cohorte de 100 patients

Background

The emergence of specialized programs for the treatment of first-episode psychoses in non-research settings calls for a better definition of this group of patients and of the psychological interventions offered.

Aims

The aim of this study is to describe a specialized program for first-episode psychotic patients and to define the patients referred to, their different distinguishing characteristics and their relative use of the different services offered them.

Method

From an initial population of 127 patients, 100 agreed to have their data used to determine their detailed socio-demographic and symptomatological characteristics, their treatment delays (duration of untreated psychosis, referral delay) and their use of specific treatment modalities offered.

Results

The sample is similar to others described in the current literature in terms of socio-demographics, diagnostic distribution, and duration of untreated psychosis. The referral delay is 1.66 year. The symptomatological and neuropsychological portraits observed are characterized by heterogeneity. Services offered appear clinically indicated for most patients referred to (81%), with different characteristics observed across the groups of patients referred to in the different modalities.

Conclusion

The heterogeneity of the clinical presentation and of needs observed implies that such a program has to include a detailed assessment of each patient and a basic range of interventions. The implementation of such interventions in a non-research setting, and eventually on a large scale, should be accompanied by an evaluation process that could help guide clinical work and the organization of psychiatric services for patients suffering from psychosis.     

Angioplastie carotidienne bilatérale chez une patiente avec infarctus cérébral sur maladie de Horton

Introduction

Giant cell arteritis is the most frequent vasculitis and can cause stroke in about 4% of the patients. The mechanism is often hemodynamic in relation with an arterial stenosis or occlusion. Optimal treatment remains to be defined. Outcome is often poor.

Case report

We report the case of a 78-year-old patient, with giant cell arteritis who developed neurological deterioration after the onset of the corticotherapy. Brain MRI revealed bilateral stroke caused by multiple preocclusive internal carotid stenosis. The patient was treated successfully with angioplasty and stenting.

Conclusion

Endovascular treatment could constitute an interesting alternative for patients with giant cell arteritis associated with neurological deterioration after a first episode of stroke because of hemodynamic instability or at stroke recurrence. For these patients, the usual medical treatment appears to be insufficient.     

Les dérivés conscients des mécanismes de défense au questionnaire de Bond (DSQ 40) : stabilité ou variabilité entre une situation non anxiogène et une situation anxiogène

Introduction

The aim of this research was to test the stability or variability of conscious by-products of defense mechanisms, evaluated with the Bond Defense Style Questionnaire (DSQ 40) so as to integrate them as transactional variables of stress in the model of Lazarus and Folkman.

Participants

One hundred and sixty-one college girls (first-year students in a French university) engaged in psychology studies participated in this research. They participated in two collective sessions: a first session took place in November in a non-stressful situation, and a second session during the January examination week.

Instruments

Two questionnaires were used twice in this experiment: (a) the Anxiety State Questionnaire of Spielberger (STAI-Y) which evaluates the anxiety-state and anxiety-traits; (b) the DSQ 40, which estimates 20 conscious by-products of defense mechanisms and distinguishes three factors corresponding to the mature, neurotic and immature styles according to the factorial analyses of Guelfi and al.

Hypotheses

Two hypotheses were formulated: (a) a first hypothesis postulates that an examination situation increases the State Anxiety score; (b) a second hypothesis postulates a variability of defense mechanisms assessed by Bond's questionnaire (DSQ 40). It verified that the scores of the Bond defensive style questionnaire are significantly different in the two sessions.

Results

The mean state-anxiety score is higher in the second session. The great majority of students are described as more anxious during the week of examinations than during the previous month. The first hypothesis is then validated. The situation of evaluation of the university performance appears as a situation more stressful than the courses period. The three defense mechanism factors vary in a significant way from the first to the second session. In the second session the scores in mature and neurotic defense mechanisms increased significantly whereas the average scores in immature defense mechanisms decreased significantly. The majority of the 20 defense mechanisms are not stable between both situations. Indeed, 17 conscious by-products of defense mechanisms differ significantly in both sessions and only three defense mechanisms show a certain stability. Eleven defensive styles (55 %) decreased, six increased (30 %) and three remained stable (15 %). The instability of conscious by-products of defense mechanisms between the two sessions was confirmed by correlations analyses.

Discussion

The results show that the instability between situations is not limited to three categories of defenses (mature, neurotic and immature): 17 out of 20 mean scores in defense styles differed significantly. Conscious by-products of defense mechanisms are, indeed, influenced by situational factors.     

Biological roles of trace elements in the brain with special focus on Zn and Fe

Introduction

Many metals like iron (Fe), copper (Cu) or zinc (Zn) fulfil various essential biological functions and are thus vital for all living organisms. For instance, they play important roles in nervous tissue, participating in a wide range of processes such as neurotransmitter synthesis, myelination or synaptic transmission.

State of the art

As in other tissues, brain cells tightly control the concentration of metals but any excess or deficit can lead to deleterious responses and alter cognitive functions. Of note, certain metals such as Zn, Fe or Cu accumulate in specific brain structures over lifespan and several neurodegenerative diseases are associated with a dysregulation of the homeostatic mechanisms controlling the concentration of these cations.

Conclusion and perspectives

This review will address some of the cellular and molecular processes controlling the entry and distribution of selected metals (mainly Zn and Fe) in the brain, as well as their roles in synaptic transmission, in the pathogenesis of some neurologic diseases such as Parkinson's disease and Alzheimer's disease, and their impact on cognitive functions.     

Angioplastie-stenting transluminale percutanée après dissection des troncs supra-aortiques

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.     

Leucoencéphalopathie multifocale progressive atypique chez un patient atteint de sarcoïdose médiastinopulmonaire

Introduction

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of central nervous system due to the JC virus. PML generally occurs in immunocompromised hosts and has a fatal outcome.

Observation

We report a case of an atypical PML in a patient with pulmonary sarcoidosis: MRI showed multifocal and punctate contrast enhancements. The diagnostic was made by brain biopsy.

Conclusion

The pathophysiology of this association is probably related to the immunodepression induced by sarcoidosis.     

Infarctus cérébral, infection VIH et syphilis méningovasculaire : étude de trois cas

Introduction

Various clinical manifestations can occur in syphilis, especially meningovascular syphilis. We report three cases of ischemic stroke related to meningovascular syphilis and HIV infection.

Case reports

Three men, aged 30, 31 and 41, presented typical neurologic deficit leading to the diagnosis of ischemic stroke. Radiographic investigations showed internal carotid thrombosis in two patients, and a basilar stenosis in the third. CSF analysis and blood samples revealed meningovascular syphilis associated with HIV infection. The other explorations were normal.

Conclusion

Due to the reemergence of syphilis, search for Treponema pallidum infection should be systematic in young stroke victims.     

Myasthénie séronégative et myasthénie avec anticorps anti-MuSK : une série rétrospective de 20 cas

Introduction

Despite the fact that anti-muscle specific tyrosine kinase (MuSK) antibodies have been discovered for seven years, only a few studies have, until now, focused on myasthenia without acetylcholine-receptors antibodies (ab) (formerly known as “seronegative myasthenia”), and among them, anti-MuSK-antibody-positive and -negative patients.

Method

We retrospectively studied 20 patients with “seronegative” myasthenia gravis, eight of them being anti-MuSK-ab positive, the remaining twelve being negative. We searched for clinical, neurophysiological, and therapeutic differences between the two groups (anti-MuSK-ab positive: anti-MuSK+ versus anti-MuSK-ab negative: anti-MuSK−).

Results

Anti-MuSK+ patients had more predominantly bulbar involvement and had more severe disease (these patients required referral to intensive care more frequently). There was no difference between the two groups concerning treatment efficiency and tolerance. Most of our patients were treated with acetylcholinesterase-inhibitors, and immunomodulatory or immunosuppressive drugs that could indirectly reflect greater severity. However, there was no difference in treatments for anti-MuSK+ versus anti-MuSK− patients.

Conclusion

These results both confirm and complete the preexisting data on RACh-negative myasthenia, and especially on myasthenia associated with MuSK antibodies.     

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.     

Paced Auditory Serial Addition Test : données normatives dans une population française

Introduction

The Paced Auditory Serial Addition Test (PASAT) is recognized as a sensitive measure of working memory, attention, and information processing speed in multiple sclerosis. The aim of this work was to provide normative data for PASAT in a healthy French population.

Observations

A total of 360 subjects were recruited from volunteers (24 groups, 15 subjects per group, considering four age classes, three educational levels and gender). The PASAT was administered in a standardized way, by the same psychologist.

Conclusion

Normative values were presented expressed as the means and standard deviations of T-score (Z-score * 10 + 50).     

Mélanome malin leptoméningé primitif : une étiologie rare de pachy- et leptoméningite

Introduction

Leptomeningitis and pachymeningitis are known to occur consecutive to many causes.

Observation

We report the case of a 24-year-old woman with symptoms of raised intracranial pressure and repeated switching transient hemiparesis. The magnetic resonance imaging (MRI) showed a pachyleptomeningitis. Search for a cause was negative. The pathology examination of meningeal tissue revealed a malignant melanoma, without any sign of cutaneous melanoma, leading to the diagnosis of primary leptomeningeal malignant melanoma.

Conclusion

A meningeal biopsy can enable the rare diagnosis of primary leptomeningeal malignant melanoma in a patient with unexplained pachyleptomeningitis.     

Syndrome de jambe douloureuse et orteils instables associé à une radiculopathie lombaire

Introduction

Painful legs and moving toes (PLMT) is a rare syndrome characterized by spontaneous neuropathic pain in the lower limbs associated with peculiar involuntary movements of the toes. It has been associated with a variety of peripheral and central nervous system diseases. Pathophysiology is unclear and treatment approaches remain largely empirical.

Clinical case

We report a case of a 42-year-old women with typical presentation of PLMT syndrome, associated with lumbar (L₅) disc prolapse. Oxcarbazepine gave a partial improvement.

Conclusion

Clinical presentations and etiological aspects of the PLMT syndrome are described and pathophysiological mechanisms and therapeutic possibilities discussed.