Diplégie faciale au cours d’une neuropathie démyélinisante inflammatoire aiguë secondaire à une varicelle

Introduction

Primo-infection by varicella-zoster virus (VZV) may be associated with several neurologic complications. Bilateral facial palsy is a rather uncommon manifestation.

Case report

We report the case of a 38-year-old woman who developed bilateral facial diplegia and paresthesia affecting all four limbs with subacute onset several days after varicella virus primoinfection. Ancillary tests showed hyperproteinorachia and signs of demyelinating polyneuropathy in nerve conduction tests. The diagnosis of Guillain-Barré syndrome was retained and a treatment with intravenous immunoglobulines was started, leading to progressive improvement.

Conclusion

Immunotherapy is a possible therapeutic approach in the context of neurologic postinfectious complications after VZV infection where an underlying mechanism is probable.     

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”.     

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.     

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.     

Première évaluation d’un programme de remédiation cognitive chez des patients atteints de sclérose en plaques : PROCOG-SEP

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.     

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.     

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.     

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.     

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.     

Une méningovascularite infectieuse de Lyme révélée par des accidents ischémiques cérébraux successifs

Introduction

Infectious vascularitis is an unusual cause of ischemic stroke (IS). We report a case of Lyme meningovascularitis complicated with multiple IS.

Case report

A 64-year-old man, without any cardiovascular risk factor, was admitted for a right hemiparesia with a left thalamic hypodensity on the initial cerebral CT scan. No cause for this presumed IS could be identified. Later, the patient developed cognitive impairment and a bilateral cerebellar syndrome. Multiple infarcts and multiple intracranial stenosis were seen on cerebral MRI with magnetic resonance angiography (MRA). Cerebrospinal fluid tests showed meningitis and positive Lyme serology with an intrathecal specific anti-Borrelia antibody index. Antibiotic treatment was followed by good biological and partial clinicoradiological outcome.

Conclusion

The diagnosis of Lyme neuroborreliosis should be entertained as a possible cause of IS in highly endemic zones.     

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.     

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.     

Progressive paraparesis with sensorineural deafness and leukoencephalopathy revealing neurobrucellosis

Introduction

Brucellosis is a rare disease with variable neurological and imaging manifestations.

Case report

A 54-year-old woman presented with progressive paraparesis and sensorineural hearing loss over 18 months. The presence of diffuse white matter changes on brain magnetic resonance imaging (MRI) and cerebrospinal fluid findings led to the diagnosis of neurobrucellosis. Spinal MRI did not show any lesion that could explain the paraparesis that was finally attributed to the leukoencephalopathic lesions. The patient improved clinically after three months of antibiotic treatment but no significant changes were noticed on brain imaging.

Discussion and conclusion

Neurobrucellosis is a treatable disease which should be discussed when a patient presents with the triade progressive paraparesis, sensorineural hearing loss and leukoencephalopathy on MRI. Outcome can be favorable if adapted treatment is given early.     

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.     

Métamémoire, remémoration et familiarité dans la maladie d’Alzheimer

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.     

Apport diagnostic des marqueurs biologiques de la maladie d’Alzheimer (MA) dans une observation de dégénérescence lobaire fronto-temporale (DLFT)

Introduction

Cerebrospinal fluid biomarkers are a fundamental contribution for the diagnosis of Alzheimer disease, in particular for young people.

Case report

We report a case of Alzheimer disease of a 51-years old man whose clinical presentation corresponded to frontotemporal behavioural criteria. The clinical evaluation noticed some troubles in verbal memory. Only the CSF biomarkers confirmed the diagnosis.

Conclusion

There are different phenotypes of presenile Alzheimer disease and among them we note frontotemporal dementia. It is very important to identify them to introduce a specific treatment.     

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.     

Rechute intracérébrale isolée dans la maladie de Hodgkin

Isolated brain relapse is uncommon in Hodgkin's lymphoma and often linked with advanced or refractory disease.

Case reports

We report two patients with stage IV Hodgkin's disease, previously treated with chemotherapy, who later developed neurological symptoms. Brain MRI demonstrated two different types of lesions. Histological findings were consistent with intracranial location of Hodgkin lymphoma. Both of the patients presented only with isolated CNS relapse of the lymphoma.

Conclusion

The two cases demonstrated the MRI appearance of intracranial involvement can be varied and mimic other disease processes in patients with a previous history of Hodgkin's lymphoma. Thus, a biopsy is necessary for diagnosis and correct treatment.     

Hémorragie méningée focale et angiopathie amyloïde cérébrale : une association non fortuite

Introduction

Cerebral amyloid angiopathy is a degenerative angiopathy due to amyloid deposits in the walls of the meningeal and cortical vessels. It is considered as a major cause of cerebral hemorrhage to the elderly. It was recently demonstrated that the association of focal meningeal bleedings and cerebral hemorrhage is very suggestive of cerebral amyloid angiopathy. In contrast, the links between subarachnoid hemorrhage and amyloid angiopathy are less well-known.

Cases reports

We report nine cases of cerebral amyloid angiopathy. The clinical presentation was variable, but all had at least one inaugural meningeal bleeding. As cortico-meningeal biopsies were not performed the Boston criteria were used to establish the diagnosis.

Conclusion

Cerebral amyloid angiopathy is an underestimated cause of subarachnoid hemorrhage. Our observations show that this diagnosis should be evoked when focal meningeal bleeding occurs without head trauma or when focal subarachnoid hemorrhage is followed by a subcortical hematoma in an elderly subject.