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.     

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

Introduction

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

Case report

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

Conclusion

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

MRI features of neurosyphilis

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.     

Démence à corps de Lewy atypique révélée par une glossodynie et des troubles pseudo-psychiatriques

Introduction

Among the degenerative diseases of the nervous system, Lewy body disease has the most psychiatric symptoms especially hallucinations, delusion and identification disorders.

Case report

We report a case of Lewy body disease that started with a burning mouth syndrome for three year as the only symptom before the development of a pseudo-psychiatric syndrome (melancholy and Capgras). None of the usual cardinal criteria were present. MRI, cerebrospinal fluid, and DAT scan^® findings enabled the diagnosis.

Conclusion

The dopaminergic hypothesis put forward in some cases of burning mouth syndrome might explain this symptom in Lewy body disease.     

Hémiballisme vasculaire par atteinte extraluysienne

Introduction

Hemiballismus is a very rare disorder; less than 7% of all hyperkinetic disorders. Classically, a lesion of the contralateral corpus Luysii is involved. We report a case of hemiballismus due to an extraluysian lesions and discuss the underlying pathophysiology.

Case report

A 74-year-old women, with a history of diabetes and hypertension, developed left-sided hemiballismus one week before admission. The brain MRI showed a right lenticular nucleus hematoma with vascular leucoencephalopathy. The patient was given haloperidol and recovered well. The brain MRI showed the integrity of both corpus Luysii.

Conclusion

Hemiballismus has been classically characterized as pathognomonic of a lesion in the contralateral corpus Luysii. However, many cases due to an extraluysian lesion of the striatum, caudate or thalamus have been reported recently. Prognosis is benign in most cases.     

Left mesiotemporal lesions and anterograde memory impairement: a case of neurosyphilis

Introduction

Neurosyphilis has become uncommun in the developed countries.

Observation

We report a case of neurosyphilis with limbic presentation, left mesiotemporal lesions on MRI and severe anterograde amnesia.

Discussion

Pathogeneses of MRI findings are unknown. We suggest the implication of arteritis wich affects small vessels, parenchymatous and excitotoxic lesions. The absence of mesiotemporal lesion in immunodeficient patients, the limbic systematization of pathology underlines the involvement of probably auto-immune process. Neurosyphilis should always be considered in the differential diagnosis of limbic encephalitis in order to initiate treatment and to prevent cognitives sequelaes. At last, partial status epilepticus should be diagnosed and excitotoxicity lesions prevents with antiepileptic treatment.     

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.     

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.     

Place de l’IRM dans l’orientation diagnostique et le suivi des maladies métaboliques : intérêt des nouvelles techniques

Introduction

Conventional MRI allows diagnostic and prognosis approaches for patients with suspected metabolic disease.

Background

Bilateral and symmetrical abnormalities are the most suggestive aspects. Signal characteristics (intensity, difference of signal on various sequences), lesion extension and location provide important etiological information. Non-conventional MRI techniques are particularly interesting for pathophysiology. Different MRI modalities offer promising techniques for monitoring treatments and patient follow-up.

Conclusion

Combining different MRI modalities can contribute to the diagnosis and help improve understanding of the pathogenic mechanisms of adult metabolic diseases; they offer promising options for the prognosis and treatment follow-up.     

Myélopathie sarcoïdosique associée à un canal cervical rétréci : aspects IRM

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.     

Hémianopsie latérale homonyme et atrophie corticale postérieure

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.     

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.     

Contribution of CSF/serum immunofixation to the diagnosis of inflammatory diseases of the central nervous system

Introduction

Multiple sclerosis and other inflammatory diseases of the central nervous system produce various and nonspecific symptoms. The diagnosis of these diseases is ultimately a clinical decision, although examination of cerebrospinal fluid (CSF) and other complementary tests such magnetic resonance imaging (MRI) and evoked potentials can be contributive. One important aspect of these diseases is intrathecal synthesis of immunoglobulins.

Patients and methods

In order to determine the contribution of CSF/serum immunofixation to the diagnosis of inflammatory diseases of the central nervous system, we conducted a retrospective study in the biochemistry laboratory of the military instruction hospital Mohammed V. 363 CSF/serum samples were collected over a period of four years.

Results

Immunofixation was less sensitive than MRI for the diagnosis of inflammatory neurological disease (44% vs 87%), but was much more specific than MRI (97% for immunofixation vs 38%). The positive predictive value was higher that for MRI (85% vs 40%). The negative predictive value (80%) was close to that of MRI (86%). The bivariate analysis showed that immunofixation results could be predicted from clinical findings and complementary test results such as the index of Link, syphilis serology in CSF, and MRI.

Conclusions/discussion

Semi-automatic Hydrasys immunofixation of CSF IgG is a technique exhibiting excellent diagnostic and analytical performance for the diagnosis of inflammatory neurological diseases.     

Neuromarketing: When marketing meet neurosciences

Introduction

The emergence of brain imaging in recent years has been accompanied by an alliance between neuroscientists and marketers. This collaboration gave birth to “neuromarketing”, a new field that uses imaging techniques with the aim of resolving marketing issues.

State of the art

Several studies have shown that pleasure felt at the sight of a product or after its consumption, is activated by a reward system involving ventral striatum. Since then, marketers seeking exploit this data and have found that some marketing actions can generate added satisfaction in a placebo-like manner. However, neuromarketing suffer from many limits that are a barrier to its development and its scope is restricted.

Perspectives

Through this article, we attempt to give an overview on neuromarketing and its neural correlates while provide a perspective toward the use of field for less commercial purposes.

Conclusion

The neuromarketing is a new field which efficiency is not proven. Its results must be interpreted with caution.     

Évènements psychotiques inauguraux de sclérose en plaques ?

Introduction

Psychotic symptoms are not readily recognized in multiple sclerosis, especially at the beginning of the disease.

Methods

We report the cases of four patients who developed psychotic symptoms that led to the diagnosis of multiple sclerosis. We describe the psychiatric and neurological features, MRI findings, clinical outcome and treatment.

Results

Two patients developed persecutory delusions, one presented a manic episode and the fourth melancholia with catatonia. Mean age was 39 years (range 20-49 years). Two patients had a personal history, but none a familial history of psychiatric disease. Examination of the cerebrospinal fluid revealed an oligoclonal pattern in all patients. All patients fulfilled Barkhof's MRI criteria. Three have had brain MRI with injection during psychotic symptoms. In these three cases, a frontal lesion appeared. The patient with catatonia also had a new lesion in the cerebellum and in the brainstem. All patients needed a “psychiatric” treatment, including antipsychotics. The psychiatric event lasted three months for two patients and the two others experienced relapse.

Conclusion

Acute psychiatric symptom may reveal multiple sclerosis at the beginning of the disease. Frontal lobe localization is suggested. We propose that a psychotic event may correspond to a multiple sclerosis event.     

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.     

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.     

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.     

Polynévrite, crise convulsive, syndrome cérébelleux, complication d’un traitement par métronidazole. Apport de l’IRM

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.     

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.