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J De Seze

Publications and source records attributed to J De Seze.

At least 19 recordsLinked to original sources
PubMed2004

Low levels of the vascular endothelial growth factor in CSF from early ALS patients.

Deletion of the hypoxia-response element in the vascular endothelial growth factor (VEGF) promoter causes motor neuron degeneration in a mouse model. "At-risk" haplotypes with low circulating VEGF levels have been demonstrated in humans. Here the authors report low VEGF levels in the CSF of ALS patients during their first year of the disease, independently of VEGF promoter polymorphism. This finding early in ALS patients suggests a possible role for VEGF gene regulation in the pathogenesis of ALS.

Aged↗
PubMed2004

Acute aphasia in multiple sclerosis: A multicenter study of 22 patients.

Aphasia is usually considered to be rare in multiple sclerosis (MS). To determine the clinical and radiologic characteristics of MS patients with acute aphasia, the authors investigated data from 2,700 patients from three MS centers and found 22 patients with acute aphasia (0.81%). Aphasia was the first clinical manifestation of MS in eight patients (36%). Brain MRI showed giant plaques in eight cases (40%). A full recovery was observed in 14 patients (64%). Furthermore, acute aphasia did not appear to be a criterion for poor prognosis.

Acute Disease↗
PubMed2004

[Multiple sclerosis with sensory disturbances and hyperhidrosis of the hand].

INTRODUCTION: Hyperhidrosis may result from brain, spinal cord or peripheral nervous system injuries. We report the case of a 26-year-old patient who presented with sensorial disorders and focal hyperhidrosis of the right hand. OBSERVATION: MRI revealed multiple lesions very suggestive of active demyelinization. A lesion in the left paramedian posterior portion of the pons was enhanced after gadolinium infusion. The diagnosis of multiple sclerosis was suggested by MRI data and oligoclonal IgG bands on cerebrospinal fluid electrophoresis. CONCLUSIONS: Focal hyperhidrosis may be due to a lesion of the crossed sympathetic inhibitory tract. Although autonomic dysfunction is common during the late course of multiple sclerosis this case is, to our knowledge, the first report of focal hyperhidrosis revealing multiple sclerosis.

Adult↗
PubMed2004

CSF isoelectrofocusing in a large cohort of MS and other neurological diseases.

The present study was performed in order to confirm the diagnostic value of isoelectrofocusing (IEF) in a large multiple sclerosis (MS) cohort and to evaluate the various neurological diseases probably to present a similar IEF profile. The cerebrospinal fluid (CSF) of 1292 patients with neurological diseases was studied by IEF. After a follow-up of 2-36 months, we only included patients with a definite MS or confirmed diagnosis of other neurological diseases (OND). MS was diagnosed in 407 patients and OND in 593 patients. For patients in whom three or more oligoclonal bands (OCB) were detected, IEF results showed a sensitivity of 85% and a specificity of 92% for the diagnosis of MS. The positive and negative predictive values were 86.5 and 90%, respectively. Inflammatory and infectious disorders of the central nervous system represented the main affections associated with OCB, including human immunodeficiency virus encephalitis, Lyme disease and less frequently Sjogren syndrome. Furthermore, when OCB were observed, 10 or more bands were more frequently found in MS than in OND (P < 0.0001). IEF of the CSF is a reliable method for the diagnosis of MS. The absolute number of bands may help to discriminate between MS and OND.

Acquired Immunodeficiency Syndrome↗
PubMed2003

Double-blind crossover study with dolasetron mesilate, a 5-HT3 receptor antagonist in cerebellar syndrome secondary to multiple sclerosis.

Cerebellar syndrome is one of the most disabling developments in multiple sclerosis (MS). In neurodegenerative disorders, cerebellar syndrome is thought to be related to a neurochemical deficit of 5-hydroxytryptamine (5-HT). Previous studies found that a levorotatory form of 5-hydroxytryptophan, a 5-HT precursor, and ondansetron, a 5-HT(3) receptor antagonist, decreased cerebellar symptoms in Friedreich's ataxia and MS. We studied the effect of another 5-HT(3) receptor antagonist, dolasetron mesilate, on cerebellar syndrome in MS patients.Thirty-four MS patients were included in a placebo-controlled double-blind crossover study. They received a single dose of intravenous dolasetron mesilate or placebo. A quantitative evaluation of cerebellar syndrome using the nine-hole peg test and an ataxia score comprising static and kinetic parameters were performed before and after each treatment. No statistical difference was observed in the dolasetron mesilate group, compared with the placebo group. There was, however, inter-individual variability in the treatment response. This double-blind study on cerebellar syndrome in MS patients did not confirm the positive effect of dolasetron mesilate suggested by previous studies.

Adolescent↗
PubMed2002

[Treatment of progressive multiple sclerosis with cyclophosphamide].

Intravenous (IV) cyclophosphamide is currently used in secondary progressive (SP) and Primary progressive (PP) Multiple Sclerosis (MS) but its efficacy remains uncertain. Furthermore, it is necessary to determine which MS should be successfully treated with IV cyclophosphamide. We retrospectively investigated 111 consecutive patients with progressive MS (21 PPMS and 90 SPMS) treated in an open label fashion with IV cyclophosphamide. We analysed clinical data (gender, age, duration of progression, primary versus secondary MS). The treatment response was assessed by EDSS change after 6 months and 1 year of treatment. The annual relapse average decreased from 1.92 before treatment to 0.39 during the treatment. Age and gender did not influence response to therapy. We did not find any difference of response between PPMS and SPMS. Duration of the progressive phase in SPMS was not a predictive factor of efficacy. A better response was noted in SPMS patients with surimposed relapses than in patients without relapses during the year before treatment (p<0.05). Furthermore, the better response in SPMS patients with relapses before treatment suggests that it is necessary to treat when MS is still in an inflammatory stage.

Adult↗
PubMed2002

[Acute rhombencephalitis with fever and bilateral hearing loss secondary to Gougerot-Sjögren's syndrome].

The usual polymorphic central nervous system involvement observed in primary Sjögren's syndrome is classified as monofocal, multifocal or diffuse involvement. We present a case of acute rhombencephalitis associated with hearing loss that could correspond to an intermediate form between diffuse and monofocal involvement. The acute onset with fever and not clinical sign of xerostomia or xerophthalmia suggest the possibility of vascular-like or infectious-like forms. We emphasize the importance of obtaining a minor salivary gland biopsy for diagnosis of Sjögren's syndrome and suggest that Sjögren's syndrome could be frequently misdiagnosed.

Abducens Nerve Diseases↗
PubMed2002

[Primary progressive forms of multiple sclerosis: application of the new diagnostic criteria in French].

Approximately 15 p.100 of all cases of multiple sclerosis (MS) are primary progressive multiple sclerosis (PPMS). Diagnosis is however frequently delayed due to the lack of relapse. The commonly used Poser criteria established in 1983 are not directly applicable to this subgroup of MS patients. In 2000, Thompson et al proposed new diagnostic criteria for PPMS. The aim of our study was to apply retrospectively these new criteria to a cohort of patients from northern France (G-SEP cohort). The cohort included 184 patients (94 women and 90 men, sex ratio=1.04). Mean age at disease onset was 40.3 years (18-67 years) and mean follow-up was 9.9 (1-39 years). Only one patient was older than 65 years but 13 patients (7.1 p.100) were younger than 25 years. Patients were classed as having definite, probable or possible PPMS on the basis of clinical, MRI, cerebrospinal fluid and visual evoked potential (VEP) data. Most of the patients (61.4 p.100) had one symptom at onset; spastic paraparesis was frequent (79 p.100). Mean delay to diagnosis was 4.4 years. Ten patients (5.4 p.100) were surgically treated for cervic arthrosic myelopathy. Mean EDSS score at the end of follow-up was 5.8 (3-10). MRI was positive in 87.3 p.100 of the patients. Oligoclonal bands were found in 78.2 p.100. VEPs ware abnormal in 79.9 p.100 of the patients. Applying the Thompson criteria, 57.7 p.100 of the patients had definite PPMS, 38.7 p.100 probable PPMS. Our results are very similar to previous studies and confirm the usefulness of the new proposed criteria, which however should be prospectively tested to determine sensitivity and specificity in a new cohort of patients.

Adolescent↗
PubMed2002

[Chronic polyradiculoneuritis disclosing sarcoidosis].

Peripheral Nervous System (PNS) involvement occurs in 10 to 40 percent of patients presenting with sarcoidosis. Axonal polyneuropathies and mononeuropathies are the most frequently reported whereas acute or chronic polyradiculoneuritis are rarely described. We report the case of a 54 year-old-woman admitted for a progressive sensorimotor deficit of the lower limbs. Bilateral ocular redness corresponding to an uveitis was also observed. Neurological examination and electrophysiological data were consistent with a chronic polyradiculoneuritis. The neurological status of the patient improved after six months of treatment with oral corticotherapy associated with immunosuppressive treatment. Our case allows a review of the literature concerning PNS involvements and treatments in sarcoidosis.

Brain↗
PubMed2002

[Candida albicans meningoencephalomyeloradiculitis].

A 25-year-old immunocompetent male heroin addict was admitted for acute confusion associated with gait disorders of three month duration. The diagnosis was meningoencephalomyeloradiculitis secondary to Candida albicans infection. Outcome was good after a 6-month regimen with antifungal drugs. Neurological complications of Candida albicans infection are rare and prognosis is generally poor. This case report illustrates diagnostic and therapeutic difficulties encountered.

Adult↗
PubMed2002

[Epstein-Barr meningoencephaloradiculitis in a immunocompetent woman].

Neurological manifestations of viral diseases are predominantly secondary to herpes viridae infections. Among them Epstein-Barr (EBV) infections are rare particularly in immunocompetent adults. A 52 year old right-handed woman was admitted for progressive walking difficulties. She presented a proximal limb weakness with proprioceptive ataxia and pain because of lumbosacral radiculopathy confirmed by electromyography. Drowsiness and mnesic troubles were explained by encephalitis on electroencephalography. Brain MRI showed an enhancement of the sub-arachnoïd spaces. The diagnosis of meningoencephaloradiculitis due to primary EBV infection was supported by a mononucleosis syndrome, an increased antibody level and positive EBV PCR in the CSF. Clinical features spontaneously improved without antiviral treatment. Primary EBV infections are rare in adults, as more than 95p.cent of them are positive for EBV serology after 20 years of age. EBV PCR is CSF in an important diagnostic tool allowing to stop antiviral therapies in these cases having a good prognosis.

Antibodies, Viral↗
PubMed2002

[Late onset multiple sclerosis].

Multiple sclerosis (MS) with clinical onset after 50 years old is unusual and frequently misdiagnosed. Clinical presentation and course seem also to be different that in MS occurring between 20 and 50 years old. The aim of this study was to evaluate the clinical and paraclinical characteristics of late onset MS. We respectively studied MS patients older than 50 years at the onset of the disease. We evaluated demographical data, clinical symptoms, cerebrospinal fluid (CSF), visual evoked potentials (VEPs) and MRI of these patients. We also studied clinical data during the follow-up with the occurrence of new symptoms and the evolution of the disease by the index of progression (EDSS unit per year). In a population of 1 417 MS, 3.4 p.cent had their first symptoms at 50 years old or older and 0.45 p.cent after 59 years old. At the time of the study patients had more frequently a progressive form: 37 p.cent had a primary progressive form and 35 p.cent a secondary progressive MS. None of the patients with onset after 60 years old had relapsing remittent MS. Motor symptoms were the most common neurologic presentation (54 p.cent of patients). Very few patients had a clinical optic nerve involvement during the follow-up. The mean progression index was 1 suggesting a most severe evolution in this subgroup of MS patients. 76 p.cent of patients had oligoclonal banding detected by CSF electrophoresis. The VEPs were abnormal in 81 p. cent of patients tested. 71 p.cent of the brain MRI were consistent with the diagnosis of MS. 60 p.cent of patients had spinal cord MRI abnormal. This study highlights the differences between the late onset MS and earlier onset. As previously reported, our study underlines the high frequency of progressive course, motor function involvement and poor prognosis.

Age of Onset↗
PubMed2001

Molecular analysis of endogenous retrovirus HRES-1: identification of frameshift mutations in region encoding putative 28-kDa autoantigen.

A possible involvement of HTLV-1-related endogenous sequence 1 (HRES-1) in autoimmune diseases has been recently reported. In primate cells, PCRs and RT-PCRs using specific primers reveal the presence and the transcription of gag-related sequences. However antisera generated against selected HRES-1 peptides failed to detect a 28-kDa protein deduced from the translated gag ORF and described previously. Such discordant results led us to perform DNA cloning and sequencing of LTR- and gag-related nucleotidic fragments. Repeated sequence analyses on distinct samples revealed frameshift mutations in the gag and LTR ORFs. Our sequence analyses detected a stop codon in the gag-related ORF, which is inconsistent with the expression of a 28-kDa protein. Instead of the two ORFs previously found, our gag-related region contained three ORFs. One of them demonstrated higher nucleotidic and peptidic homologies with the p19 gag of HTLV-I. However, the molecular analyses of our new sequence did not show evidence of potent translation capacities.

Amino Acid Sequence↗
PubMed2001

Electrophysiological effects of corticosteroids on the retinal pigment epithelium.

PURPOSE: As corticosteroids appear to intervene in pathogenesis of central serous chorioretinopathy, ion transport changes within the retinal pigment epithelium (RPE) might be involved. Electrophysiological responses to corticosteroid administration were recorded in vivo and in vitro. METHODS: Clinical study: The standing ocular potential was recorded during intravenous (IV) infusion of glucose 5% and glucose 5% + prednisolone 0.2% in 14 patients with relapsing multiple sclerosis. The results were compared with a control group receiving two successive identical glucose 5% infusions. In vitro study: Native tissue explants (RPE + choroid, porcine, and bovine) were placed in a Ussing-type chamber. After baseline determination of the transepithelial potential (PD), short circuit current (I(sc)) and transepithelial resistance (R(t)), the effect of apical hydrocortisone (HC) 10(-4) M was determined. RESULTS: Clinical study: A significant rise of the standing potential was found after glucose infusion (P = 0.005), whereas no change was detected after IV glucose + prednisolone (P = 0.695). In vitro study: In the porcine RPE, the mean baseline PD and I(sc) were significantly reduced (both P: = 0.012) after applying apical 10(-4) HC. R(t) was also significantly reduced (P = 0.01). The same type of response, observed in bovine RPE, was reduced in low chloride/low bicarbonate conditions. CONCLUSIONS: Corticosteroids modified electrophysiological parameters representing RPE function in vivo. The existence of an RPE-specific effect was confirmed in vitro. Further work is required to link the observed ion transport changes to a reduction of apical, subretinal fluid absorption.

Animals↗
PubMed2001

[Kjellin syndrome].

A previously healthy 30-year-old woman who had cognitive impairment since childhood suddenly developed progressive spastic paraparesis. Visual impairment and characteristic retinal macular spots supported the diagnosis of Kjellin syndrome. This disease, probably transmitted by autosomal recessive inheritance, is seldom observed in clinical practice. We describe the characteristics of Kjellin syndrome and the differential diagnosis, including other macular changes associated with spastic paraparesis.

Adult↗
PubMed2001

[Sarcoidosis and progressive multifocal leukoencephalopathy].

A 70-year-old woman treated for sarcoidosis complained of progressive cognitive impairment and gait disability. Magnetic resonance imaging of the brain revealed a nonenhancing lesion in T1-weighted imaging in the left parieto-occipital region and sarcoidosis of the central nervous system was evoked. However, she rapidly deteriorated with posterior and cerebellar extension of the lesions, suggesting of progressive multifocal leukoencephalopathy (PML). DNA of the JC virus (JCV) was detected in the cerebrospinal fluid (CSF) by a polymerase chain reaction. Despite antiviral therapy, she died nine months after the first neurological signs. This case illustrates the possible association between sarcoidosis and PML, and underlines the interest to detect the presence of JCV in the CSF when the diagnosis of neurosarcoidosis appeared uncertain.

Aged↗
PubMed2000

[Spinal cord magnetic resonance imaging in multiple sclerosis: importance of determining degree of atrophy as a marker of disease course].

Spinal cord magnetic resonance imaging (MRI) is of particular interest in the management of multiple sclerosis (MS) especially in primary progressive forms. Most of the demyelinating lesions are located in the cervical or dorsal cord. Spinal cord area reduction has been recently correlated with the progression of disability (Losseff et al., 1996, Lycklama a Nijeholt et al., 1998). The aim of this study was to confirm this first result, to assess the reproducibility of this method and to correlate demyelinating lesions with spinal cord area reduction. Fifty two patients were included and compared with 15 controls (normal subjects). T2 Sagittal and axial plane images were performed to localized hypersignal lesions. Spinal cord area was obtained by a volume acquired inversion prepared fast spoiled gradient echo acquisition (MP-Rage) sequence. We compared the mean area value with clinical parameters (age, course of the disease, expanded disability status scale ¿EDSS) and with the number and location of demyelinating lesions. Demyelinating lesions were found in 82p.100 of MS patients and in none of controls. Mean spinal cord area was closely similar to Losseff et al. (1996) results and was reduced compared with controls (p<0.001). Spinal cord reduction was correlated with disability, studied by the EDSS. Furthermore, no correlation was found between demyelinating lesions and spinal cord area reduction. This study confirms the interest of spinal cord area mesurement in MS. Spinal cord atrophy is a reliable marker for axonal loss. This method should be of particular interest for the follow-up of axonal loss in thepeutic trials especially in primary progressive MS.

Adult↗