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Biomedical subjects

J de Seze

Publications and source records attributed to J de Seze.

At least 37 records · Page 2Linked to original sources
PubMed2005

Autoimmune hepatitis and multiple sclerosis: a coincidental association?

In the present study we report, as part of a large multiple sclerosis (MS) cohort (1800 patients), three cases of untreated patients who developed autoimmune hepatitis (AIH). The prevalence of AIH in the general population is about 0.0169% and seems to be higher in our MS cohort (0.17%). We suggest that a liver biopsy should systematically be performed in untreated MS patients with a sustained increase of liver enzyme.

Adjuvants, Immunologic↗
PubMed2004

Treatment of progressive forms of multiple sclerosis by cyclophosphamide: a cohort study of 490 patients.

There are no generally effective disease-modifying drugs for progressive forms of multiple sclerosis (MS). Some MS centres use cyclophosphamide (CYC) in secondary progressive (SP) forms of MS, especially after interferon beta-1b (INFbeta-1b) treatment failure. Moreover, there are currently no approved drugs for primary progressive (PP) MS. Using the collected data of patients with progressive MS, we studied clinical patterns that predicted a good response to CYC treatment. Secondly, we compared the therapeutic response of SPMS and PPMS patients to the treatment. Data from 490 MS patients were collected. All patients presented an SP (n = 362) or PP (n = 128) form of the disease and 476 had been treated for at least one year with a monthly pulse of CYC associated with methylprednisolone (MP). CYC treatment was justified because of at least a 1-point worsening on the Expanded Disability Status Scale (EDSS) during the previous year. The EDSS score was assessed at baseline and after 6 months (M6) and 12 months (M12) of treatment. After 12 months of CYC treatment, 78.6% of SPMS and 73.5% of PPMS patients had stabilised or had an improved EDSS score. Response to CYC was not significantly different in the two progressive forms of MS. Twenty-two patients presented noticeable drug side effects, one of whom withdrew from the treatment due to intolerance. Patients with an improved EDSS at M12 had a shorter mean progressive time course (5.1 years) than patients who stabilised or worsened (7.1 years) (p = 0.02). We also observed that poor responders at M6 were also poor responders at M12 (p < 0.001). This large cohort study showed that CYC treatment was well tolerated and suggested that a better response occurred in cases with a short progressive time course. We did not find any difference in treatment response between the two progressive forms of MS. To date, no treatment is approved for PPMS and we therefore propose a trial to test the use of CYC treatment early in the course of the disease in PPMS patients with disability progression.

Adult↗
PubMed2004

[Can multiple sclerosis be diagnosed on a first demyelinating event?].

Progression is required. The definition of multiple sclerosis (MS) relies on the notion of temporo-spatial dissemination of inflammatory demyelinating neurological episodes. Until recently, in order to retain the diagnosis of definite MS, two successive neurological episodes had to be observed in two different territories. Since the commercialisation of new immunomodulating treatments, and in particular the recent demonstration of their efficacy in delaying the onset of a second neurological episode, it has been necessary to draw-up new criteria. New criteria have recently been proposed by a group of experts and they emphasize the fundamental role played by magnetic resonance imaging. New priorities. With these new criteria, certain aspects have become front-line. This is the case of the necessity of foreseeing more exhaustive differential diagnoses of MS, so as to avoid the erroneous initiation of a specific treatment. There is also the problem of the selection of patients at high risk of a potentially progressive MS, not all patients necessarily require such treatment at the start. Updating. We successively present the recently revised diagnostic criteria in order to be able to diagnose MS after the first demyelinising episode, we then study the clinical and paraclinical predictive elements that provide supplementary support for MS and, notably, progressive MS. We also discuss the differential diagnoses to be excluded according to the clinical context and, lastly, we will attempt to assess the practical consequences of an early diagnosis, not only with regard to its announcement, but also with regard to the therapeutic decisions to be made.

Adjuvants, Immunologic↗
PubMed2004

[Neuromeningeal tuberculosis: the contribution of genetic amplification to diagnosis].

Neurological manifestations of tuberculosis are rare, especially in immunocompetent subjects. The heterogeneity of clinical and radiological features induces frequently a delay for diagnosis. The aim of the study was to describe clinical and radiological presentation of 11 cases of neuro-tuberculosis and to evaluate clinical outcome. We performed clinical, CSF, MRI and outcome evaluation in all patients. We also performed a mycobacterium analysis by polymerase chain reaction (PCR). Patients were 6 men and 5 women with a mean age of 45.4 years. Clinical presentations were meningeal symptoms in 9 cases and focal manifestations in 4 cases. CSF was abnormal in 82 p.cent of cases (protein increase in 73 p.cent, pleiocytosis in 73 p.cent, hypoglycorrhachia in 45 p.cent and hypochlorrhachia in 36 p.cent). The best diagnostic test was PCR (positive in 45 p.cent of cases). CSF cultures were positive in only 2 cases (18 p.cent). Only 2 patients had chest involvement. MRI was abnormal in 64 p.cent of cases showing pseudo-tumor, arachnoiditis, vascular lesions or medullar involvement. Outcome was good in all cases but two (one patient died and one patient had paraplegia possibly related to late diagnosis). Neurological manifestations of tuberculosis are extremely various in terms of clinical and radiological presentation. The best diagnostic test seems to be tuberculosis PCR. Outcome is frequently favorable if late diagnosis is avoided.

Adult↗
PubMed2004

[Systemic lupus erythematosus presenting with recurrent psychiatric disturbances].

INTRODUCTION: There is a wide range of non-specific symptoms that can reveal neurolupus, sometimes making diagnosis difficult. OBSERVATION: A 29-year-old man presented, from 1996 to 2002, three episodes of mood disorders with hetero-aggression, preceded by seizures, which resolved completely. Repeated investigations were negative except for lymphopenia, an inflammatory cerebrospinal fluid and some rare non-specific areas of high intensity signals in the white matter on the brain MRI. After a six-year course, the patient was considered to have a severe mood disorder related to a schizoid personality. A new dot-blot search for antinuclear antibodies detected anti-Sm antibodies was positive, leading to the diagnosis of neuropsychiatric lupus since the patient's symptoms fulfilling four of the American Rheumatism Association criteria (neuropsychiatric events, lymphopenia, antinuclear and anti-Sm antibodies). The patient was given monthly pulses of cyclophosphamide and remained symptom free one year after the last flare up. CONCLUSIONS: Lupus can rarely be revealed by long-standing isolated psychiatric disorders. Search for auto-antibodies, using highly specialized techniques (western blot, dot blot) should be a routine practice since antibody titres fluctuate during the course of the disease; elevated titres may correlate with exacerbations. Considering the prominence and severity of these behavior disorders, systemic diseases may often be misdiagnosed.

Adult↗
PubMed2004

[Infectious myelopathies: clinical, serological, and prognostic patterns].

INTRODUCTION: Serological confirmation of an infectious acute myelitis injury is difficult to confirm as it is sometimes due to a post-infectious etiology. OBJECTIVES: The aim of this study was to define the clinical, biological and prognostic patterns of infectious myelitis. PATIENTS AND METHODS: This retrospective study included 153 subjects hospitalized in the department of neurology between 1993 and 2002 for treatment of a noncompressive acute myelopathy. Biological confirmation of recent infection was obtained in 12 patients (8 p. 100). RESULTS: An infectious syndrome, beginning prior to the neurological symptoms, was found in 67 percent of patients. The clinical symptoms were severe with loss of sensoromotor and sphincter functions and ascending spinal cord dysfunction (acute transverse myelopathy). Spinal cord MRI showed extended centromedullar high intensity signals with rapid and complete regression. CSF analysis cell count was above 30/mm3 with hyperproteinorachia, in 75 percent and 58 percent of patients respectively. CSF electrophoresis did not detect oligoclonal bands. Clinical outcome was good in all patients except one, however sphincter disorders recovered slowly. DISCUSSION: Our study illustrates a stereotypical clinical, biological and prognostic pattern for infectious acute myelitis. These findings contribute significantly to therapeutic decision making and establishing prognosis at the initial phase of acute myelopathy.

Adolescent↗
PubMed2004

Intravenous corticosteroids in the postpartum period for reduction of acute exacerbations in multiple sclerosis.

In order to assess the effectiveness of monthly intravenous corticosteroids in reducing childbirth-associated acute exacerbations in multiple sclerosis (MS), we compared pregnant patients followed up in our MS clinic. During the first period (1996-1998), 22 patients did not receive any treatment after delivery. During the second period (1999-2001), following the publication of the PRIMS study, 20 patients were treated monthly with 1 g of intravenous corticosteroids during the six months of the postpartum period. In both groups the relapse rate increased during the first trimester postpartum but it was higher in the untreated group (2+/-0.66 [mean+/-SD]) compared with the treated group (0.8+/-0.41) (P=0.018), suggesting a beneficial effect of monthly intravenous corticosteroids.

Acute Disease↗
PubMed2003

Prospective study of patients presenting with acute partial transverse myelopathy.

BACKGROUND: The clinical and radiological characteristics of myelopathy in multiple sclerosis (MS) are relatively well known. Nevertheless, it remains difficult for the clinician to ascertain conversion to MS after a first episode of acute partial transverse myelopathy (APTM). OBJECTIVE: The aims of this study were to define predictive factors for conversion to clinically definite MS after an APTM and to define predictive factors for disease severity. PATIENTS AND METHODS: Between 1994 and 2001, we prospectively included 55 patients presenting with a first episode of APTM. Three patients were lost during the follow-up. We evaluated clinical signs, spinal cord and brain MRI, cerebrospinal fluid (CSF) and visual evoked potentials on admission. After a mean followup of 35 months (range 12-86), we evaluated the diagnosis and, among the MS group, the severity of the disease. RESULTS: Of the 52 APTM patients who completed the study, 30 became clinically definite MS. The predictive factors for conversion to MS were: initial sensory symptoms, latero-posterior spinal cord lesion, abnormal brain MRI and oligoclonal bands in CSF. In the MS group, the number of spinal cord lesions on MRI was the only predictive factor for a poor outcome, being statistically correlated with a higher number of relapses. CONCLUSION: On the basis of our results, we propose that, in patients with APTM, sensory symptoms, oligoclonal bands and brain MRI are predictive factors for subsequent conversion to clinically definite MS and that within the latter patients the number of spinal cord lesions on MRI is the only predictive factor for a poor outcome.

Acute Disease↗
PubMed2003

Autonomic and respiratory dysfunction in Charcot-Marie-Tooth disease due to Thr124Met mutation in the myelin protein zero gene.

OBJECTIVE: To report the clinical and electrophysiological characteristics of a family presenting Charcot-Marie-Tooth disease (CMT) associated with autonomic nervous system disturbances. METHODS: We studied nerve conduction values, postural adaptation, sympathetic skin reflex, the variation in heart rate by the Valsalva ratio and pupillometry in 7 members of a French family in which CMT due to a Thr124Met mutation in the myelin protein zero (MPZ) gene was diagnosed. RESULTS: Clinical and laboratory evidence of autonomic nervous system disturbances were found in the affected individuals. The clinical phenotype was characterized by sensorimotor peripheral neuropathy, defined as axonal type by electrophysiological studies, and was associated with severe pain, bladder dysfunction, sudorimotor disturbances and abolished pupillary reflex to light. Moreover, two patients had severe restrictive respiratory insufficiency requiring noninvasive mechanical ventilation. CONCLUSIONS: Our study demonstrates that autonomic disturbances may be one of the major clinical signs associated with CMT secondary to MPZ gene mutation in codon 124. Testing of pupillary reflex allows the discrimination of affected and unaffected subjects in our family. However, involvement of the autonomic nervous system in this type of neuropathy is unclear and further studies are required to elucidate the role of the MPZ gene in the autonomic nervous system.

Adult↗
PubMed2003

Multiple sclerosis, interferon beta and clinical thyroid dysfunction.

The objective of this study was to investigate frequency and presentation of clinical thyroid dysfunction in patients treated with interferon beta (IFN-beta). We have collected the cases of clinical thyroid dysfunction in 700 consecutive patients receiving IFN-beta for multiple sclerosis (MS). Five patients (four women, one man) treated with IFN-beta1b developed hyperthyroidism. Three of them have secondary progressive MS, and two have relapsing-remitting MS. It was necessary to stop IFN-beta in three cases; these patients still require carbimazole after several months. In the two other cases, hyperthyroidism disappeared spontaneously. Two patients (one man and one woman) treated with IFN-beta1a developed hypothyroidism. One of them required l-thyroxine. Lastly, an increased thyroid volume without modification of thyroid hormones plasma levels was discovered in a patient receiving IFN-beta1a. Among patients treated with IFN-beta, clinical thyroid dysfunction is much rarer than laboratory thyroid dysfunction. However, this side-effect is sometimes severe.

Adult↗
PubMed2003

Is Devic's neuromyelitis optica a separate disease? A comparative study with multiple sclerosis.

Devic's neuromyelitis optica (NMO) associates optic neuritis and myelopathy without other neurological signs. Many patients with NMO may be diagnosed as having multiple sclerosis (MS). However, there have been no previous studies comparing these two pathologies and it is still unclear if NMO is a separate entity or a subtype of MS. In the present study, we compared a series of NMO patients with a series of MS patients for whom optic neuritis or myelopathy was the presenting symptom, in order to determine the place of NMO in the spectrum of MS. We retrospectively studied 30 patients diagnosed with NMO and we compared these patients with 50 consecutive MS cases revealed by optic neuritis or acute myelopathy. MS patients were only included if a relapse occurred demonstrating time and space dissemination. We compared the two groups in terms of clinical presentation, laboratory findings (MRI and CSF) and clinical outcome. NMO patients were older and more frequently women than MS patients but the difference was not significant. CSF and MRI data were clearly different: oligoclonal bands (OCB) were found in 23% of NMO cases and 88% of MS (P < 0.001), abnormal brain MRI data were observed in 10% of NMO cases and 66% of MS (P < 0.001) and a large spinal cord lesion was observed in 67% of NMO cases and 7.4% of MS cases (P < 0.001). Clinical outcome was evaluated as more severe in the NMO group (P < 0.001). On the basis of clinical data, all NMO patients but three had dissemination in time and space. When we included MRI parameters, only two of the NMO patients met criteria for MS and one of the MS patients met criteria for NMO. Our study demonstrates that NMO and MS should be considered as two different entities. The respective criteria for NMO and MS were able to distinguish these two pathologies but only when MRI data were applied. This finding could have implications for future therapeutic trials.

Adult↗
PubMed2003

[Hypertrophy of the biceps surae after post-radiation lumbar radiculopathy].

A 44 year-old man who underwent radiotherapy for Hodgkin disease in 1987 developed in 1996 pain and calf hypertrophy. Nerve conduction studies were normal and needle electromyography revealed a neurogenic pattern in the L-5 and S-1 roots, predominantly on the left side. An abdominal tomodensitometry revealed a splenic and left renal atrophy. Cerebrospinal fluid analysis showed an elevated protein level (1.35 g/l) and no malignant cell. Spinal cord magnetic resonance imaging revealed no abnormality of the roots and the cauda equina. Muscular hypertrophy, as the consequence of post-irradiation lumbar radiculopathy is rarely reported. We discuss the possible mechanism of neurogenic muscular hypertrophy.

Adult↗
PubMed2002

Devic's neuromyelitis optica: clinical, laboratory, MRI and outcome profile.

Devic's neuromyelitis optica (NMO) associates optic neuritis and myelitis without any other neurological signs. Many patients with NMO may be diagnosed as having multiple sclerosis (MS), optic neuritis and myelitis being the inaugural symptom in 20% and 5% of MS cases, respectively. The aim of our study was to compare a new NMO cohort with recent studies and to try to determine the place of NMO in the spectrum of MS. We retrospectively studied 13 patients with a complete diagnostic workup for NMO. We compared our data with the most recent studies on NMO and with the criteria proposed by Wingerchuck et al. [Neurology 53 (1999) 1107]. We also determined whether these patients fulfilled the diagnostic criteria for MS. Thirteen patients (10 women and three men, with a mean age of 37.4 years) were included in the study. We found similar results to previously published data, except for an association with vasculitis in 38% of our cases. All but three of the patients fulfilled the clinical criteria for MS and two patients fulfilled both clinical and MRI criteria for MS. However, if we applied more restrictive criteria concerning spinal cord and brain MRI and CSF, none of our NMO patients fulfilled the MS diagnostic criteria. NMO might therefore be differentiated from MS by the application of more stringent criteria. Furthermore, all NMO patients should be investigated for vasculitis, even those with no history of systemic disease.

Adrenal Cortex Hormones↗
PubMed2002

Interferon beta1a (Avonex) treatment in multiple sclerosis: similarity of effect on progression of disability in patients with mild and moderate disability.

OBJECTIVE: To compare clinical responses to once-weekly intramuscular interferon-beta-1 a [IFNbeta-1 a, Avonex, Biogen] in multiple sclerosis (MS) patients with baseline Expanded Disability Status Scale (EDSS) < or = 3.5 or > 3.5. METHODS: Patients with relapsing-remitting MS (RRMS), 124 with baseline EDSS < or = 3.5 and 64 RRMS patients with EDSS > 3.5, were consecutively recruited to receive IFNbeta-1 a 30 microg as a once weekly injection for 18 months. The primary endpoint of the study was the number of patients in each group with sustained worsening in disability, defined as 1-point deterioration in EDSS that persisted for at least 6 months during the 18 month follow-up period. Subordinate endpoints included relapse rates and the number of treatment dropouts. RESULTS: Among patients with baseline EDSS < or = 3.5,16.9% experienced a deterioration in EDSS of at least 1 point; 22.5% experienced a deterioration of at least 0.5%. Corresponding rates in patients with baseline EDSS > 3.5 were 23.4% and 29% respectively (no significant differences between patients stratified according to baseline EDSS status). The proportion of patients discontinuing therapy was significantly higher in patients with baseline EDSS > 3.5 than in those with baseline EDSS < or = 3.5 (16/64 versus 12/124; p = 0.005). At the conclusion of follow-up, IFNbeta-1 a therapy was associated with a 31.7% reduction in relapse rate in patients with baseline EDSS < or = 3.5 and a 37% reduction in those with baseline EDSS > 3.5 (difference not significant). CONCLUSIONS: During 18 months of treatment and follow-up, no difference was observed in clinical responses to IFNbeta-1 a between RRMS patients with mild and moderate disability but discontinuation of therapy was more frequent in the more disabled group.

Adjuvants, Immunologic↗
PubMed2002

Interferon beta in multiple sclerosis: relationship between sustained serum IgG levels and clinical outcome.

We investigated the effects of interferon beta-1a (IFN beta-1a) on specific response towards two immunodominant MBP peptides and on global production of IgG. We evaluated 54 sera from multiple sclerosis (MS) patients at baseline and 1 year after treatment. We did not observe any modification of immune response to the MBP peptides but we noted a significant decrease in mean IgG concentrations in patients with progression of the disease but not in stable patients. These results suggest that IFN beta1a restores or maintains a beneficial immune response.

Adult↗
PubMed2002

[Interferons and neurologic diseases].

PURPOSES: Therapeutic strategies using interferons in neurology are limited to multiple sclerosis. In this disease, only beta interferons are used and now considered as a first line treatment in the relapsing-remitting forms of the disease. MAIN POINTS: Beta interferons have reduced the relapse rate by approximately 3% and also reduced the risk of disability progression. They have also favorably influenced the brain lesions on MRI. Numerous data have stressed the importance of starting treatment as early as possible. In the secondary form of the disease, the impact of beta interferons are still discussed. In some clinical endpoints, a dose-response has been demonstrated, probably related to the frequency of injections. Side effects are acceptable despite the high frequency of flu-like syndrome. PERSPECTIVES: Recent data argue for beta interferon treatment after the first demyelinating episode in subjects at risk for early new relapses. In some patients, in cases of treatment failure, combination strategies, including beta interferons, should be addressed in the near future. Interferons were also tested with disappointing results in other neuro-inflammatory disorders, such as chronic inflammatory demyelinating neuropathies.

Adjuvants, Immunologic↗