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Biomedical subjects

P Vermersch

Publications and source records attributed to P Vermersch.

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

Mycophenolate mofetil and neurological diseases.

We describe experience with the use of mycophenolate mofetil (MMF) in neurological diseases. Although only small series of patients or case reports were described, MMF is promising in immune-mediated neuromuscular disorders. MMF has been used for the treatment of polymyositis, chronic inflammatory demyelinating polyradiculoneuropathy, and multifocal motor neuropathy. These studies showed that MMF is well tolerated and may be useful in some patients. MMF can be effective alone but mainly as an adjuvant therapy by reducing steroid requirements or the frequency of infusions of IVIg. MMF has also been tested alone as a single drug treatment or in combination with immunomodulatory drugs in multiple sclerosis in open surveillance trials or in phase II studies. None of these studies have been designed to demonstrate a clinical efficacy but preliminary results are very promising.

Humans↗
PubMed2005

One-year cyclophosphamide treatment combined with methylprednisolone improves cognitive dysfunction in progressive forms of multiple sclerosis.

We conducted an evaluation of changes in cognition in progressive multiple sclerosis (MS) patients receiving monthly intravenously pulse of cyclophosphamide (700 mg/m2) with methylprednisolone (1g). Twenty-eight consecutive progressive MS patients (10 primary progressive, 18 secondary progressive MS) were evaluated before and after six and 12 months of treatment. The WAIS-R score, memory and executive functions were evaluated. Under treatment we found a significant improvement in global cognitive efficiency, encoding abilities, planning abilities and inhibition after six and 12 months. However, mechanisms of action of the positive effect of these anti-inflammatory and immunosuppressive treatments on cognition remain unclear.

Adult↗
PubMed2005

Sequential magnetic resonance imaging follow-up of multiple sclerosis before the clinical phase.

There is much evidence of the importance of the preclinical phase of multiple sclerosis (MS). However, apart from a recent report of the incidental discovery of a case of primary progressive MS, there are no data on the sequential magnetic resonance imaging (MRI) follow-up of patients before the clinical phase. We report the incidental discovery of white matter changes on MRI and their follow-up in a patient three years before the first neurological event (optic neuritis). A 34-year-old woman presented with headaches and depression after her young daughter had been involved in a car accident and spent two weeks in intensive care. The woman's general practitioner performed a brain MRI, which revealed multiple T2-weighted hypersignals suggesting MS. During the next three years, clinical examination remained normal but we observed new T2 lesions and/or new enhanced T1 lesions after gadolinium infusion on the four successive MRIs. Thirty-seven months after the first MRI, the patient developed a right optic neuritis. The diagnosis of MS was made according to space and time dissemination on MRI criteria. We proposed a treatment with Interferon Beta 1a (Avonex).

Adult↗
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

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

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

Vocal cord and diaphragm paralysis, as clinical features of a French family with autosomal recessive Charcot-Marie-Tooth disease, associated with a new mutation in the GDAP1 gene.

Axonal forms of Charot-Marie-Tooth disease, either dominantly or recessively inherited, are clinically and genetically heterogeneous. We describe the clinical and electrophysiological characteristics of an axonal autosomal recessive form of Charot-Marie-Tooth disease in a French family, associated with a new mutation of the ganglioside-induced differentiation-associated protein-1 gene (GDAP1). Two sisters, born to non-consanguineous parents, presented severe proximal and distal sensorimotor deficit, areflexia, pes cavus, scoliosis and vocal cord and diaphragm paralysis. They lost ambulation in the third decade and since then they have been wheelchair bound. Nerve conduction studies were consistent with an axonal neuropathy. Clinical and electrophysiological examination of their parents and their brother was normal. Genetic analysis revealed a homozygous thymidine deletion at nucleotide position 558 resulting in a frameshift at codon 186 and a stop codon at position 205. This axonal form of Charot-Marie-Tooth disease associated with a new GDAP1 mutation is recessively inherited and is characterized by a severe phenotype, since patients become wheelchair bound in the third decade, and present vocal cord and diaphram paralysis, which may be missed as they had no respiratory symptoms until the third decade.

Adult↗
PubMed2004

[BCcogSEP: a French test battery evaluating cognitive functions in multiple sclerosis].

In French language, there is no standardized procedure to assess cognitive function in patients with multiple sclerosis (MS). Such an assessment is however very useful to determine the consequences of the disease on cognitive function, to evaluate the disease progression and the consequences of usual treatments on cognition. This study aimed to develop and validate a French language battery based on "the Brief Repeatable Battery of Neuropsychological tests for Multiple Sclerosis" (BRB-N) often used in other countries. In an initial phase, the battery was composed of the French version of the 5 BRB-N tests to which were added 4 tests assessing immediate and working memory as well as executive function. 52 healthy control subjects (20-50 years) participated in the study. A principal component analysis (PCA) of their data examined the contribution of each test into the battery. 93 other healthy subjects participated in a second phase where analyses of variance were carried out to investigate the effect of the main demographical variables. Finally, the performance of two patient groups (20 with a EDSS score 3) was compared to the healthy controls in order to investigate the ability of our battery to detect cognitive impairment in MS patients. The PCA showed that each test, except one, had a specific contribution to the battery. The final battery (BCcogSEP) was thus comprised of 8 tests. MANOVA and ANOVA showed significant effects of age, sex and educational level on performance. In consequence, a procedure allowing to take into account these factors was developed. The battery was able to detect cognitive impairment in MS patients, even when the disease is not severe. In this case, deficits were observed in tests assessing executive function, information processing speed, immediate and working memory. This short battery with reliable psychometric qualities allows the interpretation of a MS patient's performance considering his/her demographical characteristics and is able to detect cognitive abnormalities even in case of mild physical handicap.

Cognition↗
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

[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

[Ataxic neuropathy associated with disialosylated antibodies: description of new clinical and biochemical forms].

INTRODUCTION: Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin associated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, GD3, GT1b, GT1a, GQ1b. METHODS: We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antibodies and for two of them cold agglutinins. Such features have been previously described under the acronym "CANOMAD" (chronic ataxic neuropathy with ophthalmoplegia, M protein, agglutination and disialosyl antibodies). RESULTS: One of the patients presents extramembranous glomerulopathy and severe motor disability associated with this syndrome. The pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous immunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus. CONCLUSION: Our study demonstrates that spectrum of polyneuropathy associated with monoclonal polyneuropathy may be larger than originally described.

Adrenal Cortex Hormones↗
PubMed2004

[Pure subacute pandysautonomia: an assessment of treatment with intravenous polyvalent immunoglobulins].

INTRODUCTION: Acute or sub-acute pure dysautonomia is uncommon. We report a case of sub-acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin therapy. CASE REPORT: A 29-year-old right-handed student, with an uneventful medical history presented, for one month, bilateral loss of visual acuity and digestive disorders, associating diarrhea, vomiting and anorexia. Physical examination revealed bilateral intrinsec oculomotor nerve palsy, a dryness syndrome and severe orthostatic hypotension. Ophthalmologic examination showed bilateral diffuse parasympathic impairment associating an Argyll Robertson pupil and full pupil light reflex abolition. Elevated protein level (0.93g/l) was the only cerebrospinal fluid anomaly. Serum tests were negative for anti-gangliosides antibodies. The patient improved slowly after two series of intravenous immunoglobulin infusions. CONCLUSION: Clinical course and laboratory findings suggest that acute or sub-acute pure pandysautonomia events are likely to be related to acute polyradiculoneuritis. Therefore intravenous polyvalent immunoglobulin infusions should be attempted, even if their efficacy needs to be confirmed.

Acute Disease↗
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

[Guillain-Barré syndrome with hallucinations and onirism: an underestimated association].

INTRODUCTION: Guillain-Barré Syndrome (GBS) is generally related to peripheral nervous system involvement, but certain variants with central nervous system manifestations have been described. CASE REPORT: In the present study we report 2 patients with GBS associated with hallucinations and onirism. Two men (age 64 and 49 years) presented GBS without proven infectious origin who required intensive care because of respiratory problems. The disease progressed and manifestations of encephalitis (hallucinations and onirism) appeared. The sensorimotor signs and encephalitis manifestations evolved in parallel with full recovery in the first patient and death after 11 months of intensive care in the second. CONCLUSION: GBS may be associated with stereotypic central nervous system symptoms, mimicking delirium tremens. The manifestations would be related to the severity of the initial period, but not to long-term prognosis.

Adult↗
PubMed2004

[Neurologic emergencies].

Neurological symptoms are a very frequent cause of consultation in emergency units and require consultation with neurologists and neuroradiologists. The most frequent diagnoses are stroke syndrome, seizure, headache, confusion, meningitis and meningo-encephalitis, and facial palsy. The morbidity and mortality of neurological emergencies are strongly related to prompt medical management of the patients which often requires neuroimaging studies. The most common neurological emergencies will be reviewed.

Coma↗
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↗
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↗