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F Dubas

Publications and source records attributed to F Dubas.

At least 19 recordsLinked to original sources
PubMed2006

[Idiopathic acute transverse myelitis: application of new diagnosis criteria to 17 patients].

INTRODUCTION: Idiopathic Acute Transverse Myelitis (ATM) is an inflammatory and immune-mediated disorder, distinct from infectious ATM, ATM of systemic lupus erythematosus or Sjögren's syndrome, and medullary manifestation of multiple sclerosis. Prognosis is not well-known. OBJECTIVE: To evaluate clinical, paraclinical and pronognosis data in patients selected with new diagnosis criteria, classically described in idiopathic ATM. METHODS: Seventeen patients with diagnosis criteria were retrospectively (1996-2005) studied. A telephone investigation was conducted in 2005 to obtained data on the clinical course. RESULTS: Seven men and 10 women, ranging in age from 15 to 75 years (mean: 39.8 years) met these new criteria. Our study showed that epidemiological and clinical findings as well as laboratory results were in agreement with those presented in the literature. Conversely, prognosis was better since 76p.cent of the patients could walk without assistance. The clinical presentation of some of our patients and/or their progression towards other multifocal inflammatory disorders, suggests there might be links between ATM, neuromyelitis optica (NMO) and Acute Dissemined Encephalomyelitis (ADEM). CONCLUSION: Patients with idiopathic ATM, selected with new criteria, have a rather good prognosis. ATM seems to be part of a continuum of neuroimmunologic disorders including NMO or ADEM although reasons explaining distinct focal disorders remain unclear.

Adolescent↗
PubMed2005

Psychiatric side effects of interferon-beta in multiple sclerosis.

Psychiatric disorders, especially depression, are frequent in patients with multiple sclerosis (MS). They are attributed both to the psychosocial impact of a chronic, usually progressive, disabling illness and to cerebral demyelination. Besides, drugs such as corticosteroids and possibly interferon (IFN) may also have depressogenic effects. Major depressive disorders and/or suicidal ideation are a major concern and efforts to identify and minimize these reactions are of much importance. Psychiatric side effects, particularly depression, are widely reported with IFN-alpha and have been suspected with IFN-beta but are not yet fully established. Our review of the literature revealed that most studies discard an association between IFN-beta and depression or suicide. However, few patients, especially those with a history of depression, might be at higher risk for depression when treated with IFN-beta. Overall, considering the uncertainty of a link between IFN-beta and depression and/or suicide, as well as the complete remission of psychiatric complications after IFN discontinuation and/or antidepressant treatment, physicians should closely monitor the psychiatric status of patients, but should not refrain from including them in IFN-beta treatment programs, even when they have past or present depression.

Adjuvants, Immunologic↗
PubMed2005

Idiopathic acute transverse myelitis: application of the recent diagnostic criteria.

Despite an extensive diagnostic workup, some cases of acute transverse myelitis (ATM) remain of unknown etiology and have been referred to as "idiopathic" by the Transverse Myelitis Consortium group. In a retrospective study of 288 patients with ATM, 45 cases (15.6%) met the criteria for idiopathic ATM. The patients formed a relatively homogeneous group in terms of clinical and MRI data, but the prognosis was highly variable.

Adrenal Cortex Hormones↗
PubMed2005

[Evaluation of the attitude towards disabled persons of 3rd and 4th year medical students using the "Attitude towards disabled persons" questionnaire. Effect of courses and rotations in physical medicine and rehabilitation departments].

UNLABELLED: Modifications of the medical curriculum have included a compulsory course on disability. OBJECTIVE: To determine whether attendance in a course on disability and/or rotations in physical medicine and rehabilitation departments modify the attitude of medical students towards disabled people. METHODS: All third- and fourth-year students completed a translated version of the ATDPb. This questionnaire rates items evaluating attitude towards disabled people on a 6-point scale (minimum 0; maximum 180). Retro-translation was performed to control the translation. During the second year, all students had attended a general course in ethics. Fourth-year students had attended a 17 hours course on disability, and 21 of 78 had spent 9 weeks in the physical medicine and rehabilitation department. The study compares fourth-year students to third-year students, considered as controls, and students having spent a rotation in the physical medicine and rehabilitation department to others. RESULTS: The mean score of all students was 108.86+/-15.84 (73-160) on the ATDP scale. Males and females did not differ significantly, and the score did not change from that before the course on disability (109.95+/-14.98 vs 107.6+/-16.65, P=0.23) nor after a rotation in the physical medicine and rehabilitation department (113.52+/-11.42 vs 108.54+/-16.03, P=0.14). CONCLUSION: Development and validation of scores that would fit better to the European cultural context would be useful. The present method of theoretical courses and rotations do not improve the attitude of students towards disabled people and should be modified if this objective is to be achieved.

Attitude to Health↗
PubMed2005

[Lesion mechanism dependent, differential changes in neurofilaments and microtubules: a pathological and experimental study].

INTRODUCTION: The consequences of axonal or demyelinating injuries on the axonal cytoskeleton have rarely been described. METHODS: We have compared the density of fibers labeled by anti-neurofilaments (NF) and -beta tubulin (TUB) to the density of total fibers in nine patients with axonal neuropathies of undetermined etiology (AUE), six with necrotizing angeitis with neuropathy (NAN), seven with chronic inflammatory demyelinating neuropathy (CIDP) and in five controls, as well as in six patients with chronic multiple sclerosis (MS). We also studied demyelinated rat corpus callosum after lysophosphatidyl (LPC) microinjection. RESULTS: In AUE and NAN NF positive fibers decreased together with total fiber density, whereas TUB increased. In demyelinating lesions TUB was not altered (CIDP) or strongly decreased (MS, LPC); NF were strongly reduced in MS (where axon loss was prominent) and in LPC lesions (despite the lack of fiber degeneration) and for fiber densities<3900/mm2 in CIDP. CONCLUSION: The initial mechanism of a disease, either axonal degeneration or demyelination, could result into a specific pattern of axonal cytoskeleton alterations.

Aged↗
PubMed2005

[An OPA3 gene mutation is responsible for the disease associating optic atrophy and cataract with extrapyramidal signs].

INTRODUCTION: In 1961, Garcin et al. described a family with several members affected with optic atrophy associated with cataract, and neurological symptoms. The authors believed this condition to be distinct from other diseases known at that time, e.g. the Behr syndrome, Marinesco-Sjogren syndrome and Friedreich's ataxia. METHOD: This family was followed over a period of 40 years and genes known to be responsible for optic atrophy were sequenced. RESULTS: The G277A mutation of OPA3 gene was responsible for this familial disease. DISCUSSION: A new clinical entity is identified: autosomal dominant optic atrophy and cataract, due to a heterozygous mutation of the OPA3 gene, a nuclear gene encoding a mitochondrial protein.

Adult↗
PubMed2004

Coincidence of two genetic forms of Charcot-Marie-Tooth disease in a single family.

The authors report a family in which two affected first cousins had a severe demyelinating Charcot-Marie-Tooth disease (CMT) phenotype. One had related parents, and there were no other affected relatives, suggesting an autosomal recessive mode of inheritance. Molecular studies showed that a de novo duplication in 17p11.2 and a second mutation in MTMR2 were present.

Adolescent↗
PubMed2004

[Pulsed transcranial Doppler examination in the acute phase of stroke].

INTRODUCTION: Transcranial pulsed Doppler sonography with or without imagery is used in the acute phase of ischemic stroke in order to define stroke mechanism and intracranial hemodynamic consequences. STATE OF ART: This non-invasive low-cost investigation can be undertaken rapidly, within 10 minutes if guided by focal symptomatology. It detects middle cerebral artery stenosis in >or=50 p. cent of cases with a sensitivity between 75 and 90 p. cent, comparatively with conventional angiography, and middle cerebral artery occlusion with 90 p. cent sensitivity. When a temporal acoustic window is lacking, intravenous injection of a contrast agent allows good visualization of the intracranial vessels and the circle of Willis, in two-third of cases. Moreover, transcranial Doppler data have good prognostic value and facilitate analysis of recanalization after thrombolytic therapy. CONCLUSION: Beside its diagnostic and prognostic interest, transcranial Doppler sonography using a 2 MHz frequency appears to have therapeutic impact by favoring the thrombolysis process. Future developments may include early and prolonged insonation of patients suffering from stroke.

Brain Ischemia↗
PubMed2004

[Processing of "scripts" and frontal lobe function in Huntington's disease].

According to Norman and Shallice, the basal ganglia are involved in the activation and maintenance of overlearned or routine scripts in action planning. This study aimed to investigate how patients with Huntington disease manipulate scripts. Ten patients with Huntington's disease and 12 normal control subjects matched by age and education were asked to re-establish the sequential order of series of script actions which were given with or without distractor elements (i.e. with or without actions belonging to trivial scripts). Compared with normal controls, patients with Huntington disease made significantly more errors in script sequencing. However there was no significant difference in performance between patients with Huntington disease and normal control subjects in inhibiting irrelevant actions. These results suggest that early Huntington's disease patients exhibit early impairment in their ability to produce temporally coherent sequences without deficit in their ability to eliminate distractors in the action domain. These results support in part the predictions of Norman and Shallice's model. From a neuroanatomical point of view, this dissociation of performance fits with what is known about the neuropathological progression of Huntington's disease in which neuronal loss progresses in a dorsal-to-ventral direction and with what was shown in patients with circumscribed frontal lobe damage. In these patients, impairment in script sequencing is related to lesions in the lateral prefrontal regions.

Adult↗
PubMed2004

[Malignant non-Hodgkin lymphoma presenting with Garcin's syndrome].

INTRODUCTION: R Garcin described progressive unilateral cranial nerve palsy in 1926. Garcin syndrome is characterized by progressive involvement of the cranial nerves culminating in total unilateral paralysis of all cranial nerves. Carcinoma of the skull base or ENT regions is the most common etiology. CASE REPORT: A 74-year-old man developed signs involving the left Vth (V2 and V3) cranial nerve then the VIth, VIIth and VIIIth cranial nerves and finally the IXth and Xth. MRI showed involvement of these cranial nerves with gadolinium uptake and involvement of the pons at the terminal phase. Careful ENT explorations failed to reveal a cause. The lymphocyte count was elevated in the cerebrospinal fluid. The patient died one year after diagnosis and the general autopsy was normal. The neuropathological studies led to the post-mortem diagnosis of type B non-Hodgkin lymphoma. CONCLUSION: In patients with Garcin syndrome, lymphoma is a possible diagnosis when carcinoma of the ENT regions or of the skull bases are not present.

Aged↗
PubMed2003

Stable tubule only polypeptides (STOP) proteins co-aggregate with spheroid neurofilaments in amyotrophic lateral sclerosis.

A major cytopathological hallmark of amyotrophic lateral sclerosis (ALS) is the presence of axonal spheroids containing abnormally accumulated neurofilaments. The mechanism of their formation, their contribution to the disease, and the possibility of other co-aggregated components are still enigmatic. Here we analyze the composition of such lesions with special reference to stable tubule only polypeptide (STOP), a protein responsible for microtubule cold stabilization. In normal human brain and spinal cord, the distribution of STOP proteins is uniform between the cytoplasm and neurites of neurons. However, all the neurofilament-rich spheroids present in the tissues of affected patients are intensely labeled with 3 different anti-STOP antibodies. Moreover, when neurofilaments and microtubules are isolated from spinal cord and brain, STOP proteins are systematically co-purified with neurofilaments. By SDS-PAGE analysis, no alteration of the migration profile of STOP proteins is observed in pathological samples. Other microtubular proteins, like tubulin or kinesin, are inconstantly present in spheroids, suggesting that a microtubule destabilizing process may be involved in the pathogenesis of ALS. These results indicate that the selective co-aggregation of neurofilament and STOP proteins represent a new cytopathological marker for spheroids.

Adult↗
PubMed2003

Two clinicopathological cases of a dominantly inherited, adult onset orthochromatic leucodystrophy.

Leucodystrophies of orthochromatic type are a heterogeneous group that occur mainly in childhood and have no known enzyme deficiency. We report here the clinicopathological features of a new family of orthochromatic leucodystrophy with three main characteristics: a probably autosomal dominant inheritance; two phenotypes based on age of onset; and very few abnormalities of white matter on MRI findings in one case. The first patient, aged 58 years, had frontal dementia and epilepsy; the second, aged 38 years, had motor signs and dementia, but no epilepsy. The histopathological features of our two cases were leucodystrophy of orthochromatic subtype. However, the radiological features (MRI and mostly FLAIR sequences) of the first case did not suggest leucodystrophy.

Adult↗
PubMed2003

[Necrotizing vasculitis of the peripheral nervous system: comparison of the axon cytoskeleton abnormalities with other types of neuropathies].

Morphometric and immunocytochemical data obtained from nerve biopsies in six patients with necrotizing angiopathic neuropathy were compared with the data obtained in five control patients without neurologic disease. The density of myelinated fibers were greatly decreased in all patients (more than 55 p.cent), and reached 70 p.cent in four patients. The g myelination ratio was elevated in one patient, indicating demyelination. The number of fibers immunolabeled for the neurofilament (NF) light chain (L) was also decreased from 31 to 96 p.cent. Labeling for medium (M) and heavy (H) NF subunits was less affected. On the contrary, the number of fibers expressing beta-tubulin (TUB) increased by 38 to 141 p.cent and the ratio of the number of fibers expressing TUB and NFL (TUB/NFL) was increased 3 to 33-fold (p<0.05). We have previously reported similar, though less pronounced, anomalies in axonopathies of unknown cause. These results suggest that diverse etiologies may lead to the same type of lesions of the axon cytoskeleton.

Aged↗
PubMed2003

[Small vessel pathology and cerebral hemorrhage].

Spontaneous intracerebral hemorrhage may be due to two main arteriolopathies: HTA and age-related small vessel disease and cerebral amyloid angiopathy. Historical study of the CHARCOT-BOUCHARD microaneurysm controversy leads to conclude that altered arterioles may be ruptured with or, probably more often, without microaneurysms. Recent neuroradiological studies with gradient-echo T2*W MR imaging have confirmed a classical neuropathological fact: there is a strong correlation between subcortical ischemic lesions (lacune, leukoaraiosis) and subcortical hemorrhage (hematomas, microbleeds). Sporadic Cerebral Amyloid Angiopathy is the first cause of spontaneous intracerebral hemorrhage after 70 years. Hemorrhages are lobar, typically recurrent and/or associated with cortical microbleeds at T2*W imaging.

Arterioles↗
PubMed2001

Accuracy of color-Doppler in the quantification of proximal vertebral artery stenoses.

BACKGROUND: Vertebrobasilar (VB) strokes appear to have the same causes as carotid strokes. Obstructive lesions of proximal vertebral arteries probably occur in about 30% of stroke patients. PURPOSE: Our aim was to assess the validity of color Doppler sonography compared to selective intra-arterial angiography in the quantification of proximal vertebral artery stenoses. MATERIALS AND METHODS: A prospective blind study of 316 vertebral arteries was undertaken between 1996 and 1998. One hundred and fifty-eight patients with cerebrovascular disorders without cerebral hemorrhage were studied consecutively by frequency or amplitude color Doppler flow imaging and intra-arterial angiography. The lesions were quantified by morphological and hemodynamic criteria and classified into 6 groups: 0% 207 arteries; 1-29% 32 arteries; 30-49% 29 arteries; 50-69% 13 arteries; 70-99% 23 arteries; 100% 12 arteries. RESULTS: Ten of the 12 occlusions were identified, the 2 false-negatives were due to 2 revascularized vessels. Moderate stenoses (<50%) were differentiated from tight stenoses (>50%) using hemodynamic criteria. The majority of false-negative stenoses (38) in the different groups were related to intrathoracic or very deep origin of the artery, anechogenic stenosis or a tortuous vessel. Stenoses greater than 70% were diagnosed in 71% of cases with a specificity of 99%. The kappa value was 0.80. CONCLUSION: Duplex sonography should be proposed first in VB attacks or stroke to detect and quantify vertebral artery stenoses for surgery and angioplasty.

Arterial Occlusive Diseases↗
PubMed2001

[Trismus, pseudobulbar syndrome and cerebral deep venous thrombosis].

We report a case of cerebral deep venous thrombosis that manifested clinically by a pseudobulbar syndrome with major trismus, abnormal movements and static cerebellar syndrome. To our knowledge, only three other cases of deep cerebral venous thrombosis associated with cerebellar or pseudobulbar syndrome have been published since 1985. The relatively good prognosis in our patient could be explained by the partially intact internal cerebral veins as well as use of early anticoagulant therapy. There was a spontaneous hyperdensity of the falx cerebri and the tentorium cerebelli on the brain CT scan, an aspect highly contributive to diagnosis. This hyperdensity of the falx cerebri was found in 19 out of 22 cases of deep venous thrombosis detailed in the literature.

Cerebral Angiography↗
PubMed2001

[Multiple sclerosis and Leber's hereditary optic neuropathy mitochondrial DNA mutations].

Multiple sclerosis (MS) has long been known to be associated with Leber's hereditary optic neuropathy (LHON), a disease caused by mitochondrial DNA (mtDNA) mutations. We have investigated the possible involvement of LHON mtDNA point mutations in MS. The study covered a group of 75 unrelated Caucasian patients, with the relapse-remitting or primary progressive form of MS, and a control group of 75 volunteers (matched for age, gender and ethnic origin). Mitochondrial DNA from each subject was examined for 4 primary LHON mutations (at nucleotide positions 3460, 4160, 11778 and 14484) and 7 secondary LHON mutations (at nucleotide positions 4216, 4917, 5244, 7444, 13708, 15257 and 15812) by means of restriction site polymorphism and sequencing techniques. None of the primary LHON mutations were detected in the MS patients or in the controls, whereas the proportion of individuals with secondary LHON mutations was identical (27 p. cent) in the two groups. A combination of 2 or 3 homoplasmic mutations, defining mtDNA haplogroups, was found in the majority of cases. Haplogroups J, T and X were not particularly associated with MS. The frequency of the 13708 mutation alone (haplogroup X), or associated with the 4216 mutation (haplogroup J), was somewhat higher (p=0.059) in the subgroup of MS patients with optic neuritis (ON). ON was the initial symptom in all but one of the patients with haplogroups J or X. No other correlation was found between MS phenotypes and mtDNA genotypes. Our observations confirm previous reports that neither primary nor secondary LHON mutations are involved in the development of MS. However, MS patients with haplogroups J or X appear to have a moderately higher risk of developing optic neuritis. Thus, a specific mtDNA background may be a predisposing genetic factor for optic nerve damage in MS patients.

Adult↗