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Biomedical subjects

A Destée

Publications and source records attributed to A Destée.

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

ERG and anatomical abnormalities suggesting retinopathy in dementia with Lewy bodies.

The authors used flash electroretinography to demonstrate dysfunction of the photopic and scotopic retina in patients with dementia with Lewy bodies and visual hallucinations (VHs) compared with patients with Parkinson disease, patients without VHs, and controls. The retinal dysfunction may be related to slight alteration of the photoreceptors and numerous pale inclusions in the outer plexiform layer found at the post mortem examination, suggesting a specific retinopathy.

Aged↗
PubMed2005

Progressive MRI abnormalities in late recurrence of Sydenham's chorea.

We report four cases of adult recurrence of Sydenham's chorea (SC). The first episodes all followed childhood tonsillopharyngitis. Many years later, subsequent episodes appeared after the triggering circumstances: throat or cutaneous infection, pregnancy, childbirth, contraceptive treatment or stress. Other inflammatory diseases were ruled out and streptococcal serology was weakly positive. Magnetic Resonance Imaging (MRI) revealed focal areas of T2 hypersignal in the caudate nucleus, the pallidum, the putamen and the white matter. One year later, repeat MRI variously gave evidence of the persistence, disappearance or even new appearance of abnormalities. Specific features of SC recurrence in the adult may include: a personal history of chorea after a throat infection during childhood; a second episode of isolated chorea with or without slight neuropsychological disorders; streptococcal serology weakly positive; focal hypersignals involving the basal ganglia; the triggering circumstances. Since SC is considered to be an autoimmune disease, the progressive MRI abnormalities suggest that certain circumstances may trigger the reactivation of persistent immune disorders.

Adult↗
PubMed2005

[Parkinson's disease, progressive lumbar kyphosis and focal paraspinal myositis].

INTRODUCTION: The camptocormia (bent spine) is characterized by a severe forward flexion of the thoracolumbar spine which disappears in the supine position. Clinical case. We describe a typical case observed in a parkinsonian patient. The MRI, electromyogram and biopsy of the paraspinal muscles revealed a typical myositis pattern. DISCUSSION: This case, the sixth published to our knowledge, confirms that focal myositis is associated with the camptocormia in Parkinson's disease. Typically it is observed in male subjects, appearing 4 to 6 years after the onset of Parkinson's disease, in fluctuating patients treated by an association of L-Dopa and agonist. It appears quickly and becomes the most important symptom. Antiparkinsonian drugs are useless. CONCLUSION: This exceptional picture raises original pathophysiological and therapeutic questions. Systematic studies should be performed in order to detail the pathophysiological link between these 3 entities: Parkinson's disease, focal myositis and camptocormia.

Disease Progression↗
PubMed2005

[Management and treatment of respiratory failure associated with amyotrophic lateral sclerosis].

INTRODUCTION: In amyotrophic lateral sclerosis (ALS), respiratory muscle involvement is highly predictive of survival and quality of life (QOL). There is compelling evidence that non invasive ventilation (NIV) prolongs survival by several months and improves QOL more than any other currently available treatment. Frequent testing of pulmonary function and regular evaluations are recommended since 1999 by the American Academy of Neurology in order to take appropriate treatment decisions. STATE OF ART: There are numerous tests available to evaluate respiratory status in ALS and it is important to know their sensitivity and specificity to recognize clinical risk situations. Some recent data suggest that sniff nasal pressure and maximal inspiratory pressure (MIP) can be performed reliably by most ALS patients and are more sensitive to decrements in inspiratory muscle strength than spirometry or arterial blood gasometry. PERSPECTIVES: Airway obstruction caused by ineffective coughing is the principal cause of intolerance to NIV. Several factors other than respiratory muscle strength may affect pulmonary function: postural changes, nutritional status, infectious disease, drugs. CONCLUSION: The neurologist has to coordinate multidisciplinary care, with attention to individual patient preferences, and with a frank and compassionate discussion between the patient, the family, the physicians and the caregivers.

Amyotrophic Lateral Sclerosis↗
PubMed2005

[Can meningoencephalitis be caused by Chlamydiae pneumoniae?].

INTRODUCTION: Meningoencephalitis attributed to Chlamydiae pneumoniae has rarely been reported in the literature. OBSERVATION: We describe the case of a 21-year-old-man admitted for a meningeal syndrome and fever associated with respiratory symptoms and a temporal syndrome. Even though the clinical presentation and certain serological results suggested Chlamydiae infection, some of the biological data raised the possibility of Epstein Barr Virus infection. Intravenous fluoroquinolone therapy led to a successful outcome. CONCLUSION: Meningoencephalitis associated with non neurological symptoms, especially respiratory symptoms, is suggestive of Chlamydia pneumoniae infection. A broad spectrum antibiotic should be prescribed.

Adult↗
PubMed2005

[Pathophysiological mechanisms implicated by high-frequency stimulation in Parkinson's disease: the restoration of high and low frequency oscillatory systems].

INTRODUCTION: Increased neuronal activity in the internal pallidum (GPi) and the subthalamic nucleus (STN) has been clearly demonstrated in Parkinsonian models, and the two structures have thus been selected as therapeutic targets for functional neurosurgery. High-frequency electrical stimulation of the GPi or the STN improves the parkinsonian symptoms but also dyskinesias directly by GPi stimulation or indirectly by reduction of L-Dopa associated with STN stimulation. According to Alexander's model of the organisation of the basal ganglia, electrical stimulation of GPi or STN should have led to uncontrolled hyperkinesia. This apparent paradox could be explained on one hand by the involvement of different anatomo-functional areas within these structures and on the other by spatial and temporal changes in neuronal discharge patterns in the basal ganglia which in turn produce variations in synchronisation. RESULTS: Event-related (de)synchronisation (ERD) has enabled us to study variations in subcortico-cortical oscillatory activity: it has been shown that high-frequency electrical stimulation of the GPi/STN increases desynchronisation of low frequency rhythms (mu and beta,<30 Hz) during movement preparation and execution and augments post-movement synchronisation. Stimulation also decreases the abnormal frontocentral spreading of desynchronisation during movement preparation. CONCLUSIONS: In accordance with previous coherence analyses, electrical stimulation of STN is likely to restore the activity of high-frequency and low-frequency systems, as evidenced by a decrease in the hypersynchronisation of low-frequency rhythms at rest and restoral of a high-frequency rhythm during movement. Stimulation may improve spatial selectivity by activating the selected programs in conjunction with the primary sensorimotor cortex, whilst inhibiting competitive programs represented by abnormal spreading outside the primary sensorimotor cortex.

Basal Ganglia↗
PubMed2005

Disturbance of sensory filtering in dementia with Lewy bodies: comparison with Parkinson's disease dementia and Alzheimer's disease.

INTRODUCTION: Prepulse inhibition (PPI) is considered to mirror an organism's ability to filter out irrelevant sensory or cognitive information. The disruption of PPI has never been studied in individuals suffering from dementia with Lewy bodies (DLB). As attention deficits largely contribute to cognitive impairment in DLB, an investigation with a PPI paradigm is useful for differential diagnosis of DLB versus Alzheimer's disease (AD) and Parkinson's disease dementia (PDD). OBJECTIVE AND METHODS: PPI of the N1/P2 component of auditory evoked potentials was used to investigate the early stages of attention selectivity in 10 DLB, 10 AD, and 10 PDD patients, as well as in 10 healthy controls. The PPI paradigm consisted of the presentation of sound pulses (40 ms, 115 dB) preceded by a prepulse (40 ms, 80 dB). Sound stimuli were presented in a total of 80 trials in a pseudo-random order. RESULTS: Non-parametric analyses of variance revealed a significant group effect on the 120 ms lead interval. Retrospective analyses revealed that PPI was significantly reduced in DLB compared to healthy controls and AD. In the PDD group, the disturbance was of intermediate intensity. CONCLUSION: The present study revealed a severe disturbance of PPI in DLB patients. The DLB patients displayed a specific disruption profile in terms of magnitude as well as time course.

Acoustic Stimulation↗
PubMed2004

Assessment of Nurr1 nucleotide variations in familial Parkinson's disease.

Parkinson's disease (PD) is characterised by the death of dopaminergic neurons of the substantia nigra. As Nurr1 seems to regulate the development and maintenance of these neurons, we evaluated its potential role in Parkinson's disease using genetic methods. We genotyped two polymorphisms and screened a case-control sample for the presence/absence of two mutations recently described in exon 1. Our results failed to replicate the association initially observed and none of the mutations were present in our familial Parkinson's disease cases. These observations suggest that this gene is unlikely to play a major effect in French familial Parkinson disease.

Aged↗
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

Statistical tools for clinical gait analysis.

Gait analysis studies involve continuous curves of data measured over a gait cycle. Curve analysis and interpretation require adequate statistical methods. Three principal problems may be encountered in clinical practice: (i) the reliability of gait curves for a given patient, (ii) classifying a new subject as belonging to a given population or not and (iii) comparison of two populations (independent or paired). This paper presents three statistical tools for solving these problems: (i) intra-class correlation coefficients, (ii) confidence bands for a population (iii) a combination of analysis of variance and confidence bands for the difference between the means and shows how they can be used in clinical practice.

Adult↗
PubMed2004

Influence of aging on cortical activity associated with a visuo-motor task.

The aim of this study was to determine how cerebral aging influences the pattern of cortical oscillatory activity when a targeting movement with visual control is planned. Changes in cortical oscillatory activity were assessed by recording the event-related (de)synchronization (ERD/S) of micro and beta rhythms. Young and elderly subjects performed a distal movement, a proximal movement and a visuo-guided targeting movement. Our results demonstrated an increase in micro ERD over ipsilateral regions and showed the spatial extent of micro ERD over parietocentral and parietal regions during motor planning in elderly subjects compared to young ones. After the movement, the beta ERS was significantly modified (a decrease in slope and amplitude) in elderly subjects. The most pronounced age-related changes in ERD/S pattern were observed for the targeting movement. Our results suggest that motor planning is less efficient in elderly subjects. This deficit might result from impaired parietal integrative function and/or changes in inputs from subcortical structures. Subsequently, the changes observed in the post-movement phase might reflect a decrease in (reafferent) sensory inputs and hence impaired their input processing.

Adult↗
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

[Usefulness and limitations of polymygraphic recordings in dystonia].

INTRODUCTION: Diagnosis of dystonia is based on well-known clinical findings, but electrophysiological explorations can help to establish the diagnosis. METHODS: We report 179 patients presenting primary or secondary dystonia. Polymyographic recordings were available for all. We studied activation patterns of agonist and antagonists muscles (prolonged bursts, co-contraction), presence of antagonistic gestures and other associated movement disorders such as tremor or myoclonus. RESULTS: 83.9 percent of patients presented abnormalities which evoked dystonia (prolonged bursts: 75.3 percent, co-contraction: 48.7 percent, successful antagonistic gesture successful with reduction or suppression of EMG activity, 8.7 percent). For the patients who presented no EMG abnormality, diagnosis was maintained in 22 cases because of the limitations of the exploration: intermittent symptoms, dystonia involving deep muscles which were not recorded by the surface electromyogram, or difficulties to reproduce the conditions of apparition of the dystonic symptoms during examination or to recognize a pathologic EMG pattern in hand cramps for instance. Another diagnosis was retained because of the polymyographic findings in 16.2 p.cent of these patients, particularly those who had tremor associated with dystonia (essentially diagnosis of primary writing tremor). CONCLUSION: Polymyography is an effective tool for confirming clinical findings.

Adult↗
PubMed2004

[The presentation of small-cell lung cancer with metastatic carcinomatous encephalitis in a non-Hodgkin's lymphoma patient].

BACKGROUND: Carcinomatous encephalitis or milary cerebral metastases characterized by signs of diffuse encephalopathy is a rare form of brain metastases. Tiny tumor nodules are seen throughout the cortical and subcortical gray matter. OBSERVATION: We report the case of a patient with a history of non-Hodgkin lymphoma who developed carcinomatous encephalitis probably secondary to small-cell lung cancer. This case is discussed in light of findings of 16 cases of carcinomatous encephalitis reported in the literature. We discuss clinical, radiological, histological, pathophysiological characteristics and the survival of this form. CONCLUSION: The frequency of carcinomatous encephalitis is underestimated because clinical expression is non specific. Brain magnetic resonance imaging must be performed in all patients presenting encephalopathy without an obvious cause.

Aged↗
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

[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

[Flaccid tetraplegia following anti-tetanus vaccination].

INTRODUCTION: Anti-tetanus vaccination is considered to be very safe. However complications such as mononevritis, multinevritis or even polyradiculonevritis can be encountered. OBSERVATION: An 85-year-old man was admitted to our unit after a traffic accident caused by stroke. Seventy-two hours later the patient developed tetraplegia within a few hours, caused by neuropathy secondary to anti-tetanus vaccination. DISCUSSION: In this elderly patient, the rapid onset and severe presentation of the tetraplegia as well as the past history of normal vaccination and the axonal form of the neuropathy could have led to a misdiagnosis of neuropathy after booster anti-tetanus vaccination. We first ruled out other possible diagnoses and then reviewed the possible mechanisms of neurological complications of vaccinations. These complications are probably underestimated in elderly hospitalized patients who receive a booster shot in the emergency department. CONCLUSION: This report illustrates the importance of reconsidering the benefit/risk relationship of anti-tetanos vaccination.

Accidents, Traffic↗