Biomedical subjects

A Destée

Publications and source records attributed to A Destée.

At least 19 recordsLinked to original sources

PubMed2006

High erythropoietin and low vascular endothelial growth factor levels in cerebrospinal fluid from hypoxemic ALS patients suggest an abnormal response to hypoxia.

Animal studies have highlighted the potentially neuroprotective role of vascular endothelial growth factor (VEGF). Low levels of this growth factor have been found in the cerebrospinal fluid (CSF) of patients with amyotrophic lateral sclerosis (ALS). VEGF (and other proteins, such as erythropoietin (EPO)) are produced in response to hypoxia via a common pathway involving a specific transcription factor (hypoxia-inducible factor, HIF) and a hypoxia responsive element (HRE) in the respective genes' promoter regions. In this study, we report finding the expected, high levels of VEGF and EPO in CSF from hypoxemic neurological controls, whereas EPO (but not VEGF) levels are high in the CSF from hypoxemic ALS patients. Hence, the VEGF levels in CSF from patients with ALS were significantly lower than those seen in hypoxemic controls. There was a trend towards higher CSF levels of EPO in hypoxemic ALS patients than in hypoxemic controls. Our results suggest that VEGF may not be produced in sufficient amounts in chronically hypoxic ALS patients and that this dysfunction may participate in the pathogenesis of the disease. The high EPO levels in hypoxemic ALS patients nevertheless suggest an intact common oxygen-sensor pathway.

Adult↗

PubMed2006

Improvement of gait by chronic, high doses of methylphenidate in patients with advanced Parkinson's disease.

BACKGROUND: Therapeutic management of gait disorders in patients with advanced Parkinson's disease (PD) can sometimes be disappointing, since dopaminergic drug treatments and subthalamic nucleus (STN) stimulation are more effective for limb-related parkinsonian signs than for gait disorders. Gait disorders could also be partly related to norepinephrine system impairment, and the pharmacological modulation of both dopamine and norepinephrine pathways could potentially improve the symptomatology. AIM: To assess the clinical value of chronic, high doses of methylphenidate (MPD) in patients with PD having gait disorders, despite their use of optimal dopaminergic doses and STN stimulation parameters. METHODS: Efficacy was blindly assessed on video for 17 patients in the absence of L-dopa and again after acute administration of the drug, both before and after a 3-month course of MPD, using a Stand-Walk-Sit (SWS) Test, the Tinetti Scale, the Unified Parkinson's Disease Rating Scale (UPDRS) part III score and the Dyskinesia Rating Scale. RESULTS: An improvement was observed in the number of steps and time in the SWS Test, the number of freezing episodes, the Tinetti Scale score and the UPDRS part III score in the absence of L-dopa after 3 months of taking MPD. The L-dopa-induced improvement in these various scores was also stronger after the 3-month course of MPD than before. The Epworth Sleepiness Scale score fell dramatically in all patients. No significant induction of adverse effects was found. INTERPRETATION: Chronic, high doses of MPD improved gait and motor symptoms in the absence of L-dopa and increased the intensity of response of these symptoms to L-dopa in a population with advanced PD.

Aged↗

PubMed2006

Movement-related cortical activation in familial Parkinson disease.

We sought to determine whether or not first-degree relatives of patients with familial Parkinson disease (FDRs) present impaired movement-related cortical activity. We studied 10 familial Parkinson disease subjects, 10 FDRs, and 10 controls and analyzed event-related mu desynchronization (ERD) and beta synchronization. Forty percent FDRs presented reduced premovement mu ERD latency, suggesting that premovement cortical activation is impaired in FDRs.

Aged↗

PubMed2006

Predominance of the contralateral movement-related activity in the subthalamo-cortical loop.

OBJECTIVE: Abnormal low- and high-frequency oscillatory activities have been linked to abnormal movement control in Parkinson's disease. We aimed to study how low- and high-frequency oscillatory activities are modulated by movement in the contralateral and ipsilateral subcorticocortical loops. METHODS: We studied mu, beta and gamma rhythm event-related desynchronisation (ERD) and synchronisation (ERS) recorded from electrode contacts in the subthalamic nucleus (STN) areas and over the primary sensorimotor (PSM) cortex. RESULTS: Mu and beta ERD/ERS patterns were very similar when comparing PSM cortex and STN areas and very different when comparing contralateral and ipsilateral structures. Beta rhythm ERS was more predominant over contralateral structures than over ipsilateral ones. Gamma rhythm ERS was only recorded from the contralateral STN area (particularly following administration of L-Dopa). For all patients, the best bipolar derivations - as defined by the earliest mu and beta ERD and the strongest beta and gamma ERS - always included the STN electrode contacts that produced the best clinical results. CONCLUSIONS: Movement-related activity is involved in the movement preparation in the contralateral subthalamo-cortical loop and in the movement execution in the bilateral subthalamo-cortical loops. SIGNIFICANCE: Contralateral beta rhythm ERD seemed to be related to bradykinesia of the limb performing the movement.

Cerebral Cortex↗

PubMed2006

A biomechanical study of gait initiation in Huntington's disease.

BACKGROUND: Akinesia in basal ganglia disorders is essentially defined by delayed movement initiation; the reaction time increases and it becomes difficult (or even impossible) for the subject to initiate movement. A biomechanical study of gait initiation would help evaluate the role of akinesia in early stage Huntington's disease (HD) patients. METHODS: We recorded kinematic, spatiotemporal and angular parameters (using video motion analysis, a force platform and an optoelectronic system) for the first two steps taken by 15 HD patients and 15 gender- and age-matched controls. In order to evaluate the influence of an external cue on gait initiation parameters, we studied two movement paradigms: self-triggered initiation and initiation triggered (cued) by a "beep" sound. We analyzed kinematic, spatiotemporal (the speed, length and duration of the two first steps) and angular parameters (range of joint angles) as well as kinetic data (the trajectory of the centre of pressure (COP); the speed and trajectory of the centre of mass (COM)). RESULTS: HD patients presented akinesia in both externally triggered and self-triggered conditions. Patients had more difficulties with self-triggered gait than with triggered gait. In HD, anticipatory postural adjustments (APAs) were more impaired in self-triggered gait initiation than in cued initiation. Indeed, an alteration in the kinetic parameters revealed a reduction in first step speed in both conditions. Hypokinesia (as assessed by a reduction in the range of angle joints) played an important role in this reduction. CONCLUSION: Akinesia is a major feature of impaired gait initiation in HD. The deficiencies in self-triggered initiation in HD seen here fit with a hypothesis whereby deficient internal cueing can be replaced by an external trigger.

Biomechanical Phenomena↗

PubMed2006

Vigilance troubles in Parkinson's disease: a subjective and objective polysomnographic study.

BACKGROUND AND PURPOSE: To assess the prevalence of vigilance disorders in Parkinson's disease patients, relate the observed phenomena to potential causes and confirm these troubles with polysomnographic analysis. PATIENTS AND METHODS: A questionnaire was used to gather information on demographic data, previous and current treatments, disease characteristics, sleep and vigilance troubles. Somnolence was measured using the Epworth Sleepiness Scale (ESS). Nocturnal polysomnography (PSG) and multiple sleep latency tests (MSLT) were performed for a sample of parkinsonian patients. RESULTS: Two hundred twenty-two parkinsonian patients completed the questionnaire, and 36 patients had objective analyses. Of the patients, 43.2% had an ESS score >10, and 28.4% reported somnolence in the hour after taking dopaminergic drugs, whereas 6.8% reported unintended sleep episodes. In view of questionnaire data, these vigilance disorders may be partly explained not only by the impact of nocturnal sleep disorders (e.g. sleep apnea syndromes) but also by dopaminergic therapy (especially with dopaminergic agonists). With PSG and MSLT results, we have shown a significant correlation between mean sleep latency and ESS score. Patients with unintended sleep episodes have severe sleepiness in MSLT compared with others patients. CONCLUSIONS: Vigilance disorders are frequently observed in Parkinson's disease. We recommend informing patients of the risk of occurrence of such conditions, notably for patients with unintended sleep episodes and with sleepiness in the hour after taking dopaminergic drugs.

Aged↗

PubMed2006

A specific clinical pattern of camptocormia in Parkinson's disease.

BACKGROUND: Camptocormia, characterised by extreme forward flexion of the thoracolumbar spine and severe stooping in the supine position, seems to be prevalent in Parkinson's disease. OBJECTIVE: The aim of this study was to identify features of parkinsonian camptocormia and to describe the main clinical characteristics of patients with Parkinson's disease who develop the condition. METHODS: An extensive range of clinical, biochemical and imaging data were gathered for 23 patients with Parkinson's disease with camptocormia, notably including magnetic resonance imaging (MRI) of the brain and spine, electromyographic recordings of the paravertebral muscles and muscle biopsies. RESULTS: Camptocormia occurred in severe Parkinson's disease with axial predominance, motor fluctuations and dysautonomic symptoms. The condition was often associated with spondyloarthritic changes and pain. MRI showed paraspinal muscle signal abnormalities in five patients and fatty involution in seven patients. The seven patients had motor unit reductions on the spinal erector electromyogram. The MRI results for the girdle muscles were normal. Cranial MRI showed signal abnormalities for the basal ganglia in three patients. DISCUSSION: Various mechanisms may contribute to the development of parkinsonian camptocormia: dopaminergic depletion in Parkinson's disease induces functional changes in the organisation of the corticospinal and reticulospinal tracts, where dysfunction could contribute to axial rigidity. Furthermore, rigidity of the spinal flexion muscles could lead to under-use of the spinal extension muscles, which become progressively atrophic. Rigidity may also induce spinal deformations, leading to a neurogenic syndrome via compression of the spinal nerves. CONCLUSION: The screening and early management of camptocormia in Parkinson's disease is likely to be important for preventing axial disorders and spinal deformations.

Aged↗

PubMed2006

Preservation of cognitive function in dystonia treated by pallidal stimulation.

OBJECTIVE: To assess the effects of bilateral pallidal deep brain stimulation (DBS) on mood and cognitive performance in patients with dystonia before surgery (at baseline, while patients received their usual treatment) and 12 months postoperatively (while patients received neurostimulation and their medications) in a multicenter prospective study. METHODS: Twenty-two patients with primary generalized dystonia were evaluated with tests focused on executive functions. The authors considered the patients' severe disability and selected the following tests: Raven Progressive Matrices 38, Similarities and Arithmetic subtests of the Wechsler Adult Intelligence Scale-R, Grober and Buschke, Wisconsin Card Sorting Test (WCST), verbal fluency, Trail Making Test, and the Beck Depression Inventory. Median age at surgery was 30 years (range = 14 to 54 years), median duration of disease was 18.5 years (range = 4 to 37 years). RESULTS: Before surgery, no patients showed cognitive decline or depression. The surgical procedure appeared to be benign cognitively. One year after surgery, free recall improved. There was a significant reduction in the number of errors in the WCST. No behavioral or mood changes were found. CONCLUSIONS: Bilateral pallidal stimulation has a good benefit-to-risk ratio as it did not negatively affect cognitive performance and mood in primary dystonia, while a significant motor improvement was obtained. Moreover, a significant mild improvement in executive functions was observed, which may have been related either to the surgical treatment or to the marked decrease in anticholinergic drugs.

Adolescent↗

PubMed2006

Stimulation of the subthalamic nucleus in Parkinson's disease: cognitive and affective changes are not linked to the motor outcome.

BACKGROUND: Subthalamic nucleus deep brain stimulation (STN0 DBS) is a widely performed surgical treatment in PD. However, the relationship between motor results and cognitive/behavioural modifications is unclear. OBJECTIVE: This study investigated the correlation patterns of the motor, cognitive and behavioural consequences of STN DBS with respect to positioning of the active contact. METHODS: Fifty-eight consecutive PD patients having undergone STN DBS were assessed pre-operatively and 12 months after surgery. RESULTS: Motor, cognitive and behavioural results were neither correlated to each other nor linked to the position of the active contact. Three patients with a history of pre-operative, dopaminergic psychosis or post-surgical confusion became demented. Age and a distant history of depression were associated with the occurrence of post-surgical depression. CONCLUSION: Correct screening of patients for STN DBS remains an important issue, since the current implantation procedure is not able to take account of potential functional heterogeneity within the target.

Affect↗

PubMed2006

Gait abnormalities induced by acquired bilateral pallidal lesions: a motion analysis study.

BACKGROUND: Bilateral pallidal lesions induce a range of cognitive and motor disorders, principally a parkinsonian syndrome in which severe disturbances of gait and gait initiation are frequently reported. However, the precise clinical features of these disorders (and the role of the pallidum therein) remain to be established. OBJECTIVES: The goal of this study was to characterise gait and gait initiation disorders within the context of a parkinsonian syndrome in patients with acquired, bilateral, pallidal lesions (PAL patients), to compare these disorders to those seen in Parkinson's disease (PD), and to assess the corresponding physiopathological implications. PATIENTS AND METHODS: By using a video motion analysis system (VICON), we studied gait kinematic parameters in two patients presenting with bilateral, pallidal lesions. Kinematic and kinetic parameters were also determined during gait initiation. The two patients were compared with a group of 17 PD patients and to 20 healthy controls. RESULTS: In both PAL and PD patients, kinematic parameters (gait and gait initiation) and kinetic parameters (gait initiation) were similarly impaired, evidenced by akinesia (difficulty in initiating gait characterized by impairment of anticipatory postural adjustments). Hypokinesia and bradykinesia (respectively reduced stride length and reduced speed during gait) were also noted. CONCLUSION: The gait and gait initiation disorders seen in cases of bilateral pallidal lesions (namely akinesia, hypokinesia and bradykinesia) are similar to those observed in PD. Subject to confirmation in more extensive studies, we hypothesize that bipallidal patients may present higher level gait disorders,with potential mediation by cognitive impairment.

Aged↗

PubMed2006

[Differences in anticipatory postural adjustments between self-generated and triggered gait initiation in 20 healthy subjects].

OBJECTIVE: Preparation of upper-limb movements differs between self-paced and triggered conditions. This study analyzed the anticipatory postural adjustments (APAs) of gait initiation in normal subjects in 2 conditions: self-generated and triggered by a "beep" sound. METHODS: We recorded kinematic, spatiotemporal parameters of the first two steps by means of video motion analysis (6 infrared cameras), and kinetic parameters (using a force platform and the optoelectronic system) in 20 normal subjects. Two conditions: 1) self-generated initiation; and 2) initiation triggered by a "beep" sound were studied to evaluate the APA phase, by recording kinetic data (duration of the APAs, trajectory of the center of pressure, speed and trajectory of the center of mass). Kinematic data (first and second step speed, length and duration) were also recorded. RESULTS: First step speed and length were increased in self-paced gait initiation compared to triggered gait initiation in controls. We found no difference between the 2 conditions in terms of second step kinematic data. It was caused by a significant difference between the 2 conditions for the temporal characteristics of anticipatory postural adjustments (APAs) in the initiation of the first step, which was longer when normal subjects performed self-generated gait initiation. The trajectory of center of pressure and center of mass remained the same in the 2 conditions. CONCLUSION: APAs of gait initiation process are delayed under self-paced condition, although they do not differ qualitatively between reaction time and self-paced condition. Neuphysiological support of self-generated movement could explain these differences.

Acoustic Stimulation↗

PubMed2006

[Myasthenia gravis and pregnancy: clinical course and management of delivery and the postpartum phase].

OBJECTIVES: To study influences of pregnancy on the time-course of myasthenia gravis (MG) and of MG on pregnancy, delivery, postpartum and newborn. METHODS: We retrospectively collected data from 100 women affected with MG, hospitalized between 1994 and 2003 in departments of Neurology of Lille University Hospital. RESULTS: Eighteen patients had a total of 36 pregnancies, occurring 7.2 years on average after MG onset. MG exacerbation occurred in 7 patients (26 percent) during pregnancy and in 4 (14.8 percent) during postpartum. One patient died of acute respiratory failure during postpartum. Delay between the onset of MG and pregnancy was the only variable significantly associated with MG exacerbation: 5.8 years when exacerbation and 9.5 years when no exacerbation (p=0.03). Seven miscarriages, two therapeutic abortions and no death at birth were reported. Levels of anti-acetylcholine receptor antibodies were abnormal in 3 of 27 newborns (11 percent), but only one (3.7 percent) developed seronegative transient neonatal myasthenia gravis. DISCUSSION: During pregnancy, the clinical course of MG is variable but exacerbations were associated with a shorter delay between MG diagnosis and pregnancy. The risk of transient neonatal myasthenia gravis is relatively small but exists even when the parturient has stable MG without elevated levels of anti-acetylcholine receptor antibodies. CONCLUSION: Our study confirms pregnancy is more difficult to manage at the beginning of MG. Given the unpredictable course of MG during pregnancy, we recommend women affected with MG to begin a pregnancy when the disease is stable.

Abortion, Therapeutic↗

PubMed2006

[Lithium-induced encephalopathy mimicking Creutzfeldt-Jakob disease].

INTRODUCTION: Creutzfeldt-Jakob disease (CJD) has a poor prognosis. Certain clinical presentations can be suggestive yet mimic a curable disease. OBSERVATION: In the present study, we report the case of a 67-year-old man with a one-month history of progressive dementia, with myoclonic jerks and cerebellar syndrome suggesting a diagnosis of Creutzfeldt-Jakob encephalopathy. He had been treated for 9 years with lithium for a bipolar disorder. The results of the different investigations and the favorable course after discontinuation of lithium were in favor of the diagnosis of drug-induced Creutzfeldt-Jakob syndrome. CONCLUSION: This case illustrates the importance for researching a curable etiology in presence of clinical features suggesting a CJD.

Aged↗

PubMed2006

[Trichloroethylene intoxication presenting with temporal seizures].

INTRODUCTION: Trichloroethylene (TCE) is extensively used as a degreasing agent in the metal industry and as a solvent for organic compounds. Its neurotoxicity (with respect to both the peripheral and central nervous systems) is now widely acknowledged. OBSERVATION: Here, we report the case of a 32-year-old male drug addict presenting temporal seizures after "huffing" TCE (i.e. voluntary inhalation). The patient also deve loped a "psycho-organic syndrome" which included cognitive dysfunction (with memory disorders in particular) and personality changes. These disorders had not been noticed by the patient's wife prior to the last episode of inhalation. Four months later, the cognitive disorders had stabilized and no further seizures had been observed. CONCLUSION: The patient's overall clinical picture (notably featuring neuropsychological disorders) and electroencephalographic and brain imaging data argue in favor of a selective effect of TCE on the temporal lobe.

Adult↗

PubMed2006

Paradoxical response of VEGF expression to hypoxia in CSF of patients with ALS.

Vascular endothelial growth factor (VEGF) is implicated in motor neurone degeneration. In normal individuals, hypoxia is known to induce an overexpression of VEGF, as measured in CSF. We show that patients with ALS do not manifest this VEGF overexpression in the presence of hypoxia. Although VEGF gene expression is mainly stimulated by hypoxia, we have measured lower VEGF levels in cerebrospinal fluid (CSF) from hypoxaemic patients with amyotrophic lateral sclerosis (ALS) than in CSF from normoxaemic patients with ALS. In contrast, hypoxaemic neurological controls displayed higher levels than normoxaemic neurological controls. There was a negative correlation between VEGF levels and the severity of hypoxaemia in patients with ALS, suggesting deregulation of VEGF in ALS.

Adult↗

PubMed2006

The Lille apathy rating scale (LARS), a new instrument for detecting and quantifying apathy: validation in Parkinson's disease.

BACKGROUND: Apathy is usually defined as reduced interest and participation in various activities. It is a frequent consequence of neurological and psychiatric disorders. Although various scoring methods have been proposed, there is a lack of validated, standardised instruments for detecting apathy and assessing its severity. OBJECTIVE: To develop an apathy rating scale using a structured standardised interview capable of distinguishing between the condition's various features. METHODS: The Lille Apathy Rating Scale (LARS) is based on a structured interview. It includes 33 items, divided into nine domains. Responses are scored on a dichotomous scale. The participants used to validate the scale consisted of 159 patients with probable Parkinson's disease and 58 healthy control subjects. The Marin Apathy Scale, the Montgomery and Asberg Depression Rating Scale, and the Mattis Dementia Rating Scale were also administered. RESULTS: Principal component analysis showed that the LARS probed a single construct which forms the root of an oblique factor structure reflecting four dimensions: intellectual curiosity, self awareness, emotion, and action initiation. The main psychometric properties of the LARS (internal consistency, inter-rater and test-retest reliability) were satisfactory. Concurrent validity was evaluated by reference to the Marin scale and to judgements provided by expert clinicians. CONCLUSIONS: Standard validity indices showed that the LARS is sensitive and capable of distinguishing between apathy and depression. As a screening tool, the scale is able to support dichotomous judgements accurately and, when greater measurement sensitivity is required, also determine the severity of apathy within a four category classification.

Aged↗

PubMed2006

Clinical and economic results of bilateral subthalamic nucleus stimulation in Parkinson's disease.

BACKGROUND: High frequency stimulation of the subthalamic nucleus (STN) is an alternative but expensive neurosurgical treatment for parkinsonian patients with levodopa induced motor complications. OBJECTIVE: To assess the safety, clinical effects, quality of life, and economic cost of STN stimulation. METHODS: We conducted a prospective multicentre study in 95 consecutive Parkinson's disease (PD) patients receiving bilateral STN stimulation and assessed its effects over 12 months. A double blind randomised motor evaluation was carried out at 3 month follow up, and quality of life, self care ability, and predictive factors of outcome following surgery were assessed. The cost of PD was estimated over 6 months before and after surgery. RESULTS: The Unified Parkinson's Disease Rating Scale (UPDRS) motor score improved by 57% (p<0.0001) and activities of daily living improved by 48% (p<0.0001) at 12 month follow up. Double blind motor scoring improved by 51% at 3 month follow up (p<0.0001). The total PD Quality of Life Questionnaire (PDQL-37) score improved by 28% (p<0.001). The better the preoperative motor score after a levodopa challenge, the better the outcome after STN stimulation. Five patients developed an intracerebral haematoma during electrode implantation with permanent after effects in two. The 6 month costs of PD decreased from 10,087 euros before surgery to 1673 euros after surgery (p<0.0001) mainly because of the decrease in medication. These savings allowed a return on the procedure investment, estimated at 36,904 euros over 2.2 years. CONCLUSIONS: STN stimulation has good outcomes with relatively low risk and little cost burden in PD patients with levodopa induced motor complications.

Activities of Daily Living↗

PubMed2005

Elevated IL-6 and TNF-alpha levels in patients with ALS: inflammation or hypoxia?

Abnormal levels of interleukin (IL)-6 were described in patients with ALS, related to an inflammatory process. The authors compared IL-6 and tumor necrosis factor alpha (TNF-alpha) levels in CSF and sera from 10 hypoxemics and 10 normoxemics patients with ALS to those of 10 hypoxemics and 10 normoxemics neurologic controls. The same pattern exists in patients with ALS and controls: the highest levels are found in hypoxic conditions and undetectable levels are found in normoxemic conditions. Elevated IL-6 levels in ALS could correspond to a normal response to hypoxemia.

Aged↗