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L Vercueil

Publications and source records attributed to L Vercueil.

30 records · Page 2Linked to original sources
PubMed2000

Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study.

Pallido-luysio-nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62-year-old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2-weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO-SPECT and FDG-PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.

Age of Onset↗
PubMed2000

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Editorial↗
PubMed2000

[Clinical diagnostic criteria in Parkinson's disease and parkinsonian syndromes: review of the literature].

This paper reviews the validity of clinical diagnostic criteria developed for Parkinson's disease and parkinsonian syndromes. Proposed criteria are predominantly empirical. There are only a few studies with neuropathological verifications. For Parkinson's disease and Progressive Supranuclear Palsy clinicopathological studies have been performed and led to pathologically based criteria. In this context, the specificity and sensitivity of clinical criteria could be assessed. Reliability however has seldom been tested. In contrast, the proposed criteria for the clinical diagnosis of Multiple System Atrophy and Corticobasal atrophy have not been pathologically evaluated.

Humans↗
PubMed1999

Breathing pattern in patients with Parkinson's disease.

The improvement in motor performance resulting from levodopa administration in patients with Parkinson's disease (PD) provides the opportunity to investigate ventilatory changes brought about by the disease. The aim of this study has been to investigate these changes in order to specify the mechanisms of the impairment in breathing in PD. Breathing patterns at rest were investigated in 11 patients with idiopathic PD both before (OFF) and after (ON) administration of levodopa at a dose improving their motor performance by at least 30%. Airflow (Fleisch head mounted on a mask), rib cage and abdomen movements (inductance plethysmography) were recorded in the OFF condition 1 h after subjects woke up. Subjects then received levodopa and a new set of recordings was obtained 1 h later, in the ON condition. Breath-by-breath processing of recordings was carried out, and tidal volume (VT), inspiratory (TI) and expiratory (TE) durations were measured. The main finding was a lengthening of TI resulting in a decrease in ventilation and in VT/TI, and an increase in TI/TTOT in the ON compared to the OFF condition. In the ON condition abnormal rib cage-abdomen plots patterns were found in four out of six subjects. A hypothesis on the effect of PD on breathing is proposed on grounds of normal diaphragmatic activity but impaired activity of the other respiratory muscles and more specifically the intercostal muscles.

Aged↗
PubMed1999

Basal ganglia involvement in rotational seizures.

Body rotation during partial epileptic seizures remains a poorly understood clinical feature, possibly related to the ictal involvement of specific cortical areas (e.g. vestibular cortex). However, there are both experimental and clinical arguments which support the hypothesis of basal ganglia asymmetric activation during such complex motor behavior. We report a patient exhibiting rotational seizures which proved to arise from right anterior temporal lobe structures and the orbito-cingular cortex, as demonstrated during stereotactic EEG recordings. An ictal 99mTc-ECD-SPECT study was performed during an electrically induced seizure, similar to the seizures usually experienced by the patient but without a rotatory component, showing a marked increase of cerebral blood flow in the lenticular nucleus, ipsilaterally to the epileptogenic area. This finding suggests that lenticulate involvement might be necessary to produce an ictal circling behavior. As circling behavior can be observed during epileptic seizures originating from areas widely distributed in the cortex, one may postulate that the basal ganglia involvement is the "final common pathway" underlying the rotational part of the clinical symptomatology.

Basal Ganglia↗
PubMed1998

High-frequency stimulation of the subthalamic nucleus suppresses absence seizures in the rat: comparison with neurotoxic lesions.

High-frequency electrical stimulation of deep brain structures has recently been developed for the surgical approach of neurologic disorders. Applied to the thalamus in tremors or to the subthalamic nucleus in Parkinson's disease, high-frequency stimulation has been demonstrated to exert a local inhibiting influence, leading to symptoms alleviation. In the present study, bilateral high-frequency stimulations (130 Hz) of the subthalamic nuclei suppressed ongoing spontaneous absence seizures in rats. This effect was dissociated from motor side-effects and appears specific to the subthalamic nucleus. Bilateral excitotoxic lesions of the subthalamic nuclei only partially suppressed absence-seizures. These results confirm the involvement of the basal ganglia system in the control of generalized seizures and suggest that high-frequency stimulations could be used in the treatment of some forms of seizures.

Animals↗
PubMed1998

The role of basal ganglia in the control of generalized absence seizures.

During the last two decades, evidence has accumulated to demonstrate the existence, in the central nervous system, of an endogenous mechanism that exerts an inhibitory control over different forms of epileptic seizures. The substantia nigra and the superior colliculus have been described as key structures in this control circuit; inhibition of GABAergic neurons of the substantia nigra pars reticulata results in suppression of seizures in various animal models of epilepsy. The role in this control mechanism of the direct GABAergic projection from the striatum to the substantia nigra and of the indirect pathway, from the striatum through the globus pallidus and the subthalamic nucleus, was examined in a genetic model of absence seizures in the rat. In this model, pharmacological manipulations of both the direct and indirect pathways resulted in modulation of absence seizures. Activation of the direct pathway or inhibition of the indirect pathway suppressed absence seizures through disinhibition of neurons in the deep and intermediate layers of the superior colliculus. Dopamine D1 and D2 receptors in the nucleus accumbens, appear to be critical in these suppressive effects. Along with data from the literature, our results suggest that basal ganglia circuits play a major role in the modulation of absence seizures and provide a framework to understand the role of these circuits in the modulation of generalized seizures.

Animals↗
PubMedDate not supplied

[Control of inner speech and Gilles de la Tourette's syndrome].

UNLABELLED: Inner speech is the little voice in the head which comments on everyday life activities. To some aspect, trouble in monitoring the inner speech has been regarded as relevant in auditory hallucinations in psychosis. Another disorder that may involve inner speech is the difficulty inhibiting its vocal utterance (the so-called verbal impulsion ). Verbal impulsion is a classic feature of Gilles de la Tourette's syndrome, during which patients complain of difficulties inhibiting socially undesirable behaviors, especially vocalization (i.e. coprolalia). In a previous paper, we reported on a patient with frontotemporal dementia, whose pathology was revealed by a difficulty inhibiting the verbal utterance of reading (inability to read silently). Later in the course of the disorder, the patient exhibited a severe frontal lobe syndrome with compulsive activities and coprolalia. Externally triggered inner speech has been proposed by the author to be represented by silent reading. In this setting, loss of silent reading can be regarded as the equivalent of a loss of control of the verbal utterance of the inner speech. CASES REPORT: In the present paper, a loss of control of inner speech is suggested as partly involved in two cases of Gilles de la Tourette's syndrome with coprolalia. Two adult patients with a diagnosis of Gilles de la Tourette's syndrome since age 7 and 10, respectively, have been asked to comment on their present or previous abilities to read silently. The first case never silent read as a child while the second case read silently but had a tendency to move his lips. DISCUSSION: The frequency of observed difficulties in silent reading is likely to be a function of the age at which the subject is asked about such difficulties. Nevertheless, theoretical arguments are presented in this part of the paper which suggest that silent reading, considered as a way to challenge the control of inner speech, has been variably impaired at different stages of the disease. The author speculates that the key role of prefrontal cortex, especially the orbito-frontal areas, in the inhibitory control of current behavior, can be responsible for the deficit in the control of inner speech. In this setting, recent functional brain imaging performed in patients with Gilles de la Tourette's syndrome showed a striking deficit in the activation of these areas during tics. It would be of great interest to address this specific question in determining the frequency of difficulties in silent reading in Gilles de la Tourette's syndrome individuals as compared to those without Gilles de la Tourette's syndrome. In this way, the so-called Stroop-test should be adequate in addressing this pivotal issue. In this well-known neuropsychological test the subject is asked to orally report the color in which the name of color has been written. The discrepancy between the color of the written word and the name of the color that have been read can challenge the ability of the subject to correctly inhibit the reading (in example the word blue ) in order to say another color (in example red , if the word blue is red colored). CONCLUSION: Loss of silent reading observed in such patients is suggestive of the failure of the prefrontal inhibitory control on the verbal utterance of inner speech. Further research is needed to confirm these findings.

Adult↗