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Biomedical subjects

M Vidailhet

Publications and source records attributed to M Vidailhet.

At least 127 records · Page 7Linked to original sources
PubMed1995

Striatal dopaminergic denervation in pallidopyramidal disease demonstrated by positron emission tomography.

Pallidopyramidal disease is a rare disease of young patients in which they manifest a parkinsonian syndrome and pyramidal signs. Pallidopyramidal disease has been attributed to a degeneration of the pallidum and the pyramidal tract, although only 1 patient has been studied postmortem. In the present report, [18F]fluorodopa positron emission tomography in 2 patients showed a marked dopaminergic denervation of the striatum, suggesting that this disease corresponds more to a nigropallidopyramidal dysfunction.

Adolescent↗
PubMed1995

The bereitschaftspotential preceding stepping in patients with isolated gait ignition failure.

The cerebral activity preceding voluntary, self-paced ankle dorsiflexion movements while seated was compared with that before a stepping movement of the same foot while standing in four patients with isolated gait ignition failure. Results within the patients group were variable, but all exhibited some increase in the Bereitschaftspotential (BP) amplitude when stepping as compared with sitting, similar to that seen in normal subjects. This differs from previous results in patients with Parkinson's disease in which stepping was not accompanied by a normal increase in BP amplitude. This difference suggests that the mechanisms responsible for the start hesitation that is common to both Parkinson's disease and gait ignition failure may differ in the two conditions.

Aged↗
PubMed1995

"Pure" striatonigral degeneration and Parkinson's disease: a comparative clinical study.

Striatonigral degeneration (SND) is difficult to diagnose in vivo. The purpose of this study was to detect the best indicators for an early and reliable diagnosis of the disease. Eighteen patients clinically diagnosed as having SND were selected with rigorous inclusion criteria and compared to 18 patients with Parkinson's disease (PD) matched for age and disease duration. Apart from dysautonomia, the principal discriminant clinical features that distinguished SND from PD were the early appearance of the following symptoms and signs: (a) severe and atypical progressive parkinsonism characterized by bilateral bradykinesia and rigidity, slowness of gait, postural instability, and falls, and poor or absent response to adequate levodopa treatment; (b) increased tendon reflexes associated or not with frank pyramidal signs, severe dysarthria, and less consistently, dysphagia, stridor, antecollis, and stimulus-sensitive myoclonus, which, when present, are highly suggestive of the disease.

Aged↗
PubMed1995

Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy.

To study the neuropsychological pattern of striatonigral degeneration (SND), 14 consecutive patients with probable SND were submitted to an extensive battery of neuropsychological tests. Compared with controls the performance of patients with SND was impaired on category and phonemic fluency, frontal behaviours, trail making test A and B, and free recall of the Grober and Buschke test, but normal on the revised WAIS verbal scale, Raven 47 coloured progressive matrices, Wechsler memory scale, California verbal learning test, Wisconsin card sorting test, and the Stroop interference condition. The performance of patients with SND was also compared with that of 14 patients with Parkinson's disease and 14 patients with progressive supranuclear palsy (PSP) matched for age at onset, duration of disease, severity of intellectual deterioration, and depression. The results showed that the dysexecutive syndrome of SND is similar to that of Parkinson's disease and less severe than in PSP.

Analysis of Variance↗
PubMed1995

Levodopa-induced dyskinesias are improved by fluoxetine.

We evaluated the severity of motor disability and dyskinesias in seven levodopa-responsive patients with Parkinson's disease after an acute challenge with the mixed dopamine agonist, apomorphine, before and after the administration of fluoxetine (20 mg twice per day) for 11 +/- 1 days. After fluoxetine treatment, there was a significant 47% improvement (p < 0.05) of apomorphine-induced dyskinesias without modification of parkinsonian motor disability. The dyskinesias were reduced predominantly in the lower limbs during the onset and disappearance of dystonic dyskinesias (onset- and end-of-dose dyskinesias) and in the upper limbs during choreic mid-dose dyskinesias. The results suggest that increased brain serotoninergic transmission with fluoxetine may reduce levodopa- or dopamine agonist-induced dyskinesias without aggravating parkinsonian motor disability.

Antiparkinson Agents↗
PubMed1995

The neuropsychological pattern of corticobasal degeneration: comparison with progressive supranuclear palsy and Alzheimer's disease.

The pattern of cortical and subcortical neuropathologic lesions in corticobasal degeneration (CBD) should predict a specific cognitive profile in this disease. To characterize this profile and to determine its specificity by comparison with progressive supranuclear palsy (PSP) and senile dementia of the Alzheimer's type (SDAT), we used an extensive neuropsychological battery assessing global efficiency, executive functions, various tests of encoding and retrieval, dynamic motor organization, and upper limb praxis. We compared the performance of patients with CBD (n = 15) with that of controls (n = 19) matched for age and education, and with that of patients with PSP and SDAT (15 in each group), matched for severity of dementia and depression. Patients with CBD showed: (1) a moderate global deterioration; (2) a dysexecutive syndrome similar to that of patients with PSP and more severe than in SDAT; (3) explicit learning deficits, without retention difficulties and easily compensated by using the same semantic cues at encoding and retrieval as in PSP; this was in contrast with SDAT where cued recall and recognition were also impaired; (4) disorders of dynamic motor execution (temporal organization, bimanual coordination, control, and inhibition) similar to those of patients with PSP and not in SDAT; (5) asymmetric praxis disorders (posture imitation, symbolic gesture execution, and object utilization) that were not observed in PSP or SDAT. Patients with CBD show a specific neuropsychological pattern associating a dysexecutive syndrome, likely due to degeneration of the basal ganglia and prefrontal cortex, and asymmetric praxis disorders, which might be related to premotor and parietal lobe lesions. This neuropsychological profile may help to distinguish this condition clinically from other neurodegenerative diseases.

Aged↗
PubMed1995

[Role of home care in the management of patients with mucoviscidosis].

Home care, sometimes in the technical form of home hospitalization, is essential in caring for patients with cystic fibrosis, both to avoid separating the children from their environment and rupturing their life rhythms, but also to reduce the risk of hospital contamination. Home care requires an evaluation of the disease status, good patient and family information and good coordination between the different partners. Thus the reduction in hospitalization greatly improves the quality of life in patients with cystic fibrosis.

Cystic Fibrosis↗
PubMed1995

[Iron and pregnancy].

Infants, young children, and childbearing aged women are particularly exposed to iron deficiency. Pregnancy further increases iron requirements. Nevertheless the consequences of anemia and/or iron deficiency on pregnancy outcome, development of the foetus and postnatal iron status of the infant, remain to be determined. There is a 3-fold increase of premature deliveries in iron deficient anemic pregnant women whose anemia is discovered in early pregnancy: however this increased risk of premature delivery is not observed when iron deficiency anemia is discovered in late pregnancy. Iron supplementation during pregnancy improves the maternal hematological parameters but it is still unclear whether it also improves the maternal health and the pre and postnatal development of the child. Based on our actual knowledge, iron supplementation during pregnancy is to be recommended in risk groups only (ie mainly adolescents, low income women, women with multiple pregnancies), using ferrous iron at a dosage of 30 mg per day.

Anemia, Iron-Deficiency↗
PubMed1994

Eye movements in parkinsonian syndromes.

Eye movements were recorded in 14 patients with Parkinson's disease (PD) in the "off" condition, 14 patients with striatonigral degeneration (SND), 10 patients with corticobasal degeneration (CBD), and 10 patients with progressive supranuclear palsy (PSP), with comparison with 12 control subjects. Vertical saccade paralysis was not observed in the PD, SND, and CBD groups but was present in 9 patients of 10 in the PSP group. In the PD and SND groups, horizontal reflexive visually guided saccade latency and accuracy were similar, and differed only slightly from those of controls. In the CBD group, saccade latency was significantly increased and correlated to an "apraxia score"; whereas, in the PSP group, saccade amplitude was significantly decreased. Thus, the abnormalities of both horizontal saccade parameters in the PSP group contrasted with those observed in the CBD group. The percentage of errors in the antisaccade task, an index of prefrontal dysfunction, was markedly increased only in the PSP group. The smooth pursuit gain was decreased in all groups but more severely in the PSP group. It may be concluded that saccade abnormalities are clearly different in SND, CBD, and PSP, and might help in early differential diagnosis in individual patients, but that SND cannot be differentiated from PD on the simple basis of eye movement abnormalities.

Aged↗
PubMed1994

Sequences of memory-guided saccades in Parkinson's disease.

Sequences of ocular saccades were studied in 8 patients with Parkinson's disease, with (i.e., "on") and without (i.e., "off") levodopa treatment. The amplitude of single saccades was decreased and not improved by treatment. The chronology of saccades during sequences was impaired in patients in the "off" state, but significantly improved during the "on" period. These results are compatible with a decreased activation of the supplementary motor area, which can be reversed by levodopa.

Electrooculography↗
PubMed1994

Levodopa-induced dyskinesias in Parkinson's disease phenomenology and pathophysiology.

The aim of this study was to provide further insight into the phenomenology and pathophysiology of monophasic and biphasic dyskinesias induced by levodopa in Parkinson's disease. For this purpose, the type, localization, severity, and timing of dyskinesias were evaluated in 15 parkinsonian patients in relation to motor disability after administration of levodopa using a video-electromyographic recording device. Foot-dystonia, myoclonus, and akathisia were observed in most patients. The dyskinesias started in the foot, usually on the side most affected by the disease, and spread in an "ascending wave" to the contralateral side, the trunk, and upper extremities. In a few patients, onset was axial, spreading almost instantaneously to all limbs. The dyskinesias were dystonic and ballistic at the start, and became increasingly choreic as they attained the upper limbs. Their intensity was maximal in the lower limbs, then progressively decreased, while increasing in upper limbs and head. The results indicate that there is no strict dichotomy between biphasic and monophasic dyskinesias. In other words, there is a "continuum" between the first dyskinesias and those observed during the period of maximal clinical improvement. These dyskinesias can also appear in reverse order, as if there were an "oscillator" determining a sequence of alternating patterns.

Activities of Daily Living↗
PubMed1994

The auditory startle response in progressive supranuclear palsy.

The EMG characteristics of the normal auditory startle response in man are compatible with an origin in the pontine reticular formation and with conduction down the spinal cord in a slowly conducting, possibly reticulo-spinal pathway. The startle was reduced or absent in patients with progressive supranuclear palsy, consistent with loss of neurones in the lower pontine reticular formation. In contrast, the startle was present and of normal form in patients with Parkinson's disease. However, it was delayed in onset. This result was not influenced by treatment with L-dopa. The late auditory startle in Parkinson's disease might be related to withdrawal of facilitatory input to brainstem centres from the basal ganglia.

Aged↗
PubMed1994

Null alleles of the aldolase B gene in patients with hereditary fructose intolerance.

We report three new mutations in the gene for aldolase B that are associated with hereditary fructose intolerance (HFI). Two nonsense mutations create opal termination codons: R3op (C-->T, Arg3-->ter, exon 2) was found in homozygous form in four affected members of a large consanguineous Turkish pedigree and R59op (C-->T, Arg59-->ter, exon 3) was found on one allele in a woman of Austrian origin known to harbour one copy of the east European mutation, N334K (Asn334-->Lys). The third mutation occurred in a French HFI patient known to be heterozygous for the widespread mutation, A174D (Ala174-->Asp): a single mutation, G-->A, in the consensus acceptor site 3' of intron 6 was found on the remaining allele. These mutations are predicted to abrogate synthesis of functional protein and thus represent null alleles of aldolase B. The mutant alleles can be readily detected in the amplification refractory mutation system (ARMS) or (for R59op and 3' intron 6) by digestion of amplified genomic fragments with DdeI or A1wNI, respectively, to facilitate direct diagnosis of HFI by molecular analysis of aldolase B genes.

Alleles↗