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Biomedical subjects

A Destée

Publications and source records attributed to A Destée.

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

Tomographic measurements of regional cerebral blood flow in progressive supranuclear palsy and Parkinson's disease.

Intellectual changes observed in progressive supranuclear palsy (PSP) are sometimes seen with lesser intensity in Parkinson's disease (PD). Cognitive impairment of PSP has been attributed to a frontal lobe dysfunction explaining the frontal cortex hypometabolism detected by PET. To establish whether this frontal hypometabolism is more pronounced in PSP than in PD, we compared frontal and temporo-parietal cerebral blood flow (CBF) indexes studied by SPECT using Tc99m HmPAO in 18 PSP, 18 PD and 8 control subjects. For each patient neuropsychological performances were also assessed. A significant left frontal hypoperfusion was observed in PSP (mean index value: 0.78 +/- 0.03, p < 0.01) and PD (0.78 +/- 0.04, p < 0.05) as compared to controls (0.84 +/- 0.03), whereas there was no difference between PSP and PD. No correlation was discovered between neuropsychological performances and frontal cortical index changes. This frontal uptake reduction of Tc99m HmPAO in PSP and PD could result from a disconnection phenomenon secondary to subcortical lesions. In both groups mean frontal indexes showed only a left frontal hypoperfusion suggesting that subcortical structures might be asymmetrically involved in early stages of the diseases. The lack of difference for indexe values between PSP and PD might be explained by the difference between the mean disease duration: 4.3 years for the PSP and 7.8 years for the PD. It might also suggest that frontal CBF reduction exists in the same proportions in PD and PSP, but at a later stage in the former case.

Aged↗
PubMed1995

[Demonstration of a specific profile of pathological Tau proteins in frontotemporal dementia cases].

We compared samples of different brain areas from patients with Alzheimer's disease (AD), progressive supranuclear palsy (PSP), controls subjects and from 4 patients who met the clinical and pathological criteria for frontotemporal dementia (FTD), using a Western blot analysis. We used polyclonal antibodies directed against Tau proteins and the monoclonal antibody AD2 for the immunodetection of the pathological Tau proteins which are the basic components of neurofibrillary degeneration. In the PSP and AD cases, we respectively detected the abnormal Tau proteins 64 and 69 and the Tau proteins 55, 64, and 69, systematically associated with bands and smears, corresponding to catabolic products or aggregates of these abnormal Tau proteins. In FTD cases, the abnormal Tau proteins 55, 64 and 69 were also detected in the frontal and temporal poles from the autopsied case and in the cortical biopsies. However, the profiles were different because smears and proteolytics products of Tau proteins were absent. There was no detection of abnormal Tau proteins in control brain homogenates and in biopsies from patients with other neurodegenerative disorders such as spongiform encephalopathies or primitive gliosis. These results demonstrate that pathological Tau proteins are produced during FTD degenerating process, despite the absence of neurofibrillary lesions.

Adult↗
PubMed1994

Spatiotemporal study of Bereitschaftspotential and event-related desynchronization during voluntary movement in Parkinson's disease.

Bereitschaftspotential (BP) and Event-Related Desynchronization (ERD) were simultaneously recorded during a voluntary wrist flexion in 10 patients with Parkinson's disease (PD) treated with L-Dopa therapy and 10 control subjects. BP and ERD were analyzed 2 s before and 0.5 s after the movement, during two successive left and right experimental conditions. ERD (9-11 Hz) was computed from 11 source derivations (frontocentral, central, parietocentral). The BP was averaged from these 11 electrodes. For the BP, no spatiotemporal difference was found between the two groups. BP began bilaterally over the 3 groups of electrodes, 1250 ms before movement onset for the right flexion and 1500 ms for the left flexion. A contralateral predominance appeared 500 ms before movement onset over the central area. ERD began in the control group 1750 ms before movement over the contralateral central area, and then appeared bilaterally after its execution. In the PD group, the ERD appeared with a shorter latency than in the control group, 1250 ms (left flexion) and 1000 ms (right flexion) before movement onset; diffusion over the ipsilateral side was found 500 ms before movement onset. ERD also involved the frontocentral area and could be interpreted as a compensatory activity of the supplementary motor area. These findings suggest that with Parkinsonian and control subjects, ERD gives additional and maybe more information than the BP about changes of cortical activity during the motor preparation period. In the PD group the delay of ERD appearance seems to confirm that the programming of movement would be affected, thus explaining partially akinesia.

Aged↗
PubMed1994

[Klippel-Trenaunay syndrome and ischemic neurologic complications].

In 1900 Klippel and Trenaunay defined a new entity characterized by cutaneous angioma associated with varices and hypertrophy of bone and soft tissues. The syndrome is present in the earliest years and becomes accentuated as the child grows. It typically affects one half of the body. We report two cases of Klippel-Trenaunay syndrome. The first patient presented with flaccid paraplegia. On T1-weighted MRI sequences a diffuse heterogeneous high-intensity signal was visible opposite the T8 and T9 vertebral bodies. Spinal cord angiography showed occlusion of the anterior spinal artery issued from Adamkiewicz's artery, suggesting thrombosis. Serum fibrinopeptide A level was very high and compatible with hypercoagulability. The second patient presented with left hemiplegia caused by a right superficial sylvian artery infarct, and carotid angiography showed an image of right internal carotid artery dissection. These two cases suggest that Klippel-Trenaunay syndrome includes a state of hypercoagulability facilitating the occurrence of arterial thrombotic accidents, and an abnormality of the arterial wall capable of determining spontaneous dissection. A study of arterial wall-dependent coagulation factors should establish a link between these two aetiological factors. All this argues in favor of a diffuse vascular pathology, which makes this syndrome close to phakomatoses.

Adult↗
PubMed1994

Octreotide in the management of orthostatic hypotension in multiple system atrophy: pilot trial of chronic administration.

The aim of the study was to determine the potential effect of octreotide chronic administration on the functional prognosis of patients suffering from multiple system atrophy (MSA) with orthostatic hypotension (OH). Five patients received octreotide (100 micrograms s.c. injections t.i.d.) during 6 months in an open trial. Patients experienced functional improvement after 6 months of treatment. All of them continued to receive octreotide after the 6-month evaluation period (mean duration: 12 months).

Aged↗
PubMed1992

[From Parkinson's disease to Lewy body disease].

Lewy bodies are intraneuronal inclusions initially found in the pigmented brainstem nuclei of patients with Parkinson's disease. Their aspect varies according to their neuronal or cerebral situation. They have been a long time the hallmark of Parkinson's disease, but in recent years it has emerged that a small group of rare disorders or rare variants of common degenerative diseases are also sometimes associated with Lewy bodies in the nervous system. Pathological studies have also individualized a new disorder characterized by the presence of numerous Lewy bodies throughout the cerebral cortex and the brainstem: Lewy body disease. The clinical syndrome associates dementia, parkinsonian features, dysautonomia and motor neuron disease. The dementia is cortical in type and psychiatric symptoms such as agitation, hallucinations or delusions are frequent. The pathological features are nerve cell loss, diffuse Lewy bodies, and sometimes senile plaques. The origin of this disorder remains unclear, but it could be a primitive abnormality of neuronal cytoskeleton.

Alzheimer Disease↗
PubMed1992

Frontal lobe dysfunction in unilateral lenticulostriate infarcts. Prominent role of cortical lesions.

Most studies on frontal lobe dysfunction (FLD) in patients with striatal lesions did not consider possible associated cortical lesions not seen on computed tomographic scans. To determine the possible role of such cortical lesions, we assessed FLD in 10 patients with unilateral lenticulostriate infarct on computed tomographic scans. Magnetic resonance imaging revealed an associated cortical infarct not seen on computed tomographic scans in four patients. Using a battery of neuropsychological tests sensitive to FLD, we found that (1) the crossed tapping test was the only FLD test significantly disturbed in patients with pure unilateral lenticulostriate infarcts, (2) FLD was only present in patients with associated cortical infarct, and (3) caudate lesions only account for the number of echopraxic errors in the crossed tapping test. We conclude that unilateral isolated lenticulostriate infarcts might not lead to FLD, even though they may disturb the development of strategies involved in motor procedural learning.

Adolescent↗
PubMed1992

[Hashimoto's encephalopathy: toxic or autoimmune mechanism?].

A 36-year-old woman presented with partial complex status epilepticus. Magnetic resonance imaging with T2-weighted sequences showed a high-intensity signal in the left posterior frontal area. Hashimoto's thyroiditis was then discovered. The disappearance of the high-intensity signal after corticosteroid therapy was suggestive of an autoimmune mechanism. However, improvement could be obtained only with a hormonal treatment, which supports the hypothesis of a pathogenetic role of the Tyrosine-Releasing Hormone (TRH).

Adult↗
PubMed1992

[Cerebrotendinous xanthomatosis. 2 cases with magnetic resonance imaging].

A 40-year-old woman presented with bilateral juvenile cataract, tendinous xanthomas, intellectual deterioration, spastic tetraparesis, proprioceptive deficit and parkinsonian syndrome. A younger sister's clinical picture differed by the absence of xanthomas and the presence of a cerebellar syndrome. The diagnosis of cerebrotendinous xanthomatosis was confirmed by a high concentration of plasma cholestanol and by urinary chromatography. Magnetic resonance imaging displayed some abnormalities in the hemispheric and cerebellar white matter. Under chenodesoxycholic therapy the biological abnormalities decreased while the clinical disturbances were unchanged.

Achilles Tendon↗
PubMed1992

[Facial paralysis and chicken-pox].

Ten days after chicken-pox, a 24-year old man presented with a right facial palsy without meningitis. Albumino-cytologic dissociation in CSF--not observed in two other published cases--suggests an immune-mediated process. The patient was treated by prednisone and aciclovir. The upper facial muscles remained paretic 2 months after onset.

Acyclovir↗
PubMed1992

[Cavernous angioma of the cervical spinal cord].

A 34-year old man gradually developed a paraparesis which spontaneously regressed within a few months. One year later, the patient suffered from acute torticollis immediately followed by flaccid tetraparesis with pyramidal signs, dissociated sensory deficit and acute retention of urine. Magnetic resonance imaging (MRI) showed, in the cervical cord, a vascular malformation that was not opacified at angiography. The lesion, a cavernous angioma, was surgically removed, leading to recovery. So far, thirty five cases of spinal cord angioma have been published, most of them since the advent of MRI which makes it possible to determine the exact incidence of this lesion, as well as its signs, symptoms and course.

Adult↗
PubMed1992

[Mixed pre- and postsynaptic neuromuscular block].

We report a new case of neuromuscular block overlap between Myasthenia Gravis and Eaton-Lambert syndrome. A 64-year-old man with a 4-months history of gait disturbance was admitted for ophthalmoplegia worsening during exercise and decreasing at rest. Clinical examination after exercise, revealed limbs weakness and areflexia, palsy of the left eye abduction and a left ptosis. The level of anti-acetylcholin-receptor antibodies was high. Electrophysiological explorations revealed a decrement at 3 Hz and a increment at 30 Hz, with a reduction in amplitude of the initial motor potential. This patient improved under a combination of guanidine and anticholinesterase drugs. From this case and 9 previously reported cases, we propose 4 criteria for the diagnosis of such neuromuscular blocks: 1) exercising symptoms and signs, including areflexia, 2) presence of anti-acetylcholin-receptor antibodies, 3) reduction of the amplitude of the initial motor potential, with a decrement at 3 Hz, and an increment at 30 Hz, and 4) clinical and electrophysiological improvement under guanidine and anticholinesterasic drugs therapy.

Cholinesterase Inhibitors↗
PubMed1991

[Epidural angiolipoma and multiple familial lipomatosis].

In a 35-year old woman presenting familial multiple lipomatosis, spastic paraparesis developed and became worse under tetracosactide therapy. Signs of spinal cord compression at T3 were present. CT and MRI revealed a fat-containing epidural tumour (angiolipoma). The association of angiolipoma with familial multiple lipomatosis has not yet been reported. This association supports the theory that angiolipomas are hamartomatous lesions. The deterioration observed under tetracosactide suggests that iatrogenic epidural lipomatosis is due to the development of a pre-existing lipoma.

Adult↗
PubMed1991

[Hereditary parkinsonism-dystonia syndrome of juvenile onset with diurnal fluctuations].

Symptoms of fluctuating dystonia developed in 4 subjects of the same family during childhood or adolescence. In the 2 sisters, these symptoms were initially or subsequently associated with signs of parkinsonism, whereas in the 2 brothers they disappeared, spontaneously in at least 1 case, and signs of parkinsonism appeared later after a free interval. Anticholinergic agents and L-Dopa proved very effective against all extrapyramidal signs. These cases are similar to those gathered by Nygaard et al. in 1988 under the term "Dopa-responsive dystonia". Yet laboratory data seem to confirm that the common physiological mechanism is a disorder of tetrahydrobiopterin metabolism. Serum and urinary biopterin levels were lowered in our 4 cases but were normal in an unaffected sister. However, like the subjects affected this third sister showed a decrease of platelet serotonin which was taken as being a consequence of aromatic aminoacid hydroxylation defect due to tetrahydrobiopterin deficiency.

Adolescent↗