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Biomedical subjects

A Destée

Publications and source records attributed to A Destée.

At least 19 recordsLinked to original sources

[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

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

[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

[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

[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

[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

[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

[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

[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

[Bilateral cavernous sinus syndrome: Burkitt's lymphoma].

Bilateral cavernous sinus syndrome is usually due to a vascular disease, such as thrombophlebitis or arteriovenous malformation. In a 29-year old woman this syndrome revealed a malignant non-Hodgkin's lymphoma of the Burkitt type. In this case, the physiopathological mechanism was metastatic extension to the dura mater of a systemic lymphoma. The contribution of MRI to the diagnosis is emphasized.

Adult

[Correlation of thalamic aphasia and cerebral blood flow].

Relations between linguistic deficits and cerebral blood flow (CBF) were studied in 20 cases of thalamic aphasia due to hemorrhage. Language analysis was based on BDAE, verbal intelligence quotient and verbal subtest of the memory quotient (Wechsler). CBF analysis (and of asymmetry index: AI) was done with 133 Xenon by SPECT technique in tomographic slices and in 15 areas of interest, i.e. cortical and deep areas. Relationships were analyzed by multiple correlations procedure and stepwise regression. Significant correlations were observed between linguistic results and AI of cortical but also deep areas (lenticular). Dynamic anomalies (fluency) were correlated with the IA and/or CBF of the frontal cortex. Verbal comprehension, naming and paraphasia were related to the AI of deep structures (insula and lenticular nucleus) and the AI of posterior cortex (temporo-occipital). Several correlations were found significant between results on verbal IQ of the WAIS and IA of the insula and lenticular nucleus.

Aged

[Benign, monomelic juvenile amyotrophy of a hand (Hirayama type): a new case report].

Benign, monomelic juvenile amyotrophy of the hand (Hirayama type): new data.--Within the space of a few months a 21-year-old man developed isolated amyotrophy and paresis of the right hand; thirteen years later the clinical picture was unchanged. Medullo-cervical MRI was normal. The EMG confirmed the involvement of the anterior horn. Unilateral chronic distal juvenile amyotrophy located in an upper limb (Hirayama type) was diagnosed on clinical findings. But the spread of electrical abnormalities to the lower limbs suggested a transitional form between this syndrome and other disorders of the anterior horn.

Adult

[Hemiballismus. Hemichorea. Striatal infarction].

Lesions of other structures than corpus Luysii, may rarely cause hemiballism. A case is reported of hemiballism due to infarction of the territory supplied by the lenticulo-striatal arteries. CT and MR imaging showed lesions of the caudate nucleus and putamen. This suggested that the cause of the hemiballism was the suppression of the regulatory activity of the caudate nucleus on the pallidum.

Aged

[Prolonged remission in subacute sclerosing panencephalitis: 2 cases].

Long survival in subacute sclerosing panencephalitis (SSPE), including total disappearance of clinical signs, is rare. Two cases are reported. They concern a girl and a boy who, at age 13 and 15, developed SSPE and are still in remission 6 and 5 years later. After a typical onset and course over periods of 12 and 18 months, clinical improvement was observed and periodic EEG complexes disappeared. However, the electrophoretic oligoclonal pattern of CSF proteins and the elevated measles titers persisted (in one case specific CSF IgM were still increased 6 years after the onset). MRI showed asymmetrical areas of high-intensity signal in both white and gray matter, predominant in the temporal, parietal and occipital regions. The age at which SSPE begins and the interval between measles and SSPE onset are not prognostic factors. On the other hand, in reported cases with lasting remission SSPE did not progress beyond Jabbour's stage II. The second typical feature of these long-term improvements is disappearance of EEG periodic complexes and emergence of a normal basic background activity. No other prognostic factor has been reported.

Adolescent

[Memory disorders in chickenpox encephalitis].

A 31-year old man with chicken-pox encephalitis presented with a cerebellar syndrome and disorders of memory. The latter consisted of severe anterograde amnesia with normal retrograde memory, without confabulation or anosognosia. This suggested that the hippocampus was involved, probably functionally, since the outcome was rapidly favourable and the MRI was normal.

Adult

[Protracted recurrent neuroleptic malignant syndrome].

The natural course of untreated neuroleptic malignant syndrome (NMS) does not usually exceed 3 weeks. Its duration is reduced by dantrolene and/or bromocriptine. We report a patient in whom NMS developed after an injection of haloperidol decanoate and fluctuated during 3 months. This was due perhaps because the initial treatment with oral neuroleptic was pursued, or because the doses of dantrolene were insufficient.

Adult

[Hypothermia and multiple sclerosis. A case with 3 episodes of transient hypothermia].

On three occasions over a 21-month period, a woman with multiple sclerosis presented with hypothermia accompanied by altered consciousness, neurological signs and inappropriate antidiuretic hormone secretion. One of the episodes included hypoglycaemia. Although repeated MRI examinations, one of them with gadolinium injection, gave negative results, hypothalamic demyelination was suspected. The 4-year follow-up of this patient suggests that this lesion has no prognostic value.

Adult