Biomedical subjects
Publications and source records attributed to A Autret.
A 62-year-old woman was admitted for a disorder of color vision. This cerebral achromatopsia was isolated, without prosopagnosia, alexia, object agnosia. MRI showed bilateral temporo-occipital infarcts, including lingual and fusiform gyrus. Neuropsychological examination and topographic hypotheses are discussed.
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A migrainous patient who experienced chest pain attributed to angina pectoris by coronary artery spasm during a migraine attack is reported. Previous reports have already mentioned the association of these two conditions and suggested that it might be the manifestation of a generalized vasospastic disorder. This new report offers an opportunity to review and discuss the available data on such an association.
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Eleven patients with Juvenile Myoclonic Epilepsy were treated using small doses of a sustained-release form of valproic acid (500 mg/day). This treatment has lasted for an average of 33 months (8-69) and has been continued in all of the patients. None of them has experienced a new generalized tonic-clonic seizure. The myoclonias have become considerably less frequent and less intense. No side-effects have been spontaneously reported by the group of patients. Thus, we suggest to start newly diagnosed patients on small doses of valproic acid.
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Hemisphere specialization for language was studied in 10 children with expressive developmental dysphasia (DD) (mean age 10 years 4 months) submitted to a dichotic listening task (in a word free-recall task and forced-attention task) and a finger tapping/vocalization dual-task paradigm. A nonsense shape dichaptic task was also introduced to control right hemispheric processing. Performances of dysphasic children were compared to those obtained from 15 normal children. The results showed that controls had a right ear advantage in free-recall (words) dichotic listening task and a significant right ear advantage in forced-right-attention task, with a change in ear asymmetry as a consequence of instruction. In the dysphasic group we observed a significant right ear advantage in the free-recall dichotic listening task and no change in ear asymmetry during forced right or forced left condition. Results in time sharing paradigm and nonsense dichaptic task are more difficult to interpret, because there was no interaction between group and condition. These results cannot support a complete left hemisphere dysfunction in developmental dysphasia.
From this review it appears that the slow wave sleep (SWS) increases the mean density of electroencephalographic paroxysmal activities (PA) whatever the epileptic syndrome. This pattern is not marked according to a bell curve among the epileptic population: about half the patients exhibit few or no PA during SWS, 20% show an increase during waking and another 20% during SWS. Begnin epilepsy with centro-temporal spikes is associated with an important sleep PA increase. In partial epilepsy, stage 3 and 4 sleep should increase the PA transmission. In children, a large increase in PA during SWS defines the continuous spike-wave during sleep syndrome, which is also observed in the syndrome of acquired aphasia with epilepsy of Landau-Kleffner; both conditions raise the issue of the neuropsychological consequences of the sleep PA. The sleep effect on the various epileptic models is analysed, showing a mean increase in PA during SWS and during transition between sleep and waking. This evidence is in agreement with the fact that during light sleep thalamocortical loops are functioning with an oscillatory pattern which facilitates PA expression. More hypothetic is the effect of sleep on the discharge rate of epileptic focus and on the cortical diffusion of the epileptic discharges. Gaba certainly participates in the thalamic influence, but its role on PA by the cortical and brain stem inhibition is speculative. Noradrenaline and acetylcholine, implicated in waking, reduce PA activity. Interindividual variations suggest that each epileptic has his own pathological neuronal organisation in which cortex and thalamocortical connexions are variously sensitive to the neurotransmitters implicated in sleep and waking.
The word agrypnia, ie, organic insomnia, was first used to describe a patient with a Morvan fibrillary chorea, which is an ill-delineated syndrome. This review considers the experimental insomnia created by raphe nuclei, anterior hypothalamic, or thalamic lesions. There are some papers reporting REM and non-REM sleep reduction in man after vascular, traumatic or degenerative lesion of the pons. There is only one case of agrypnia due to a bilateral stereotatic thalamic injury. Infectious agrypnia (trypanosomiasis, Von Economo) may exist but has not been documented by polygraphic means. Fatal familial insomnia induces a precocious agrypnia and leads to death with vegetative and motor disturbances. It is associated with an abnormal prion-protein which may interfere with gabaergic synapses. Finally agrypnia in humans corresponds to either lesionnal or infra microscopic synaptic prion linked disorders.
In order to clarify the relationship between developmental dysphasia and EEG abnormalities, paroxysmal activities during sleep were studied in a series of 24 children with expressive developmental dysphasia (mean age 8 years) and compared to a control group of 39 children (mean age 9 years). The children of both groups were selected excluding cases with prior history of neurological disease or epilepsy. In the control group, 37 children had normal sleep EEG while 2 children had paroxysmal abnormalities. In the dysphasic group, epileptic abnormalities were observed in 9 cases, rare in 4 cases and frequent in 5 cases (density: 2.5 to 66.2% of total sleep time). Nevertheless, paroxysmal abnormalities did not reach the frequency described in the Landau-Kleffner syndrome, and it is unlikely that EEG abnormalities could have produced dysphasia.
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Progressive supranuclear palsy (PSP) may sometimes be misdiagnosed as Parkinson's disease in its early stages, hence an early positive diagnosis of PSP based on dopamine D2 receptor density could be extremely valuable. In the present case report, the absence of dopamine D2 receptors was clearly demonstrated in the striatum using 123I-iodobenzamide (IBZM) tomoscintigraphy. This illustrates the potential use of IBZM tomoscintigraphy to identify Parkinson-like's disease presenting with decreased dopamine D2 receptor density; and hence to predict L-Dopa effectiveness. Further studies are needed to evaluate the vaue of IBZM tomoscintigraphy in the different Parkinson's like diseases.
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Sleep decreases considerably the abnormal movements seen during waking in the degenerative extra-pyramidal diseases. However, the electrophysiologic recordings reveal that muscular contractions are not completely abolished. This decrease parallels the reduction of the waking system which act likely as a non-specific system of amplification. One can notice that sleep modifies the characteristics of the parkinsonian tremor and that some palatal myoclonias persist during sleep.
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We describe the case of a human immunodeficiency virus-infected 34-year-old man with progressive multifocal leukoencephalopathy (PML). His case displayed unusual features, including a bizarre movement disorder, predominant involvement of the subcortical U fibers on neuropathologic examination, and the absence of MRI abnormalities suggestive of PML. Anatomic-clinical correlations are discussed.
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A 64-year-old woman was repeatedly hospitalized for various recurrent clinical signs of central nervous system involvement. The diagnosis of primary Sjögren's syndrome was established 3 years 6 months after the onset of the disease. Sicca symptoms, as well as inflammatory biological abnormalities were absent. Moreover, both lacrymal and salivary gland secretions were affected. A high level of antinuclear antibodies to SSA and SSB was associated with inflammatory lesions in minor salivary glands biopsy samples consistent with the diagnosis of Sjögren's syndrome.