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Biomedical subjects

J L Signoret

Publications and source records attributed to J L Signoret.

At least 37 records · Page 2Linked to original sources

[Aphasia without amusia in a blind organist. Verbal alexia-agraphia without musical alexia-agraphia in braille].

A 77 year old right handed male was blind since the age of 2. He presented with an infarction involving the territory of the left middle cerebral artery involving the temporal and the inferior parietal lobes. He had learned to read and write language as well as read and write music in braille, ultimately becoming a famous organist and composer. There were no motor or sensory deficits. Wernicke's aphasia with jargonaphasia, major difficulty in repetition, anomia and a significant comprehension deficit without word deafness was present; verbal alexia and agraphia in braille were also present. There was no evidence of amusia. He could execute in an exemplary fashion pieces of music for the organ in his repertory as well as improvise. All his musical capabilities: transposition, modulation, harmony, rythm, were preserved. The musical notation in braille remained intact: he could read by touch and play unfamiliar scores, he could also read and sing the musical notes, he could copy and write a score. Nine months after the stroke his aphasia remained unchanged. Nevertheless he composed pieces for the organ which were published. Such data highly suggest the independence of linguistic and musical competences, defined as the analysis and organization of sounds according to the rules of music. This independence in an extremely talented musician leads to a discussion of the role of the right hemisphere in the anatomical-functional processes at the origin of musical competence. The use of braille in which the same constellations of dots correspond either to letters of the alphabet or musical notes supports the independence between language and music.

Aged

[Myoclonic cerebellar dyssynergia (Ramsay-Hunt syndrome) and cerebellar telangiectasia].

A 8 year-old girl presented with generalized epileptic seizures followed by the progressive onset of myoclonic jerks, sometimes associated with willed movements, and a static and kinetic cerebellar syndrome without conspicuous intellectual impairment. Death occurred 10 years after the onset of the disorders. There was no family history. Neuropathological studies showed lesions confined to the cerebellum. Diffuse and bilateral telangiectases were present in the cerebellar white matter. They were associated with patchy cortical alterations of the distal parts of some folia involving mainly the granule-cells ans sparing the Purkinje cells. No Lafora bodies and no abnormal lipofuscin storage were observed. The dentate nuclei, superior cerebellar peduncles and red nuclei were normal as were the inferior olives and inferior cerebellar peduncles. The spino-cerebellar tracts were unaffected. This case confirms the hypothesis that dyssynergia cerebellaris myoclonica corresponds only to a clinical entity. It may be encountered in various degenerative or metabolic disorders involving the cerebellum and/or its pathways. To our knowledge the association of a Ramsay Hunt syndrome with a vascular malformation has not been previously reported.

Adolescent

[Memory disorders in lesions of the thalamus in man].

Isolated memory disorders occur after bilateral involvement of thalamus. Anterograde amnesia is not complete; retrograde amnesia is always severe. Four groups of thalamic nuclei may be affected: anterior nuclei; midline nuclei, medialis dorsalis nuclei; intralaminar nuclei. A pathophysiologic interpretation is proposed. Three kinds of memory processes may be involved: consolidating process that leads to the formation of memory traces (anterior nuclei, midline nuclei); retrieving process that leads to the activation of memory traces (intralaminar nuclei); temporal organizing process that regards ancient et recent memory traces (medialis dorsalis nuclei).

Amnesia

[Neuropsychological analysis of Alzheimer's disease].

Neuropsychological analysis, which aims at establishing correlations between mental disorders and alterations in one or several functional cerebral structures, cannot be performed at the dementia stage of Alzheimer's disease. From the nature and variety of the mental disorders observed it may be hypothesized that the disease does not involve one single biochemical system (cholinergic theory) but several systems of neurotransmitters, each of which plays a specific role in man's mental activities.

Aged

[Contribution of the right hemisphere to language in aphasic patients. Disappearance of this language after a right-sided lesion].

Assessments vary as to the contribution of the right hemisphere to language in aphasic patients. Results of a clinicopathologic study in two right-handed subjects with aphasia are reported. The lesions involved the territories of the left middle and anterior cerebral arteries. Oral productions in one case, studied during the three weeks of survival, were limited to automatic series, recitation of a fable, and completion of sentences. Follow-up in the other case was possible during 2 years of language rehabilitation. At the end of this period, repetition of an echolalic type was possible; a propositional expression had appeared, reduced to substantives and verb infinitives; denomination was possible but rich in semantic paraphasias. A second infarction in the right sylvian region caused the recovered language to disappear and to be replaced by an abolition of all communication. Proof was thus obtained that the progress accomplished was dependent on the right hemisphere. These findings are discussed in the light of observations of patients following left hemispherectomy and of the capacity of the right hemisphere to generate language as demonstrated in patients after commissurotomy. A dynamic interpretation of the taking over of expression by the right hemisphere during some aphasias is proposed.

Aphasia

[Creation of Charcot's chair].

A decree signed by the President of the French Republic on the 2nd of January 1882 provided for the creation of the first chair in the world for the teaching of neurology at the Faculty of medicine in Paris. The chair was named: Clinic for Diseases of the Nervous System and was attributed to J. M. Charcot. The historical and political circumstances related to the creation of this chair are reviewed. J. M. Charcot's hospital and university careers are retraced, emphasis being placed on the turning point in 1867 when J. M. Charcot, having been refused the chair of Medical Pathology, dedicated his time to the study of hysteria and nervous system diseases. The birth of neurology is therefore situated in relation to that of psychiatry. But this chair is also and still that of Charcot; it was thus convenient to remind the man, his origins, his personality, his ideas, and his social role.

History, 19th Century

Bromocriptine associated with a peripheral dopamine blocking agent in treatment of Parkinson's disease.

A peripheral dopaminergic blocking agent, domperidone (60 mg daily), or placebo was given, double-blind, to 17 parkinsonian patients who also received increasing doses of bromocriptine. Combined treatment with domperidone reduced total disability by 76% in 8 patients receiving a mean dose of 148 mg of bromocriptine daily. There was no vomiting and involuntary movements and psychic disturbances were similar to those in patients on levodopa and a peripheral decarboxylase inhibitor. In 9 patients taking placebo instead of domperidone, the average daily dose of bromocriptine could not be raised beyond 92 mg. The mean total disability score in this group was reduced by only 48%. Thus, peripheral blockade of dopamine receptors is a promising means of limiting the adverse side-effects of the treatment of parkinsonism with central dopaminergic receptor stimulating agents such as bromocriptine.

Adult

[Influence of choline on amnesia in Alzheimer's disease (author's transl)].

Eight patients with Alzheimer's disease presenting whith memory difficulties and no significant intellectual deterioration were treated with 9 grams daily. Choline base orally for 3 weeks. Quantitative testing, which evaluate memory capabilities such as learning and recall after delays of 1 hour and 24 hours, were used before and after the administration of Choline. The results of a global statistical analysis did not show any significant difference in the performances obtained before and after choline. However, in two patients, recall performance improvement was confirmed clinically. Most likely, these two patients were suffering from an mild foorm of Alzheimer's disease.

Aged