[The evolution of adult clinical neuropsychology in Paris from 1957 to 2000]. / L'évolution de la neuropsychologie clinique de l'adulte à Paris de 1957 à 2000.

This sketchy account of the history of French-speaking clinical neuropsychology from 1957 to 2000 first recalls the Salpêtrière school leaded by Alajouanine and his disciples, and second the Ste Anne psychiatric hospital, with on one hand the Chair of Mental and Brain Diseases, and on the other hand a number of research teams gathered around disciples of Dejerine such as Jean Lhermitte. Because of the large scope of this topic, the authors could not elude the need for a bibliographic selection that may seem questionable.

Identification and discrimination disorders in auditory perception: a report on two cases.

Auditory perception was investigated in two brain-damaged subjects. The first patient had a left temporoparietal ischaemic lesion. He presented a right-ear extinction in dichotic tasks, as well as difficulties in understanding and repeating verbal material and impaired identification of melodies. All discrimination tests were well performed. The second patient had a right capsulolenticular and frontal ischaemic lesion. He presented a left ear dichotic extinction and severe difficulties in discrimination of environmental sounds and melodies but no major difficulty in naming and identification. From these results, it is hypothesized that identification and discrimination involve distinct mechanisms within the processing of auditory stimuli, and that they may be selectively disrupted in brain-damaged subjects.

Characteristics and evolution of writing impairment in Alzheimer's disease.

Rapcsak et al. (Archs Neurol. 46, 65-67, 1989) proposed a hypothesis describing the evolution of agraphic impairments in dementia of the Alzheimer type (DAT): lexico-semantic disturbances at the beginning of the disease, impairments becoming more and more phonological as the dementia becomes more severe. Our study was conducted in an attempt to prove this hypothesis on the basis of an analysis of the changes observed in the agraphia impairment of patients with DAT. A writing test from dictation was proposed to 22 patients twice, with an interval of 9-12 months between the tests. The results show that within 1 year there was little change in the errors made by the patients in the writing test. The changes observed however were all found to develop within the same logical progression (as demonstrated by Correspondence Analysis). These findings made it possible to develop a general hypothesis indicating that the agraphic impairment evolves through three phases in patients with DAT. The first one is a phase of mild impairment (with a few possible phonologically plausible errors). In the second phase non-phonological spelling errors predominate, phonologically plausible errors are fewer and the errors mostly involve irregular words and non-words. The last phase involves more extreme disorders that affect all types of words. We observe many alterations due to impaired graphic motor capacity. This work would tend to confirm the hypothesis proposed by Rapcsak et al. concerning the development of agraphia, and would emphasize the importance of peripheral impairments, especially grapho-motor impairments which come in addition to the lexical and phonological impairments.

New questions on the hemispheric encoding/retrieval asymmetry (HERA) model assessed by divided visual-field tachistoscopy in normal subjects.

According to the hemispheric encoding/retrieval asymmetry (HERA) model, based on data obtained through functional neuroimaging, the left and right prefrontal cortices are preferentially, and, respectively, involved in long-term episodic memory encoding and retrieval. In this study, the HERA model was tested from a behavioral perspective using divided visual-field tachistoscopy. A recognition paradigm with both verbal and visuospatial materials was devised to differentiate memory-related effects (encoding vs. retrieval) from effects linked to the materials. The paradigm used lists of 12 and four items to assess long-term episodic memory and short-term memory, respectively. The aim of the latter condition was to test whether the HERA model is applicable in short-term memory. For long-term episodic memory, the data obtained validated the HERA model; the direction of the hemispheric asymmetry was found to depend on the type of materials used, whereas its magnitude was determined by the type of memory process. For verbal short-term memory, the HERA model seems to be confirmed. The pre-existing representations of the material could take into account the similarity of the hemispheric asymmetry pattern between short-term memory and long-term memory. In contrast, for visuospatial short-term memory, Baddeley's working memory model seems to better explain our results insofar as the asymmetries were essentially linked to the material in encoding but not in retrieval. This latter difference between short-term memory and long-term indicates that processes involved in LTM depend on episodic processes per se, hence, lending more support for the HERA model. Accordingly, these two memory systems seem to bring into play two different modes of hemisphere specialization.

Healthy aging, memory subsystems and regional cerebral oxygen consumption.

The present study was designed to search for concomitant age-related changes in memory subsystems, defined according to current structural theories, and resting oxygen consumption in selected brain regions. We have investigated a sample of subjects between 20 and 68 years of age and strictly screened for their good health. We applied in the same subjects a battery of neuropsychological tests selected to investigate several memory subsystems, and high-resolution positron imaging with stereotaxic localization to study a purposely limited number of cerebral structures, selected on a priori hypotheses to match the different memory subsystems. Our results showed significant age-related changes in performance on some tests, consistent with the literature, including an increase in semantic memory and a decrease in both working memory (central executive system) and verbal episodic and explicit memory. There was also an age-related linear decrease in global brain oxygen consumption which regionally reached statistical significance for the neocortical areas and the left thalamus. There was a limited number of significant, age-independent correlations between the raw psychometric test scores and resting regional oxidative metabolism. Consistent with our present understanding of the functional anatomy of memory, the Associate Learning scores (verbal episodic and explicit memory) were positively correlated with left hippocampal and thalamic metabolism. The positive relationships found between right hippocampal metabolism and performance in the Associate Learning and the Brown-Peterson tests were less expected but would be consistent with findings from recent PET activation studies. The results from this investigation are discussed in the light of current knowledge concerning the neuropsychology and the neurobiology of both aging and memory.

Topographic EEG activations during timbre and pitch discrimination tasks using musical sounds.

Successive auditory stimulation sequences were presented binaurally to 18 young normal volunteers. Five conditions were investigated: two reference tasks, assumed to involve passive listening to couples of musical sounds, and three discrimination tasks, one dealing with pitch, and two with timbre (either with or without the attack). A symmetrical montage of 16 EEG channels was recorded for each subject across the different conditions. Two quantitative parameters of EEG activity were compared among the different sequences within five distinct frequency bands. As compared to a rest (no stimulation) condition, both passive listening conditions led to changes in primary auditory cortex areas. Both discrimination tasks for pitch and timbre led to right hemisphere EEG changes, organized in two poles: an anterior one and a posterior one. After discussing the electrophysiological aspects of this work, these results are interpreted in terms of a network including the right temporal neocortex and the right frontal lobe to maintain the acoustical information in an auditory working memory necessary to carry out the discrimination task.

Inclusions in familial cytoplasmic body myopathy are stained by anti-dystrophin antibodies.

We report here for the first time positive anti-dystrophin labelling of inclusions in three cases belonging to the same family affected by familial cytoplasmic body myopathy (CBM). Inclusions are also stained, as reported previously, by anti-actin antibodies. The anti-desmin reaction was negative in the centre of cytoplasmic bodies (CB) but showed an enhancement of staining in the peripheral part. Abnormal sarcoplasmic staining of fibres with CB was also observed with that antibody. Anti-vimentin antibody labelling was negative. At present, the significance of this labelling by anti-dystrophin antibodies is unknown, but will open new fields for further investigations in an attempt to understand CB pathogenesis.

Plasma superoxide dismutase and glutathione peroxidase activity in sporadic amyotrophic lateral sclerosis.

To determine the possible role of oxydative stress in the pathology of amyotrophic lateral sclerosis (SALS), we measured the plasma activities of superoxide dismutase (SOD) and glutathione peroxidase (GPX), together with GPX and malone dialdehyde (MDA, a marker of lipoperoxydation) plasma concentrations in a sample of 21 SALS patients and 7 normal control (NC) subjects. MDA concentration and SOD activity were significantly higher, whereas GPX activity was significantly lower in SALS patients than in NC. Increased MDA concentration provides indirect confirmation of excess lipoperoxydation. Increased plasma SOD activity might reflect the involvement of extra-cellular SOD (SOD3), a hitherto unreported finding in SALS. Impaired GPX activity, which has already been found in red blood cells and brain tissue of SALS patients, might play a part in the pathogenesis of this disease.

Auditory agnosia with relative sparing of speech perception.

We report a case of auditory agnosia in which the initial clinical picture began with generalized auditory agnosia for verbal and non verbal sounds, but rapidly changed to a selective auditory agnosia confined to the perception of non verbal sounds. CT scanning and MRI did not demonstrate cortical or subcortical damage, except for bilateral ventricular enlargement. The patient was submitted to audiological investigations including physical and psychoacoustic studies. Deficits were revealed during the decay and loudness discrimination test, but no temporal auditory acuity deficit was observed. The results of these studies are discussed in relation to the clinical picture. Also the dissociation between verbal perception and non verbal perception is discussed.

Infarct of the anterior limb of the right internal capsule causing left motor neglect: case report and cerebral blood flow study.

A sixty-nine year old hypertensive man had left motor neglect following an infarct of the anterior limb of the right internal capsule. He also had left auditory extinction on verbal dichotic listening and a sligh constructional apraxia. Regional cerebral blood flow (CBF) was measured at rest with Xenon 133 inhalation and was found to be slightly decreased in a diffuse fashion. Motor activation of the right hand resulted in an increase of CBF in the contralateral superior rolandic area, whereas no such increase was found during motor activation of the left hand. This lack of cortical CBF increase on contralateral motor activation is interpreted as a consequence of the failure of some corticosubcortical connexions involved in motor arousal. The specifically dynamic appearance of regional CBF abnormalities, i.e. during selective activation as opposed to rest measurements, is consistent with the functional character of neglect.

Memory disorders in Alzheimer's disease and the organization of human memory.

The Squire and Zola-Morgan parallel organization model of the memory and the Tulving hierarchical model were developed mainly through the study of amnesic patients. The predictions of these two models are different, the first being more open to double dissociations and less restrictive than the second. Alzheimer's Disease is characterized by a differential impairment of the memory systems and by an interindividual variability which may take the form of dissociations between preserved and disturbed abilities in some patients. The objective of this study was to use the memory dysfunctions of patients with AD to test the validity of the two models. Analysis of the group data provided an average profile of memory disturbance consistent both with much of the data given in AD literature and with the two models. Using a multiple single-case strategy, we demonstrated several simple dissociations which are for the greater part compatible with the two models. Two of the dissociations underline the limits of the Tulving model, which otherwise accounts for a lot of results. The study supports the relevance of AD for the understanding of the cognitive architecture of the human memory.

Disorders of auditory identification in dementia of the Alzheimer type.

Fifteen patients with probable DAT and 18 matched controls were given tests that required the identification of verbal (phonemes and words) and non verbal (sounds and melodies) stimuli. In all tests, DAT patients made significantly more errors than controls. Errors predominated in non verbal tests in both groups. DAT patients (and, to a lesser degree, control subjects) made almost exclusively acoustic errors in word-identification, while errors in the identification of sounds and melodies could be either semantic or acoustic. Some categories of errors were observed predominantly in DAT patients. These results suggest that, in addition to their cognitive impairment, DAT patients have a specific deficiency of central auditory perception.

Agraphia in Alzheimer's disease: an independent lexical impairment.

This study was conducted to delineate the pattern of the writing impairments in 12 patients with Alzheimer type dementia. The patients performed writing tasks involving regular and irregular words and nonwords given by dictation as well as a decision test composed of printed words and pictures requiring phonologic, lexical, and semantic processing. Writing from dictation demonstrated a predominant, but nonisolated, lexical deficit. In order to better evaluate this lexical disorder, the correlation between the dictation writing scores and the decision task scores was analyzed. No significant correlation was found among scores for irregular words, phonologically plausible errors, and scores of the lexical or semantic decision tasks, but there was a significant correlation among scores for the nonwords, nonphonological spelling errors, and scores of the phonologic decision task using printed words. These results would suggest that the "lexical" deficit in agraphia, i.e., difficulty in retrieval or loss of the spelling representations of words, is independent of the lexical or semantic capacities involved in other modalities.

Regional cerebral blood flow during comprehension and speech (in cerebrally healthy subjects).

Regional cerebral blood flow (rCBF) was measured by the xenon-133 inhalation method in 10 cerebrally healthy subjects at rest and during linguistic activation tests. These consisted of a comprehension test (binaural listening to a narrative text) and a speech test (making sentences from a list of words presented orally at 30-s intervals). The comprehension task induced a moderate increase in the mean right CBF and in both inferior parietal areas, whereas the speech test resulted in a diffuse increase in the mean CBF of both hemispheres, predominating regionally in both inferior parietal, left operculary, and right upper motor and premotor areas. It is proposed that the activation pattern induced by linguistic stimulation depends on not only specific factors, such as syntactic and semantic aspects of language, but also the contents of the material proposed and the attention required by the test situation.

Proliferating cell nuclear antigen (PCNA) in cerebral cavernomas: an immunocytochemical study of 42 cases.

BACKGROUND: The natural history and growth mechanisms of cerebral cavernous angiomas are unclear, which makes them difficult to manage. We attempted to evaluate the evolutive potential of cavernomas by studying the proliferative capacity of cells. METHODS: We studied 42 histologically verified cavernomas with monoclonal antibody to proliferating cell nuclear antigen (PCNA), an accessory protein of the cell cycle, the rate of which is increased in proliferative cells. The PCNA Labeling Index (PCNA LI) was calculated in each case, and the results were compared with histologic findings (lacy areas, thick walls, thrombi, hemosiderin) and clinical features (epilepsy, hematomas, pseudotumorous signs). RESULTS: Thirty-six of 42 cases (85.7%) revealed stained cells. PCNA LI ranged from 1 to 48% (mean: 23.39%). Statistical analyses showed a positive correlation between PCNA LI and the extent of lacy areas (p < 0.05). On the contrary, collagenous-walled and thrombotic areas rarely showed positively stained cells. We found no relationship between PCNA LI and clinical features. CONCLUSIONS: A proliferative capacity of endothelial cells does exist in some areas of cavernomas and may explain, besides thromboses and hemhorrages, the growth and even de novo appearance of these lesions. Occurrence of fragile blood cavities, thickening of others, and changes in blood flow may influence the evolution of lesions. Our results suggest that in cavernomas, some areas may undergo specific changes, which makes them more dynamic lesions than previously thought.

[Cerebellar atrophy associated with lesions of the pallidum and luys' body and presence of lewy bodies. Relationships with degenerative disorders of the pallidum, substantia nigra and luys' body (author's transl)]. / Atrophie cérébelleuse et lésions pallido-luyso-nigriques avec corps de lewy. Rapports avec la dégénérescence pallido-luyso-nigrique.

Clinical and pathological findings in a case of hereditary degenerative disease of the nervous system are reported. The clinical picture associated cerebellar signs with an action hypertonia, which was the cause of severe hypertonic paroxysms in the terminal stages of the illness. Pathological lesions were found in the cerebellar cortex, the external part of the pallidum, Luys' body, and the substantia nigra which included Lewy bodies. The relationships of this case with atrophic lesions of the pallidum and Luys' body are discussed.

[Intracranial cavernous angioma]. / Cavernomes intracrâniens.

Cavernous angiomas are vascular malformations that belong to the group of hamartomas. Evidence to support the hypothesis that some intracranial cavernous angiomas are benign vascular tumors is lacking. As they are angiographically occult, cavernous angiomas were largely overlooked and were considered rare before CT scan and MRI became available. Both of these techniques provide a quick and accurate diagnosis in cases of epilepsy, brain hemorrhage or intracranial hypertension. Most of the hemispheric cavernous angiomas are situated subcortically, in the neighborhood of the rolandic fissure. In the brain stem, they can be found mostly in the pons. Cerebellum and spinal cord are seldom involved. Cavernous angiomas may occur in children, as well as in familial forms, or in association with other visceral locations. It is important to know that some cavernous angiomas are able to grow, owing to a variety of mechanisms, which may result in rupture and cerebral hemorrhage. The improvement of both imaging and surgical techniques have reduced the risks of death or heavy sequelae. Some deeply located cavernous angiomas, however, are still inaccessible to surgery.

[Neuropsychology of musical identification]. / Neuropsychologie de l'identification musicale.

Several neuropsychological observations have suggested that disturbances of the process involved in identifying music is related to lesions of the dominant cerebral hemisphere. The studies have been confirmed by different data from experimental psychology and functional brain imaging techniques. Based on these inference sources we propose different hypotheses to describe the cognitive processes involved in identifying music.

[Vascular dementia: clinical and radiological characteristics in 26 cases]. / Démences vasculaires: caractéristiques cliniques et radiologique dans 26 cas.

Twenty six patients with vascular dementia were studied. Five of them only, were referred for a cognitive decline and all the others because of focal neurological symptoms or a stroke. Twelve patients had a previous history of stroke and 21 an history of high blood pressure. Twenty three patients had gait disturbances. CT scanner and MRI showed a large proportion (23 patients) of lacunar or small sub-cortical strokes. Leuko-araiosis was present in 21 patients and cortical stroke only in 6. Finally, the lack of information concerning the onset and the course of the cognitive decline in 12 patients explain the difficulties to fulfill the criteria in some diagnostic evaluation scores particularly those of the DSM III R.

[Peripheral nerves in a case of lead neuropathy]. / Etude du nerf périphérique dans un cas de neuropathie saturnine.

A patient developed lead neuropathy with an asymmetrical distal weakness of the four limbs and sensory signs. Tap water contained high amounts of lead and biopsy confirmed the intoxication. The electromyogram was altered from the onset of the disorder and was still abnormal one year after treatment with EDTA, which however brought frank improvement. Biopsies were taken from nerves of the upper and lower limbs. Examination of teased nerve fibers showed the predominance of fibers of small diameter and segmental demyelinization with signs of remyelination in the lower limbs. Semi-thin sections demonstrated depopulation of large myelinated fibers, regeneration clusters and Schwann cells with globular nuclei. Electron microscopy showed myelin sheath alterations and neuritic lesions, some hyperactive Schwann cells. Main alterations were seen in basal membranes of Schwann cells of unmyelinated fibers and endoneuronal capillaries. Such alterations to our knowledge have never been reported in human peripheral lead neuropathy, but have been described after experimental intoxication. These findings together with recent experimental studies allow a discussion about the site of entry of lead into the nerve and about its site of action on the various constituents of the peripheral nerve.