[Specialized consultation of memory disorders or multidisciplinary consultation?].
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Humans↗
Biomedical subjects
F Pasquier
Publications and source records attributed to F Pasquier.
[Huntington disease. Current state of research].
Huntington's disease is a hereditary disease with autosomal dominant transmission which generally occurs in adults. The gene was discovered in 1983 and the genetic abnormality in March 1993. The most commonly recognized clinical manifestation is choreiform movements although other signs often appear more invalidating to family and friends. Cognitive decline, modifications in behaviour, and sometimes psychiatric disturbances are perceived as the major handicap in everyday life. In this review, emphasis has been placed on late onset forms of the disease. Recent research has focused on morphologic and functional imagery, the neuropathologic stages of selective neurone loss beginning in the striatum, and the role of excitotoxic amino-acids. Certain ethical considerations must be addressed when determining prognosis. Predictive tests and follow-up must be prepared and conducted scrupulously and proposed to voluntary, informed, major subjects at risk. Tests should be performed by a qualified laboratory working on anonymous samples, independent of the clinical team, and should be given to the subject orally by a genetic counsellor. A prenatal test may be requested by parents at risk. The question of treatment, which to date can only offer symptomatic relief, should be re-addressed in light of the recent discovery of the mutation published in March 1993.
Adolescent↗
Motor memory and the preselection effect in Huntington's and Parkinson's disease.
Patients with Huntington's disease (HD) and Parkinson's disease (PD) show different patterns of preserved and impaired memory performance. This study investigates explicit memory for movements in HD and PD with a linear positioning apparatus using Dick et al.'s procedure (J. Gerontol. 43, 127-135, 1988). In the first experiment, 12 HD patients were compared to 12 matched-controls. HD patients were more impaired than the controls by the delay between criterion and recall movements, whether the delay was filled or unfilled. Switching the limb between criterion and recall movements did not lead to more effects in HD patients and in controls. In the second experiment, 12 non-demented PD patients were compared to matched-controls. PD patients were more impaired than controls when the recall movement was executed with the contralateral hand, but were not more affected by the delay. In both experiments, HD and PD patients, as well as the controls, recalled self-generated preselected movements better than imposed movements. These results suggest the existence of distinct forms of motor memory impairment in some subcortical neurodegenerative diseases.
Adult↗
Methylenetetrahydrofolate reductase deficiency revealed by a neuropathy in a psychotic adult.
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Female↗
SPECT data in a case of secondary Capgras delusion.
Capgras' delusion (CD) may be secondary to a neurologic lesion, particularly in the right frontal or occipital regions. A 40-year-old woman with multiple sclerosis underwent SPECT during and after an episode of CD. Analysis during delusion showed an uptake defect in the right parietal cortex with an 11% index of asymmetry (normal: < or = 4%). Post-delusion SPECT showed decreased bifrontal and biparietal cortical uptake indices but a normal index of asymmetry. Functional brain imaging may provide clues to the psychopathology of CD.
Adult↗
Behavioral effects of trazodone in Alzheimer's disease.
BACKGROUND: Central serotonin depletion may contribute to the anxiety, restlessness, irritability, and affective disturbance seen in a variety of psychiatric conditions, particularly dementia of the Alzheimer's type (DAT) in which brain concentrations of both 5-hydroxytryptamine (5-HT) and its 5-hydroxyindoleacetic acid (5-HIAA) metabolite are reduced. METHOD: Trazodone, a serotonergic antidepressant with alpha 2-adrenergic blocking activity, was administered to 13 patients with DAT in an open 10-week pilot study at a dose of 25 mg t.i.d. Behavioral and affective disturbance was assessed pretreatment and posttreatment using semistructured interview and Jouvent's Depressed Mood and Gottfries-Brane-Steen scales. RESULTS: Irritability, anxiety, restlessness, and affective disturbance were all decreased (p < .05). No side effects were observed. Mean Mini Mental State scores were unaffected by treatment. CONCLUSION: The hypothesis that trazodone corrects behavioral and affective disturbance induced by serotonin depletion in DAT requires confirmation in a double-blind placebo-controlled trial.
Affective Symptoms↗
Euphoria with buspirone after fluoxetine treatment.
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Aged↗
[Cerebellar syndrome after carbon monoxide poisoning. Magnetic resonance imaging and single photon emission tomography].
A 19-year-old woman presented with severe carbon monoxide poisoning resulting in coma, brain stem signs, cerebellar syndrome, anterograde memory disorder and some frontal signs. Nine years later, generalized seizures appeared. At the age of 31, the cerebellar syndrome and memory disorders persisted. MRI showed cerebellar and internal temporal atrophy with high-intensity signals, and hippocampal and callosal atrophy. SPECT (Xe133) showed a low cerebellar blood flow.
Adult↗
[Adrenoleukomyeloneuropathy in an adult].
We report a case of adrenomyeloneuropathy in a 35-year-old man. He experienced acute adrenal insufficiency at 29 years of age, followed by psychic disorders and mental deterioration, then spastic paraparesia, dysarthria and peripheral neuropathy. MRI showed high-intensity signal areas in the cerebellum and periventricular white matter. Clinical symptoms, neurophysiological and neuroradiological examinations disclosed both features of adrenoleukodystrophy and adrenomyeloneuropathy. This indicates that adrenoleukodystrophy and adrenomyeloneuropathy probably represent two portions of the same entity.
Adrenal Insufficiency↗
[Infarction of the red nucleus and crossed cerebellar diaschisis].
We report a case of rubral infarct involving adjacent ipsilateral thalamic structures in a 54-year-old man with hypertension and coronary heart disease. Clinical picture at onset was suggestive of Weber's syndrome leading to a Claude's syndrome with vertical gaze palsy and sleep disturbances. Single photon emission computed tomography using Hm-PaO-Tc revealed a crossed cerebellar diaschisis. This is, to our knowledge, the first case of such crossed cerebellar diaschisis in a patient with rubral infarction.
Cerebellar Diseases↗
Some clinical aspects of late onset parkinsonism.
Thirty-six parkinsonian patients were selected by age of onset of over 70 and a minimum of five years duration of illness. The mean age of onset was 73.5 years and 30 patients were still alive after a mean of 7.2 years. We found that late onset Parkinson's disease has a relatively benign course with more "axial symptoms" especially dysarthria, freezing and postural instability. Dyskinesias and fluctuations are rare and dementia occurs in few patients in spite of their old age.
Aged↗
Meningitis caused by Salmonella enteritidis in an adult.
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Aged↗
[Stiff-Man syndrome with late onset].
We report a case of Stiff-Man syndrome according to Gordon, Januszko and Kaufman's criteria. Onset at age 76, association with insomnia and a rapid course leading to death within 2 years were the characteristic features. CSF data, electromyographic and immunological findings suggest abnormalities of catecholaminergic and GABA ergic systems, with release of segmental or suprasegmental inhibitory influence. The presence of antibodies against glutamic acid decarboxylase, considered a useful marker of this syndrome, raises the possibility of an autoimmune pathogenesis.
Aged↗
[Neuropsychological evaluation before and after thalamic stimulation in 9 patients with Parkinson disease].
Chronic thalamic-VIM stimulation was performed in 9 parkinsonian patients with disabling tremor and poor response to drugs. Neuropsychological assessment was performed before and after deep brain electrode implantation and stimulation. Mild cognitive disorders were observed prior to thalamic implantation. Neuropsychological testing failed to show intellectual function worsening after implantation and stimulation. We conclude that thalamic stimulation could be an appropriate treatment of untractable tremor as this could provide less neuropsychological side-effects than thalamotomy, especially in Parkinson's disease.
Aged↗
[Mental akinesia and memory disorders following carbon monoxide poisoning].
A 32-year old woman presented with serious memory impairment and a mental syndrome named loss of psychic auto-activation or psychic akinesia following carbon monoxide poisoning. The MRI findings were bilateral pallidal lesions probably associated with thalamic lesions. The evolution was favourable.
Adult↗
[Right unilateral auditory agnosia following left lenticular hemorrhage].
A 33-year old patient who had had left lenticular hemorrhage presented with an inability to understand with the right ear oral language and, in a less dramatic way, nonverbal sounds. This unilateral auditory agnosia was first associated with a right motor underutilization and right motor, sensitive, visual and auditive extinctions. Speech discrimination scores were 100% with the left ear and 15% with the right ear, even less in dichotic conditions. Tonal audiogram, as well as early and late components of the auditory evoked potentials were normal. Cerebral regional perfusion and metabolism were impaired over the left parietotemporal area. There was severe hypoactivation of the left hemisphere with right monaural verbal stimulations. Rehabilitation consisting of non-specific attention tasks and repetitions of words reaching only the right ear was undertaken 15 months after the stroke. The oral language comprehension improved, as did the left hemisphere activation, and the extinction phenomena disappeared, except for the auditory one. The unilaterality of the auditory agnosia could be due, in part, to a peculiar physiological processing in this patient, such as poor performance of his right ipsilateral auditory pathway which could be improved with practice. A striatal lesion could induce a spatial hemi-inattention as reflected by the multimodal extinction in this case. Besides, a lack of selective activation for verbal stimulation of the left hemisphere is suggested.
Adult↗
[Fulminating cryptococcal meningoencephalitis. An anatomo-clinical case].
We report a case of cryptococcal meningoencephalitis in a 52 year-old man. The patient had complained of headache and drowsiness for a few hours before he became comatose with a bilateral sixth cranial nerve palsy. The cerebrospinal fluid contained less than one lymphocyte per cubic millimeter, a low glucose level (0.20 g/l) and numerous Cryptococcus neoformans encapsulated yeasts. A neuropathological study showed that the cortex and midbrain were involved. To our knowledge, such an acute case with death within the first 20 hours has not yet been reported.
Brain↗