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Biomedical subjects

F Lhermitte

Publications and source records attributed to F Lhermitte.

At least 37 records · Page 2Linked to original sources

The clinical spectrum of alcoholic pellagra encephalopathy. A retrospective analysis of 22 cases studied pathologically.

A retrospective clinical study of 22 heavy alcohol drinkers is reported in which postmortem study showed diffuse chromatolysis of neurons identical to that found in neurological pellagra, associated in 13 cases with Marchiafava-Bignami disease and/or Wernicke-Korsakoff disease. The clinical features included confusion and/or clouding of consciousness, marked oppositional hypertonus ('gegenhalten') and myoclonus. Because of the frequent coexistence of other alcoholic encephalopathies in the same patient, alcoholic pellagra was often unrecognized. Fifteen patients received thiamine and pyridoxine therapy without niacin. It appeared to aggravate the neurological state or to trigger the development of alcoholic pellagra encephalopathy in 9 cases. The relationship between pellagra occurring during thiamine and pyridoxine therapy and 'nicotinic acid deficiency' is discussed. Multiple vitamin therapy should be given in the treatment of undiagnosed encephalopathies in alcoholic patients.

Adult↗

Immunological determination of galactosylceramide level in blood as a serum index of active demyelination.

An enzyme-linked immunosorbent assay (ELISA) to determine the level of galactosylceramide (GalC) in biological fluids is described. The assay uses GalC-coated plastic microtiter plates, with binding of an antibody to GalC detected by a peroxidase-labeled second antibody. The GalC level was directly estimated in the biological samples, without prior extraction, by competition with the coated hapten. This method allows the detection of 62 pmol of GalC (1.2 nmol/ml). Results using this procedure revealed positive sera only among patients suffering a myelin-destructive process: either primary, as in multiple sclerosis, or secondary to brain damage, as during ischemic strokes.

Cerebrosides↗

[Benign intracranial hypertension and minocycline].

A 19 year-old woman complained of headache and nausea occurring while she was taking minocycline for acne. Examination showed bilateral papilloedema and a bilateral VIth nerve palsy. Symptoms and signs rapidly resolved after the drug was stopped. Benign intracranial hypertension due to tetracyclines is well known in infants. It is rare in adults. Its pathophysiology remains unknown. The role of vitamin A is inconsistent. Others biological factors or personal susceptibility could be involved.

Acne Vulgaris↗

Alexia without agraphia in a left-handed patient with a right occipital lesion.

A left-handed patient who had suffered an ischemic vascular accident in the territory of the right posterior cerebral artery displayed alexia without agraphia. Clinical examination of this alexia revealed semiological characteristics comparable with those observed in cases of left temporo-occipital lesions in right-handed patients. Analysis of these disturbances indicated that they were agnosic in nature. The associated disturbances included problems of visual representation and topographic memory together with an amnesic syndrome.

Aged↗

Cholinergic-dependent cognitive deficits in Parkinson's disease.

In a double-blind cross-over study, the effects of a subthreshold dose of scopolamine (0.25 mg) on memory were compared in 32 control subjects and 32 parkinsonian patients who were without any sign of intellectual and mnemic impairment. Although the scores of the controls in the memory test battery showed no deterioration after the administration of scopolamine, the same dose resulted in significantly reduced memory performance in parkinsonian patients in two tests which involved the recognition of meaningless drawings. The selective vulnerability of parkinsonian subjects without cognitive impairment to a subthreshold dose of scopolamine suggests the existence of an underlying alteration of central cholinergic transmission. The neuropsychological findings in our study agree with postmortem biochemical data, which showed decreased cortical choline acetyltransferase activity in all parkinsonian patients, suggesting the existence of neuronal compensation in parkinsonian patients who are without cognitive impairment.

Acetylcholine↗

Does long-term aggravation of Parkinson's disease result from nondopaminergic lesions?

The motor score with and without levodopa was estimated in 193 parkinsonian patients with variable length of evolution. The effect of levodopa on akinesia, rigidity, and tremor remained quite stable during the course of the disease. In contrast, the aggravation of gait disorder, postural instability, and dysarthria was more severe, with decreased percentage of improvement on levodopa in patients with longer evolution. It is suggested that aggravation of Parkinson's disease mainly results from increasing severity of cerebral nondopaminergic lesions.

Brain↗

[Treatment of progressive and severe forms of multiple sclerosis using a combination of antilymphocyte serum, azathioprine and prednisone. Clinical and biological results. Comparison with a control group treated with azathioprine and prednisone only. 4-year follow-up].

45 patients were treated by combined application of antilymphocyte serum, azathioprine and prednisone: a control group of such 22 patients was besides treated by azathioprine and prednisone only. The treatment by antilymphocyte serum included a four weeks initial treatment, and one year continuous treatment one infusion per week. Azathioprine and prednisone were given every day during the same time. Azathioprine only was given during the three following year. The two groups were followed up during four years. This treatment was well accepted by patients under strict technical survey conditions. Serum sickness was noted in 11 p. cent of the cases. The equine antiglobulin antibody titre, increased during the initial treatment, was connected with serum sickness or, when the treatment was continued, with the inefficacy of the treatment. Rosettes titre decreased in ten cases, no clinical correlation was possible. The complement, immune complexes, were normal before treatment with most of the observed patients. The delayed hypersensitivity skin tests were negative after a month in 80 p. cent of the cases. The cerebrospinal fluid analysis, for the elements as well as for the rate of proteins and gammaglobulins, showed no significant variations before and after the treatment. The comparison with the control group showed a significant statistical difference as too the number of aggravated and improved patients after the first year. When grouping together stable and improved patients, the difference was statistically significant after the third year. The difference was not significant in years "two" or four. No difference in the frequency of the relapses could be noted, in the first year or the following three years. In spite of inconsistent results comparison showed favorable data after three years. The good results seemed due to the additional use of antilymphocyte serum. Such a treatment associating antilymphocyte serum to azathioprine and prednisone is beneficial in evolutive, remittent and recent multiple sclerosis. Through the length of the treatment remains difficult to determine, we conclude that it is really beneficial for such patients.

Antilymphocyte Serum↗

Disappearance of parkinsonian signs after spontaneous vascular 'thalamotomy'.

Disappearance of parkinsonian symptoms was observed in a 57-year-old patient with Parkinson's disease after a contralateral thalamopeduncular infarct. From analysis of stereotaxic data in parkinsonian patients, this may be explained by lesion of the ventro-oral internal nucleus or of Forel's field, both of which are involved in thalamopeduncular infarcts.

Female↗

Cognitive function in recent-onset demyelinating diseases.

To determine cognitive disturbances in recent demyelinating disease, we studied 21 patients with definite or probable multiple sclerosis (MS) of less than two years' duration and nine patients with recently isolated optic neuritis. None had any clinical or social evidence of cognitive impairment. Mild to moderate cognitive impairment. Mild to moderate cognitive impairment was present in 18 (60%) of 30 cases, affecting visual and/or verbal efficiency. These abnormalities were statistically significant when compared with the results of a control group of 29 patients. There was no correlation with a depressive status, between the presence of cognitive impairment and either the degree of handicap or the activity of the disease. The frequency of cognitive dysfunction (60%) appears to be comparable to that reported in other series in which MS evolution is over ten years. The natural history of cognitive functions in MS has to be identified. Neuropsychologic tests could be useful in the diagnosis of monosymptomatic or paucisymptomatic forms of MS (ie, visual or medullary).

Adult↗

Human autonomy and the frontal lobes. Part I: Imitation and utilization behavior: a neuropsychological study of 75 patients.

A type of pathological behavior, imitation behavior (IB), is newly described. In this behavior patients imitate the examiner's gestures, although not instructed to do so. Patients explain that they thought they had to imitate the examiner. IB is the first stage of utilization behavior (UB). Neuropsychological examination of 40 patients with IB, of 35 with UB, and of 50 disease controls demonstrates the existence of a frontal syndrome and two determining features of such behavior: dependence on (1) the social and (2) the physical environments. Loss of intellectual control was also required for the occurrence of such behavior. UB and/or IB were present in 96% of the 29 patients with focal lesions of the frontal lobes. Computed tomographic scans in 26 of these patients showed involvement of the inferior half of the anterior part of one or both frontal lobes. IB and UB are interpreted as release of parietal lobe activities, resulting from impairment of frontal lobe inhibition.

Behavior↗

Human autonomy and the frontal lobes. Part II: Patient behavior in complex and social situations: the "environmental dependency syndrome".

Imitation and utilization behavior have previously been described in terms of a simple interaction between an examiner and a patient, and were interpreted as an excessive dependence on environmental cues. In this study, patient dependence was observed in complex situations of everyday life. Two patients with focal unilateral frontal lobe lesions were observed while in a doctor's office, a lecture room, a car, and a garden, while visiting an apartment where various activities were possible, and while in a gift shop. The patients' behavior was striking, as though implicit in the environment was an order to respond to the situation in which they found themselves. The term environmental dependency syndrome is proposed for this condition. It implies a disorder in personal autonomy. Individual psychological traits influenced the way in which loss of autonomy was manifested. This study does not offer a physiological model of autonomy, but it does provide clinical and behavioral observations on the loss of autonomy secondary to unilateral lesions of the frontal lobe.

Brain Diseases↗

Early onset multiple sclerosis. Clinical study of 12 pathologically proven cases.

Among 70 pathologically proven MS cases, we found 12 cases with onset before 25. Only females were affected. There was no positive familial history of MS. In every case the diagnosis was considered during life. Mean duration of the disease was 29.1 +/- 12.5 years. This was significantly longer (p less than 0.001) than in the control group (58 pathologically proven cases with onset after 25). There were no acute courses. Progressive courses (1 case) and remittent courses (5 cases) were less frequent than in the control group (p less than 0.001 and p less than 0.01, respectively). The frequency of remittent-progressive courses (6 cases) was similar in both groups. The length of remittent-progressive courses (30.2 +/- 16.2) and remittent courses (28.6 +/- 9.7) was longer than that of control group (p less than 0.001). The symptomatology of onset was similar in both groups and had no predictive value for prognosis. The overall frequency of signs and symptoms was not statistically different in both groups. For each sign, the percentage of affected patients, throughout the course of the disease, was always smaller in early onset MS than in control group, except for optic neuritis which was as frequent in both groups. However, this was statistically significant only for ocular motricity and nystagmus.

Adult↗

Nerve and muscle microvasculitis in peripheral neuropathy: a remote effect of cancer?

In a series of 50 cases in which nerve and/or muscle microvasculitis was seen on biopsy, seven were associated with malignancy. In two cases, the cancer was found after the discovery of microvasculitis. All patients exhibited sensory-motor neuropathy, which was often painful and asymmetrical, with a progressive course. ESR and CSF protein levels were always elevated. Motor conduction velocity was slightly reduced in three cases, unmeasurable in one case, and normal in three. Cancers involved were adenocarcinoma in five cases (three prostate and two lung), Hodgkin's disease in one and immunoblastic lymphadenopathy in one. A thorough search for cancer should be performed when microvasculitis is seen in nerve or muscle biopsy specimens, especially when ESR and CSF protein levels are elevated.

Adenocarcinoma↗

Heterogeneity of cognitive impairment in progressive supranuclear palsy, Parkinson's disease, and Alzheimer's disease.

Patterns of cognitive and behavioral impairment were analyzed in patients with progressive supranuclear palsy (PSP), Parkinson's disease (PD), and senile dementia of Alzheimer's type (SDAT), matched for age, sex, manual laterality, educational level, and degree of intellectual deterioration. The scores of the three groups of patients were significantly lower than those of controls and were comparable on tests of verbal and visuospatial functions as well as global memory. Patients with SDAT could be distinguished by the severity of verbal memory disorders; patients with PSP, and to a lesser degree those with PD, by impaired performances on tests sensitive to frontal lobe dysfunction.

Aged↗