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Biomedical subjects

P Davous

Publications and source records attributed to P Davous.

53 records · Page 3Linked to original sources
PubMed1984

[Adrenomyeloneuropathy. 4 cases developing with the features of familial spasmodic paraplegia].

Four cases of adrenomyelopathy, the adult form of adrenoleukodystrophy, three of which were familial, were reported. The neurological disease consisted of progressive spasmodic paraplegia, sphincter disorders, peripheral neuropathy, occasional deep sensibility impairment and psychiatric disturbances in one case. Adrenal insufficiency was confirmed after the appearance of the neurological signs in two cases; in another case it had been diagnosed in childhood. Gonadal insufficiency was present in all cases and clinically apparent in two patients. Detailed genetic studies were carried out in two patients of the same family. Histocompatibility tests showed A2 and B15 antigens in three out of four cases. The diagnosis was confirmed in three cases by measurement of serum long chain fatty acid with increased C26 levels and C26/C22 ratios. Peripheral nerve biopsy in 1 patient showed characteristic inclusion bodies in the Schwann cells. The authors discuss the relationship between ALD and familial spasmodic paraplegia, the mode of genetic transmission of the disease and the diagnostic value of neuromuscular biopsy and measurement of serum long chain fatty acid levels.

Adrenoleukodystrophy↗
PubMed1983

[Alzheimer's type dementia: recent data].

Dementia of the Alzheimer type (DTA) is the most common form of adult-onset dementia. The clinical features of the disease include progressive memory defect, intellectual impairment and behavioral disturbances. The number of patients suffering from DTA is increasing dramatically, due to the growing proportion of old people. DTA therefore is a major public health problem. Brain lesions include several histopathological changes (mostly in the cerebral cortex and hippocampus): neurofibrillary tangles, senile plaques, granulo-vacuolar degeneration, Hirano bodies, angiopathy, loss of nerve cells. Postmortem brain examination reveals characteristic neurochemical deficits. The most consistent reduction in neurotransmitter-synthesizing enzyme observed so far is a reduction in choline acetyltransferase activity, suggesting a cholinergic deficit. However, other deficits involving noradrenaline and somatostatin have also been reported. Several hypotheses (genetic, viral, toxic, immunologic, metabolic) have been put forward in order to explain DTA. The relationship between these hypotheses and the neurochemical deficits is still unclear, but the existence of characteristic neurotransmitter-related deficits allows specific therapeutic trials.

Aged↗
PubMed1983

Menzel's hereditary ataxia with slow eye movements and myoclonus. A clinico-pathological study.

The patient we describe had cerebellar ataxia, slow eye movements, myoclonus, facial dystonia and signs of spinal cord and peripheral nerve involvement. The patient's mother, brother and sister died from the same disease. Neuropathological examination revealed lesions of olivo-ponto-cerebellar atrophy (OPCA) associated with spinal cord degenerative changes characteristic of Menzel's hereditary ataxia. Although myoclonus was similar to Hunt's dyssynergia cerebellaris myonica, pathological findings did not show significant involvement of the dentate nucleus or superior cerebellar peduncle and physiopathological hypotheses for myoclonus are discussed. Slow eye movement is emphasized in the propositus and we suggest that it could be specific of one type of OPCA. Its pathological significance is discussed, but a primitive and unique involvement of the paramedian pontine reticular formation is unlikely.

Adult↗
PubMed1983

Recurrent alternating ophthalmoplegia with iridoplegia in a young diabetic.

Regressive painless left third nerve paralysis with pupillary involvement was followed 6 months later by a right painful complete ophthalmoplegia. The two attacks resolved without treatment. The patient, 22 years old, was recognized diabetic and no other cause was found. It is suggested that this clinical pattern is characteristic of the involvement of two different territories of third nerve vascularization.

Adult↗
PubMed1982

[Spinal subdural hematoma. An unusual complication of lumbar puncture (author's transl)].

Spinal subdural hematoma following lumbar puncture occurred in a patient with embolic hemiplegia. The authors discuss the possible responsibility of heparin administered immediately after the tap. Physiological mechanisms of spinal subdural hematoma are reviewed. The authors emphasize the poor prognosis associated with this rare complication. They suggest that anticoagulant therapy be discontinued during and after a spinal tap. The first signs of medullary and radicular compression should be promptly recognized and an emergency neurosurgical procedure undertaken in order to forestall definitive paraplegia.

Female↗
PubMed1982

[Extrapyramidal rigidity with dystonia, optic atrophy and bilateral putaminal lesions in an adolescent. Juvenile form of Leigh's disease (author's transl)].

A 19-year-old man presented with an apparently non-familial neurological disorder that had progressed from the age of 6 years. Dystonia of the trunk and limbs with extrapyramidal rigidity, dysarthria, a pyramidal syndrome with spasticity of the lower limbs, bilateral optic atrophy, and nystagmiform ocular movements were present. CT scan demonstrated symmetrical putaminal lesions. The different aetiologies of bilateral striatal lesions are considered, the final diagnosis being a juvenile form of Leigh's disease.

Adult↗
PubMed1980

[Hemiplegia].

Explore the source record for details and available documents.

Adolescent↗
PubMed1978

Circulating immune complexes in multiple sclerosis. Relation to clinical and biological parameters.

Circulating immune complexes were looked for on 38 clinically definite MS (multiple sclerosis) patients compared to 35 other neurological patients and 26 healthy subjects. 29% of the MS sera and 8.6% of the other neurological sera were positive, whereas none of the control sera were positive. These differences are significant. Clinical status was analysed as regards the age at onset, the duration and course of the disease, the disability of the patients and the treatment they received. CSF (cerebrospinal fluid) parameters studied were pleocytosis, concentration of total protein, electrophoresis. The results suggest that IC (immune complex) are more frequent during the first 10 years of the MS disease, in patients with blood-brain barrier damage and in patients without oligoclonal IgG.

Adult↗
PubMedDate not supplied

[Apolipoprotein E and bleomycin hydrolase. Polymorphisms: association with neurodegenerative diseases].

Several studies indicate a possible association between different genes and chronic neurodegenerative diseases including Alzheimer's disease (DTA). To further investigate, we have analyzed association between the apolipoprotein E (apo E) and bleomycin hydrolase (BH) polymorphisms and three groups of elderly patients: control subjects (T) (n = 68), late-onset sporadic DTA patients (DTAst) (n = 65) and other non vascular neurodegerative diseases (MNDA) (n = 52). Apo E-epsilon4 and BH-G alleles frequencies (%) are: 8.2 (T), 31.5 (DTAst), 16.4 (MNDA) and 41.4 (T), 35.6 (DTAst). No association has been observed between carrying the G allele and DTA in epsilon4 negative subjects but, our data have confirmed the earlier reports: carrying the epsilon4 allele is a dose-dependent risk factor for the DTAst (OR: 6.0, IC 95 %: 2.6-13.7) and decrease the age of symptom onset (p < 0.005). They have also suggested that apo E genotyping may be of interest to perform differential diagnosis of neurodegenerative diseases in elderly subjects.

Aged↗
PubMedDate not supplied

[Neuropathologic validation of clinical diagnosis of senile dementia of the Alzheimer type].

The purpose of this work was to establish a neuropathological confirmation of the clinical diagnosis of senile dementia of the Alzheimer type (SDAT) in a group of patients prospectively studied in a geriatric hospital since 1984 (Charles Richet Study). The sample consisted of 45 cases, 35 of which had received a clinical diagnosis of SDAT and 10 others a diagnosis of either vascular or mixed dementia. The mean age at death was 85 +/- 6.9 years (range 64-97). The neuropathological diagnosis was established independently of the clinical findings. Senile lesions typical of SDAT were found in 32/35 cases, giving a 91.4% rate of clinico-pathologic agreement. However, vascular lesions were present in 10 cases (28.5%) and the final pathologic diagnosis was mixed dementia, lowering the score of agreement for SDAT to 63%. Considering that the pathological criteria for the diagnosis of SDAT are not uniform, we independently applied 3 inclusion (senile lesions) and 3 exclusion (vascular lesions) criteria for the diagnosis of SDAT to each of the 45 cases. This permitted a comparison of nine combinations of neuropathological criteria with the clinical diagnosis. The sensitivity values ranged from 37 to 80% and the specificity values ranged from 55 to 100%. The highest agreement rate with the clinical diagnosis was achieved when were associated the criterion which specified that plaques and tangles must be present in the hippocampus regardless of neocortical findings and the criterion which excluded cases with vascular lesions of any site if their volume was 50 ml or more.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMedDate not supplied

[Standardized neurologic study in senile dementia of Alzheimer's type].

Seventy five patients affected by senile dementia of the Alzheimer type (mean age 82) have been submitted to a standardized neurological examination of 88 items. Gait abnormalities were present in 57% of the cases. Extrapyramidal symptoms (akinesia or rigidity or tremor) were noticed in 64% of the cases but they were rarely associated with a typical parkinsonian syndrome. Myoclonus was observed in only 4 patients. The gait abnormalities were significantly associated with the presence of rigidity and grasp reflex but not with other primitive reflexes. The only symptoms to be correlated with dementia severity as assessed by the Mini Mental State were rigidity and optokinetic nystagmus abolition. Tremor and amyotrophy of the hands appeared to be negatively correlated to dementia severity.

Aged↗
PubMedDate not supplied

[Quantified neuropsychological study in senile dementia of Alzheimer's type].

Forty six patients affected by senile dementia of the Alzheimer type and 20 control subjects of similar age, sex and educational level have been studied, using a quantified neuropsychological battery (Wechsler memory scale, Raven progressive matrices and quantified tests for aphasia, apraxia and agnosia). There was a significant correlation between the scores of all cognitive functions with one another and with the Mini Mental State. The results show a rather global impairment of cognitive functions in these patients and suggest that intellectual impairment progresses as a continuum in senile dementia of the Alzheimer type.

Aged↗
PubMedDate not supplied

The Hoffmann reflex (H-reflex) in senile dementia of the Alzheimer's type--preliminary results.

The Hoffmann reflex (H-reflex) and direct motor response (M) were investigated (latency, amplitude and excitability curves were analyzed) in patients with senile dementia of the Alzheimer's type (SDAT). M responses had similar latencies in SDAT patients and old control subjects. H reflex latencies were similar in SDAT patients and old control subjects but longer than in younger controls. The H max/M max ratio was also lower in SDAT patients and old control subjects than in younger controls. The excitability curve of the H-reflex (using a double shock procedure) in SDAT patients was lower than in non-demented old controls for all values of the interstimulus intervals.

Aged↗
PubMedDate not supplied

[Adrenomyeloneuropathy: clinical, genetic and morphologic features. Apropos of 5 cases].

The authors describe five cases of adrenomyeloneuropathy, an adult form of adrenoleukodystrophy. Three of the cases were related. The clinical picture comprises progressive spasmodic paraplegia, sphincter disorders, peripheral neuropathy, sometimes hollow foot and posterior columns disorders. In one case psychic disorders were observed. Biochemical analyses confirmed or revealed adrenal insufficiency in four cases and gonadal insufficiency in three cases. A detailed genealogical study was made in two patients belonging to the same family. Histocompatibility tests revealed the presence of A2 and B15 antigens in three cases. Diagnosis was confirmed in three cases by plasma determination of long-chain fatty acids, with an increase in the level of C26 acids and the C26/C22 ratio; biopsy of the peripheral nerve in one patient revealed inclusions characteristic of Schwann's cells. Symptomatological links between ALD and familial spasmodic paraplegia, the mode of genetic transmission of the disease, the contribution of neuromuscular biopsy to the diagnosis and the therapeutic prospects of a diet low in long-chain fatty acids are discussed.

Adrenoleukodystrophy↗