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Biomedical subjects

L E Claveria

Publications and source records attributed to L E Claveria.

At least 19 recordsLinked to original sources

Fatal familial insomnia: clinical, neuropathological, and genetic description of a Spanish family.

The clinical presentation and evolution, neuropathological findings, and genotyping of three members of a Spanish family affected with fatal familial insomnia are reported. The mother and two of her offspring developed a rapidly evolving disease with insomnia and behavioural disorders as the initial symptoms and died between 5 and 10 months after the onset of the illness. Frontal brain biopsy in the mother disclosed only non-significant spongiosis, and full neuropathological examination of her offspring showed thalamic and olivary degeneration with isolated focal cortical spongiosis. Genetic examination could only be performed in the contemporary patients and both harboured the prion protein (PrP) 178Asn mutation and homozygous 129 Met/Met genotype.

Biopsy↗

Prevalence of age-associated memory impairment and dementia in a rural community.

To obtain accurate estimates of the prevalence of age-associated memory impairment, dementia, and Alzheimer's disease, a population study was carried out in Turégano, a rural community of 1011 inhabitants in the Segovia province of Spain. The study was divided into two phases: a door to door survey of the entire population aged 40 years and over (503 persons), followed by a clinical examination of suspected cases for positive and differential diagnosis of dementia and cognitive impairment. The prevalence of age-associated memory impairment was 3.6% in individuals of 40 years and over and 7.1% in individuals of 65 years and over, whereas dementia was found in 2.6% and 5.2%, respectively. The prevalence rates of both clinical conditions increased with age. The most prevalent clinical category of dementia was dementia of Alzheimer type, which represented 1.8% and 3.8% of these two age groups. The corresponding figures for vascular dementia were 0.4% and 0.9% and for secondary dementia 0.4% and 0.5%. Age-associated memory impairment is an age-dependent disorder with a high prevalence among the elderly; some of these patients may represent an early stage of Alzheimer's disease, suggesting that the prevalence of this disorder may be higher than previously estimated.

Adult↗

Q fever mimicking herpetic encephalitis.

We describe a patient with temporal lobe encephalitis associated with primary Coxiella burnetii infection who presented with CT and MRI findings suggestive of herpes simplex encephalitis and an initial improvement during treatment with acyclovir. Q fever should be considered in the differential diagnosis of patients whose manifestations suggest herpes encephalitis.

Coxiella burnetii↗

Intracranial infections: investigation by computerized axial tomography.

The value of computerized axial tomography (CAT) in the management of intracranial infections is described and the findings compared with those of other conventional neuroradiological investigations. The most important contribution is in the diagnosis and localization of cerebral abscess. CAT provides not only information of the nature of the lesion but also the number of loculi and the presence of multiple lesions; it is the investigation of choice for follow-up of patients with cerebral abscess treated either by operation or conservatively. CAT is invaluable in the investigation of other infectious conditions affecting the intracranial structures, especially when patients present with a focal neurological deficit and it is vital to exclude an abscess.

Adolescent↗

The use of computed axial tomography (CAT) for the diagnosis and management of intracranial angiomas.

The manifestations of angiomas on computed axial tomography (CAT) are described. Diagnostic changes were present in 66% and a focal abnormality, which was frequently suggestive of the diagnosis, in a further 27.5% of a consecutive series of 41 patients. It failed to show some focal abnormality in only two cases in which intravenous contrast was not given and in one case with a dural angiomatous malformation. The value of CAT in elucidating the nature of the complications of arteriovenous malformations is emphasized.

Adolescent↗

The encephalopathic action of five-carbon-atom fatty acids in the rabbit.

1. Five-carbon-atom organic acids (C-5 acids) have been administered intravenously to rabbits with ventriculocisternal perfusion and continuous electroencephalographic recording (EEG). The concentration of the acids in the cerebrospinal fluid (CSF) perfusate have been compared with changes in integrated low-frequency activity in the EEG. 2. The C-5 acids investigated were those accumulating in inborn errors of metabolism, i.e. isovaleric acid, beta-methylcrotonic acid, tiglic acid and alpha-keto- and alpha-hydroxy-isovaleric acid. There activity was compared with that of valeric acid. 3. Valeric acid and isovaleric acid produced coma and pronounced increase in slow-wave electrical activity and these changes paralleled the increase in concentration of the acids in the CSF perfusate. 4. The concentration of beta-methylcrotonic acid and tiglic acid in the CSF perfusate reached values comparable with valeric acid and isovaleric acid but showed less encephalopathic activity. An interaction between beta-methylcrotonic acid and isovaleric acid was observed. 5. Although the concentrations of alpha-ketoisovaleric acid and alpha-hydroxyisovaleric acid rose to the lesser extent in the CSF perfusate, changes in rousability of the animal and in the EEG recording were demonstrated. 6. It is concluded that all the C-5 acids tested have encephalopathic activity although this is lessened by the presence of either a double bond or an oxygenated functional group.

Animals↗

In vitro polarographic studies of rat brain oxygen uptake in simulated uraemia and hepatic coma.

A polarographic technique has been used to study the oxygen uptake of rat brain homogenates incubated with metabolites known to accumulate in uraemia and hepatic coma. In general, results with individual metabolites were in agreement with those previously obtained with brain slices using the Warburg technique. Inhibition was induced at higher concentrations with urea, creatine, indoxysulphate, guanidine, dimethylamine hydrochloride, ammonium chloride, taurocholate, glycodeoxycholate, sodium deoxycholate, skalote and indole. Serum and ultrafiltrate from drowsy uraemic patients also caused inhibition, and this was reversed by renal dialysis. Similarly serum and ultrafiltrate from patients in hepatic coma inhibited oxygen uptake but in this instance reversal of the inhibition was not demonstrated following exchange blood transfusion to the patients.

Adenosine Diphosphate↗

Penicillin transport from cerebrospinal fluid.

The passage of penicillin G from cerebrospinal fluid (CSF) was studied by continuous ventriculocisternal perfusion in conscious rabbits. The concentration of penicillin G in the perfusate, collected from the cisterna magna, was 76.5 percent +/- 1.0 (SEM) of that entering the ventricles (having adjusted for normal secretion of CSF). The mean concentration of penicillin G rose 15 percent (p less than 0.005) in the cisternal CSF after probenecid (2 mg per milliliter) was added to the perfusion fluid. We conclude that an active transport system removes penicillin G from the CSF; this mechanism can be inhibited by intraventricular probenecid. Our results are in accord with observations deriving from studies on anesthetized animals given probenecid intravenously or intraperitoneally.

Animals↗

Tardive dyskinesia treated with pimozide.

We have investigated the action of pimozide in tardive dyskinesia induced by prolonged administration of phenothiazines. Improvement was recorded in a double blind study of 18 patients treated with maximum tolerated dosage (mean 18.8 mg/day) for 6 weeks. There was no deterioration in the therapeutic action of pimozide over this time. Parkinsonism and sedation were the main adverse effects. They were corrected by reduction of the dose of pimozide, but often recurred so that further adjustments of dosage were necessary. Our findings support the view that tardive dyskinesia is produced by a disturbance in the balance of central transmitters such that dopaminergic transmission is increased.

Aged↗

Hypothermic action of bromocriptine.

The body temperature of rats has been studied in a cold environment. In this setting, bromocriptine induced hypothermia. This fall in core temperature was inhibited by pimozide. It was concluded that bromocriptine has a dopamine-like action in areas of the brain concerned with the control of body temperature, as has previously been shown for apomorphine and amphetamine.

Amphetamine↗

Bromocriptine in Parkinsonism.

Bromocriptine, a drug acting directly upon dopaminergic receptors, has been found to have a significant therapeutic action in a double-blind study of 20 patients with idiopathic Parkinsonism who were already receiving conventional therapy, including levodopa. Neurological deficits improved by almost 20% in severely disabled patients; amelioration of mildly affected patients was about 10%. Adverse reactions were similar to those encountered with levodopa-they were all dose-dependent and reversible. These observations are discussed in relation to certain theoretical advantages which might be expected from a drug which acts directly on dopaminergic receptors.

Aged↗

Amine metabolites in the cerebrospinal fluid of patients with disseminated sclerosis.

Mean homovanillic acid and 5-hydroxyindoleacetic acid concentrations were significantly low in the lumbar CSF of a group of patients with disseminated sclerosis (DS) who were severely disabled but not in the CSF of a less severely restricted group with DS. CSF concentrations were also low in a group of patients with superior cerebellar peduncular tremor due to DS and in a single patient with a similar tremor associated with a post-traumatic brain-stem lesion. The interpretation of abnormal CSF amine metabolite concentrations is discussed in the light of these findings.

Cerebellar Diseases↗