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Biomedical subjects

H Carton

Publications and source records attributed to H Carton.

At least 91 records · Page 5Linked to original sources

HLA antigens and progression of multiple sclerosis. Part II.

The HLA-A, -B, -C and -D were determined in 200 multiple sclerosis (MS) patients, 64 of whom underwent a CSF examination. Their frequencies were correlated with the rate of disease progression and with factors thought to influence disease progression, including onset age, type of clinical course and intrathecal IgG synthesis. No correlation was found between HLA haplotypes and progression rate or type of disease course. Patients starting MS after age 31 years carried the DR2 determinant more frequently (P = 0.016) than patients with onset before this median age. Although the greatest proportion of patients with an IgG index greater than 1.5 or more than 15 oligoclonal bands in their CSF were DR2 carriers, no statistical difference was found in the intrathecal IgG synthesis of HLA-DR2 (+) and (-) patients.

Adolescent↗

Interferon production and natural killer (NK) activity in leukocyte cultures from multiple sclerosis patients.

Peripheral blood leukocyte (PBL) cultures from only 37% of MS patients produced detectable HuIFN-gamma in response to ConA as opposed to 85% of the cultures derived from normal blood donors. However, the yields in patient-derived cultures that were responsive, were not lower than those in cultures from controls. Production of HuIFN-alpha after stimulation with Sendai virus was not aberrant in cells taken from MS patients. The difference in HuIFN-gamma response rate between MS and normal donor-derived cells was more pronounced when DR2+ carriers were compared amongst each other than when DR2-k carriers were compared. Among the MS patients, the failure of PBLs to produce HuIFN-gamma in response to ConA was not correlated with age, sex, disease duration and type of disease. However, positive correlations were found with current disability indices and past disease progression rates. Unstimulated NK-activities of MS patient-derived PBLs were not different from those of normal donor-derived cells. the degree of augmentation of the activity by stimulation with ConA and interferon-alpha was also normal. Within the MS patients group, but not in the control group, there was a trend for DR2+ carriers to have lower spontaneous and stimulated NK-activities than DR2- individuals.

Adult↗

Success and failure of evoked potentials in detecting clinical and subclinical lesions in multiple sclerosis patients.

The value of visual, brain stem auditory and somatosensory potentials in detecting clinical and subclinical lesions as compared to the routine neurological, ophthalmological and vestibular examinations was investigated in 100 M.S. patients. It would appear that the VEP and SEP are far superior to the routine techniques in demonstrating lesions. On the other hand, the BAEP is inferior to the clinical and vestibular test as an indicator of brain stem lesions. All clinically manifest posterior column lesions are associated with abnormal SEP. However a substantial proportion of clinically evident lesions in the visual pathways or the midbrain and pons are not detectable by the VEP and BAEP.

Adolescent↗

On the role of calcium ions in the regulation of glycogenolysis in mouse brain cortical slices.

Using mouse brain cortical slices, we investigated the relative roles of cyclic AMP and of calcium ions as the intracellular messengers for the activation of glycogen phosphorylase (EC 2.4.1.1; alpha-1,4-glucan:orthophosphate glucosyltransferase) induced by noradrenaline and by depolarization. Activation of phosphorylase by 100 microM noradrenaline is mediated by beta-adrenergic receptors and does not require the copresence of adenosine. The role of the concomitant small increase in cyclic AMP is questioned. Short-term treatment with EGTA or LaCl3 abolishes the noradrenaline activation of phosphorylase, pointing to a critical role of extracellular calcium. Depolarization by 25 mM K+ or 100 microM veratridine produces a rapid and large (fourfold) activation of phosphorylase. Only veratridine increases the cyclic AMP levels; exogenous adenosine deaminase essentially blocks this cyclic AMP accumulation but not the phosphorylase activation. A half-maximal activation of phosphorylase occurs at about 12 mM K+. Addition of EGTA or LaCl3 reduces the effect of both depolarizations to a slight and transient activation of phosphorylase. These results indicate that activation of glycogen phosphorylase by K+ or veratridine occurs by a cyclic AMP-independent and calcium-dependent mechanism. The calcium dependency of brain phosphorylase kinase renders this kinase the prime target enzyme for regulation of glycogenolysis by calcium ions.

Animals↗

Progressive pontobulbar palsy with deafness: clinical and pathological study of two cases.

Among the hereditary affections of the nervous system associated with deafness, a rare condition called "progressive pontobulbar palsy with deafness" has been described. In this slowly progressive condition, hearing loss and vestibular are-flexia are almost always the first symptoms, occurring in late childhood or early adulthood. Only 18 cases-some sporadic, several familial-have been published without a full report of pathological findings. The clinical and pathological data of two new cases-one familial, one sporadic-are described here. There are differences from other forms of bulbar paralysis, lower motor neuron diseases, and some spinocerebellar hereditary affections. In view of the homochrony and homotypy in familial cases and the pathological findings, progressive pontobulbar palsy with deafness appears to be an abiotrophic process wih autosomal recessive inheritance.

Adolescent↗

Short-term intensive cyclophosphamide treatment in multiple sclerosis. A retrospective controlled study.

The effect of short-term intensive cyclophosphamide therapy upon 21 patients with moderately advanced multiple sclerosis is compared with a non-treated control group of 21 patients retrospectively matched for global disability. The progression of the disease was evaluated by regular disability scoring, and the results were analyzed by non-parametric statistical tests. No significant differences in the progression of the disability could be detected during a follow-up period of 2 years.

Adult↗

Tissue distribution of human interferons after exogenous administration in rabbits, monkeys, and mice.

Preparations of human leukocyte (alpha) and fibroblast (beta) interferon were given intramuscularly to rabbits and monkeys, and circulating interferon was measured. In rabbits, but not in monkeys, a marked difference between the two interferons was noted in that higher titers of circulating antiviral activity were obtained with leukocyte than with fibroblast interferon. In mice, injected interperitoneally, a similar difference could be noted. However, levels of antiviral activity in homogenates of spleens and lungs did not differ between mice injected with either interferon. Fibroblast interferon that was injected intrathecally in monkeys was found to diffuse throughout the cerebrospinal canal and to reach the serum compartment. Some interferon could also be recovered from the pia mater surrounding the brain hemispheres, but none was found in the deeper layers of the brain.

Animals↗

Adult ceroid-lipofuscinosis (Kufs' disease) in two brothers. Retinal and visceral storage in one; diagnostic muscle biopsy in the other.

Two brothers developed a neurological condition characterized by homochrony and homotypy: the first symptoms in both were generalized epileptic seizures, occurring at about the same age (30 years in the elder, 32 years in the younger), followed by a cerebellar syndrome with myoclonic jerks and some extrapyramidal symptoms. The elder of the two boys died at the age of 33 years. Histology showed extensive storage of ceroid-lipofuscin in the central nervous system (curvilinear bodies), in hepatocytes, in heart muscle and in the retina. In the younger boy, still living, a muscle biopsy (peroneal muscle) revealed accumulation of membrane-bound osmiophilic inclusions with curvilinear profiles. Retinal storage in Kufs' disease has never been documented. Muscle biopsy as a diagnostic tool for Kufs' disease has not been reported.

Adult↗

Clinical and ultrastructural study of a sporadic case of hereditary sensory neuropathy. Morphological evidence for a slow rate fiber degeneration.

A sporadic case of hereditary sensory neuropathy, with a clinical course and severe trophic and sensory alterations typical for type II, is presented. There was a severe loss of myelin in the sural nerve biopsy taken from the ankle. The most impressive microscopic feature was the number of rudimentary onion bulbs with an empty core which contained nude axons in the peripheral schwann cell layers; they were interpreted as vestigial structures left by the former myelinated fibers. Electron microscopy also revealed a definite involvement of unmyelinated fibers with attempted regeneration, which was confirmed by the overrepresentation of small axons on their frequency distribution curve. The whole ultrastructural picture suggested the protracted nature of the fiber involvement. This may be considered as agreeing with the slow course proposed for a system degeneration.

Adolescent↗

Percutaneous epidural dorsal cord stimulation in multiple sclerosis.

The effectiveness of percutaneous epidural stimulation has been evaluated in 11 patients with multiple sclerosis. Trial stimulation was performed at the thoracic level and lasted for 4 to 10 days. Evaluation was accomplished before the start and at regular intervals during and after the treatment. The scoring systems of the Standard Neurological Examination and of the Disability Status Scale of Kurtzke were used for evaluation of the neurological status. In addition, functionally useful changes were assessed by a physical therapist using a disability scoring technique designed at the National Multiple Sclerosis Centre at Melsbroek. Visual and somatosensory evoked potentials were also recorded at regular intervals. Six out of 11 patients claimed a subjective improvement following stimulation. But only four patients had better scoring results in the quantitative neurological testing, and for only two patients was this objective improvements functionally useful.

Activities of Daily Living↗

Biochemical findings in multiple sclerosis. V. Transformation of lymphocytes from patients with multiple sclerosis by human basic protein.

Lymphocyte stimulation tests with human basic protein of myelin were performed on patients with multiple sclerosis, with other neurological diseases and on normal subjects. In both MS and OND group, a hypersensitization to basic protein was seen in about one third of the cases. All normal subjects, except one, had negative responses. In the MS group, a positive correlation could be found with some features of the disease: significantly more positive responses were found in independent patients with a short duration of illness and in those with an oligoclonal distribution in the CSF. The authors compare their results with those of the literature. The possible role of BP in pathogeny of MS and OND is discussed.

Histocytochemistry↗

Benign intracranial hypertension due to A-hypervitaminosis in adults and adolescents.

Three new cases of chronic vitamin A intoxication are reported and a review of the literature with special reference to chronic intoxication in adolescents and adults is presented. The most prominent features are intracranial hypertension, skin and hair deviations, pain in the musculoskeletal system, and fatigue. Intracranial hypertension occurs in 50% of chronic intoxications, but is not invariably linked with the other symptoms. Young women are the major age group represented. There seems to be no relation between the severity of the clinical picture and the vitamin A serum level. Discontinuance of vitamin A intake is sufficient for cure.

Acne Vulgaris↗