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Biomedical subjects

E Hirsch

Publications and source records attributed to E Hirsch.

At least 199 records · Page 11Linked to original sources

Characterization of two antigens in parkinsonian Lewy bodies.

Two antigens, G7 and G9, which are labelled by monoclonal antibodies in Lewy bodies in brains from patients with Parkinson's disease, were characterized by two-dimensional electrophoresis, followed by electroblotting, in order to explore their possible relationship with neuronal degeneration in this disease. The G7 antigen was found in the substantia nigra of subjects with Parkinson's disease and progressive supranuclear palsy, as well as in normal subjects. It was also found in the dopaminergic nucleus paranigralis and the locus coeruleus. The G9 antigen was found in the substantia nigra and locus coeruleus, but also in the caudate nucleus, terminal region of the nigrostriatal dopaminergic neurons, and in the cortex and cerebellum, terminal regions of the noradrenergic neurons in the locus coeruleus. The identity of the antigens remains unknown. They do not correspond to tyrosine hydroxylase or neurofilaments previously detected in Lewy bodies, or to other cytoskeletal proteins. Nor are they related to the presence of neuromelanin in the cells that degenerate in Parkinson's disease. The proteins, or at least the epitopes labelled by the antibodies, are found in normal brain, suggesting that these proteins do not play a causal role in the formation of the Lewy bodies in degenerating neurons in Parkinson's disease. The G7 antigen is absent from the cholinergic substantia innominata, where Lewy bodies are also found, indicating that these antigens are not essential for the formation of the corpuscle.

Antigens↗

[Epidemiological research on primary hyperparathyroidism. A prospective multicenter study].

Serum calcium determination of 11,588 hospitalized patients during a twelve-month period revealed hypercalcaemia in 74 (0.64%). Further clinical and biochemical investigation established primary hyperparathyroidism in 20 of them (27%), and in 15 (20%) a malignant tumour (with bone metastases in six) as the cause of the hypercalcaemia. Rarer causes were found in 11 patients (15%): diuretic medication (5), lithium treatment (3), immobilization (2) or hyperthyroidism (1). In the remaining 28 patients (38%) no cause of the hypercalcaemia could be established with certainty. In at least six patients, however, there were clinical pointers towards hyperparathyroidism in the absence of unequivocal biochemical findings. Leaving out of account borderline cases, one must reckon on a prevalence of hyperparathyroidism in 0.17% of an unselected group of hospitalized patients. Parathyroid hyperfunction must always be considered in the differential diagnosis because of its manifold clinical presentation.

Austria↗

[Role of the electrophysiologic examination in the diagnosis of Bassen-Kornzweig syndrome].

An autosomal recessive disorder, abetalipoproteinemia or Bassen-Kornzweig disease, concerning two sisters are described. This disorder, clinically similar to Friedreich ataxia, should be examined by electrophysiological and laboratory procedures because of the possibility of treatment by high doses of vitamin A and E. The routine electrophysiological examination of the two sisters revealed a degenerative spinocerebellar and peripheral nervous process which confirmed the damage of large myelinated fibers, as reported in the literature: neurogenic muscular atrophy of distal muscles, polyphasic motor unit potentials, moderately decrease of lower motor and sensory nerve conduction rates, and reduced amplitude of evoked responses in sensory nerves and muscles. We stress out the diagnostic value of the heterogenous conduction decrease in the distal motor fibers, signs of processes of demyelination or distal regeneration.

Abetalipoproteinemia↗

Brain abscesses in neonates. A study of 30 cases.

Since the introduction of ultrasonography and computerized tomography (CT) scanning, brain abscesses are found more frequently in cases of neonatal meningitis and septicemia, particularly when the offending pathogen is Proteus. Thirty cases of brain abscess in neonates are reported, 27 of which were caused by Proteus species infections. Twenty infants had meningitis and 13 had septicemia. Most of the abscesses were enormous, and multiple abscesses were observed in 17 cases. The frontal region was involved in 22 cases (12 unilaterally and 10 bilaterally). The ventricles were enlarged on the first CT scan in 13 cases. The abscesses were treated by aspiration and antibiotics in 25 cases, and by antibiotics alone in five. A shunt for hydrocephalus was necessary in 14 infants. Four infants died, three from the initial illness and one from a shunt complication. Sixteen children have seizures. Subsequent intelligence quotient (IQ) testing was performed in 22 children: eight (36%) have an IQ at or above 80 and eight have an IQ of less than 60. In the 17 children followed for more than 2 years, the proportion with an IQ at or above 80 fell to 24% (four cases). The absence of initial seizures, sterile cerebrospinal fluid, normal ventricles on CT scans, and early aspiration of the abscess seem to be factors portending a better prognosis in terms of epilepsy and mental sequelae.

Anti-Bacterial Agents↗

Motor neuron disease, parkinsonism and dementia. Report of a case with diffuse Lewy body-like intracytoplasmic inclusions.

The clinicopathological findings in a 49-year-old man who presented multisystemic neurological degenerative disease are reported. The patient presented, at the age of 36, distal upper limb amyotrophy and 8 years later pyramidal signs with fasciculations. In his last year of life, he suffered extrapyramidal hypertonus and mental deterioration. Neuropathological examination showed anterior spinal root and cerebral atrophy. Myelin pallor was mild and predominated on posterior cords. Anterior horn neuronal loss was noted in the spinal cord as well as Alzheimer-type changes in the brain. Inclusion bodies consistent with Lewy bodies were diffusely apparent. The peculiar clinical progression in this case and the extension of neuropathological lesions with inclusion bodies mainly in the substantia nigra and cerebral cortex are an interesting subject of discussion.

Brain↗

Brain somatostatin concentrations do not decrease in progressive supranuclear palsy.

The concentrations of somatostatin and choline acetyl transferase (CAT) were measured in nine brains from patients with progressive supranuclear palsy (PSP) and compared with those obtained from 19 matched control brains. In PSP, CAT activity was reduced in the caudate nucleus and limbic areas (amygdala, hippocampus and cingulate cortex) but was not different from controls in neocortical areas (frontal and temporal). Somatostatin concentrations were not different from controls in any region tested. In contrast to Alzheimer's disease and Parkinson's disease, intellectual deterioration in PSP is not associated with a deficit in neocortical somatostatin and CAT levels.

Aged↗

Sucrose-induced obesity: effect of diet on obesity and brown adipose tissue.

Adult male Sprague-Dawley rats were divided into three groups and fed diets containing either 10, 20, or 40% protein for 56 days. Half of the rats in each dietary condition were given a 32% sucrose solution plus the standard diet and water. Sucrose intake varied directly as a function of dietary protein levels. Rats fed either the 10 or 20% protein diet and sucrose had higher caloric intakes, gained more weight, were more efficient at using calories for weight gain, and had more adipose tissue than rats given the same diet without sucrose. Rats fed the 40% protein diet and sucrose did not exhibit overeating, excess weight gain, or increased feed efficiency relative to animals fed the 40% diet alone. Animals given sucrose had more interscapular brown adipose tissue (IBAT) and a greater metabolic potential for thermogenesis in IBAT as determined by GDP binding in mitochondria than rats not fed sucrose. These results demonstrate that dietary protein is important in the development of sucrose-induced obesity and that increases in IBAT mass and activity can occur concomitant with increased feed efficiency.

Adipose Tissue, Brown↗

[Meningoradiculitis after a tick bite. Study of 31 cases].

A retrospective study covering a period of 20 years identified reports on 31 cases of meningoradiculitis of the Garin-Bujadoux-Bannwarth type (MRGBB). Clinical, biological, electromyographic characteristics and course of the disease were studied. The most recent cases (n = 8) in 1984 and 1985 had serological tests for Borrelia Burgdorferi and half of the cases had negative results. Conversely, in some patients with meningoradiculitis, even in the absence of a tick bite or of migrating chronic erythema, serology was positive for Borrelia Burgdorferi antigen. The efficacy of antibiotic therapy against pain and on the quality and time of functional recovery justifies the use of this therapy under these two circumstances.

Bites and Stings↗

[Abetalipoproteinemia. Apropos of 2 cases].

The cases of two sisters with abetalipoproteinemia are reported. Both presented the complete clinical and biological features of the disease: ataxia, retinitis pigmentosa, lack of apolipoprotein B, chylomicrons, LDL and VLDL, reduced titers of serum cholesterol and triglycerides, acanthocytosis, very low levels of serum vitamin A and E. Abetalipoproteinemia is a rare autosomal inherited disease. It is usually revealed during early childhood by steatorrhea and failure to thrive; ataxia and retinitis pigmentosa appear later. The originality of these two cases stems from: 1) their late and fortuitous diagnosis: the first sister was investigated at the age of 42 after the discovery of a vitamin K induced coagulation disorder. The other sister was 39 when she was routinely examined as a family member; 2) the presence of constipation without any other suggestive digestive complaint. However, white discoloration of the duodenal mucosa seen at endoscopy and lipid droplets within the intestinal absorptive cells at biopsy were characteristic. Barium studies showed diffuse involvement of the small bowel which was displaced by an enlarged sigmoid. Treatment consists of administration of vitamin A and vitamin E which prevent or delay ocular and neurologic symptoms. Vitamin K is associated whenever necessary.

Abetalipoproteinemia↗

Environmental effects on the intake of overweight and normal-weight men.

The effects of changes in portion size and social condition on food intake were compared for overweight and normal-weight men. Subjects believed they were participating in a luncheon test of the acceptability of lasagna. In Study 1, under conditions designed to mimic social dining in a cafeteria, subjects were given either 255 or 426 g of lasagna. All subjects at a particular session received the same size portion and could ask for as many helpings as they wished. While overweight subjects ate more than normal-weight subjects, changes in portion size had no significant effects on intake. In Study 2, the effects of social vs. isolated dining were compared. Overweight subjects ate more than normal-weight subjects, and both overweight and normal-weight subjects eating socially ate more than subjects eating alone. In both studies there was a significant relationship between preference rating and intake for overweight subjects but not for normal-weight subjects. The results support the existence of differences in intake between overweight and normal-weight individuals and also indicate the potential importance of environmental factors in the intake of both groups.

Adult↗

Surgical anatomy of the arteries of the posterior fossa.

The development of revascularization for vertebrobasilar ischemic events has created a need to identify the best sites at which to perform bypass procedures. Since the occlusive process may selectively affect various levels of the vertebrobasilar tree, sites in different vessels must be used to reestablish flow distal to the area of occlusion. Twenty-seven unfixed human brains were obtained 4 to 8 hours post mortem, and the vertebrobasilar system was injected with polyester resin. Under a surgical microscope the outer diameter, length, and site of origin of major branches were recorded for the following arteries: vertebral, basilar, posterior inferior cerebellar (PICA), anterior inferior cerebellar (AICA), superior cerebellar (SCA), and posterior cerebral (PCA). The ideal sites for an anastomosis were identified as the pretonsillar segment of the PICA, the second portion of the AICA, the perimesencephalic segment of the SCA, and the perimesencephalic part of the PCA. Based on the anatomical observations reported here, these were the best sites because of their outer diameter, degree of mobility, least number of branches, and frequency of occurrence. Use of two of these arteries, however, may pose potential problems: although the PCA has an ideal outer diameter, it also has numerous branches to the brain stem in its most accessible site in the perimesencephalic area; and the PICA is not consistently present, being found in only 75% of the 27 specimens studied.

Basilar Artery↗

[Peroperative echography in pediatric neurosurgery. Experience in a series of 40 procedures].

Forty neurosurgical operations (supra-tentorial: 27; infra-tentorial: 10; intra-spinal: 3) have been performed under real time ultrasonography control. The supra-tentorial anatomical Landmarks visualized on the screen were the base of the skull, the cranial vault, the falx cerebri and the ventricles. The infra-tentorial landmarks were the petrous bone, the tentorium and the brainstem. The dura-mater, the subarachnoid space and the medulla were easily detected in the spinal canal after laminectomy. Intra operative real time ultra sonography performed on the dura-mater or on the cortex allows the detection of pathological structures. Tumors, whatever their location, are hyperechogenic; cysts, on the contrary, are hypoechogenic. Tumors are therefore precisely delimited by intra operative ultrasonography; thus the amount of normal nervous tissue which must be removed to reach a subcortical or intra medullary tumor can be reduced to a minimum. Moreover, the use of intra operative ultra sonography gives control ever the completeness of tumor removal as it shows any residual tumor intra operatively. Echography also facilitates other operations such as placement of a ventricular catheter, tapping of an abscess or biopsy of a tumor. Large arterial vessels or arterio-venous malformations are easily located because their pulsations are visible on the screen. For all these reasons, real time ultrasonography should always be available in the operating theater. In this series, it has provided essential information in 15 cases out of 40.

Brain Abscess↗

Monoclonal antibodies raised against Lewy bodies in brains from subjects with Parkinson's disease.

Monoclonal antibodies which immunocytochemically label Lewy bodies on sections of substantia nigra from subjects with Parkinson's disease were produced by immunization of mice with substantia nigra and locus coeruleus containing Lewy bodies from parkinsonian subjects post-mortem. Tests of specificity indicate that the antibodies do not recognize the same antigen. One of the antibodies (G7) immunocytochemically labels only Lewy bodies, the other (G9) also faintly labels the cell bodies of nigral dopaminergic neurons and cerebellar Purkinje cells in both normal and parkinsonian brains. Absorption experiments show, however, that the G7 antigen is present in normal substantia nigra and the G9 antigen in normal substantia nigra and Purkinje cells. Neither of the antibodies seems to be directed against neurofilament protein. Immunoblots after two-directional electrophoresis indicate that antibody G7 labels a protein with an iso-electric point around 5.6 and a mol. wt. of approximately 40 kdalton, whereas the protein labeled by antibody G9 has an iso-electric point of near 8 and a mol. wt. above 70 kdalton.

Antibodies, Monoclonal↗

A comprehensive approach to extremity vascular trauma.

A review of our experience with 368 patients with 382 extremity injuries has resulted in a treatment plan resulting in improved patient care and limb survival. A thorough examination of the injured extremity will help diagnose a significant vascular injury in the presence of certain clinical findings, eg, bruit or thrill (100%), signs of acute ischemia (100%), absent pulse (91%), shock (89%), neurologic deficit (78%), and hematoma (55%). Arteriography in proximity injuries has resulted in an abnormal finding in 16% of cases. A total of 165 vascular injuries, including 112 arterial and 53 venous injuries, were repaired using a variety of techniques. In 136 patients undergoing operative intervention, there was no operative mortality and a 1.5% amputation rate.

Adult↗