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[Tolosa-Hunt syndrome and orbital pseudotumour. Overlapping conditions in a case with an unusual clinical profile].

INTRODUCTION: The origin of Tolosa-Hunt syndrome (THS) and orbital pseudotumour (OP) is not fully understood. It is acknowledged as having an unspecific granulomatous inflammatory nature in different locations. Although there are differences between the clinical features of the two conditions, they also share a number of physiopathogenetic, therapeutic and, in some cases, iconographic similarities. Possible clinical recurrences are common in the two conditions and a broad differential diagnosis is required in all cases. Yet, the association of both processes in the same patient, with radiological proof of the migration of the inflammatory injury, is not frequent. CASE REPORT: We report the case of a male patient with a long history of recurring unilateral painful ophthalmoplegia that was sensitive to steroids; criteria for THS were fulfilled and there was later development of homolateral OP, six years after the onset of his symptoms. The findings in serial studies conducted with magnetic resonance imaging must be highlighted. The patient was submitted to a surgical intervention to treat the orbital injury and a chronic inflammatory process was observed in the fibrotic phase. CONCLUSIONS: Inflammatory pseudotumour and THS perhaps have more points in common than has traditionally been accepted. To our knowledge few cases of the above-mentioned association have been reported in the same patient. When confronted by cases of painful ophthalmoplegia with excessive recurrences the physician must consider the possibility of other alternative diagnoses.

Adult↗

[Long-term risk of relapses in Tolosa-Hunt syndrome].

INTRODUCTION: The reviewed diagnostic criteria of Tolosa- Hunt síndrome (THS) by the International Classification of Headache Disorders (ICHD-II, 2004) includes demonstration of inflammatory changes by magnetic resonance imaging (MRI) in the cavernous sinus region as an alternative to biopsy. Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia. It remains unclear for how long such close observation should be extended to assure the diagnosis nor whether THS represents a self-limited condition in the long-term. CASE REPORT: An observational study along 13 years of a patient fulfilling current ICHD-II criteria for THS to clarify the natural history of the disorder. RESULTS: We witnessed three episodes, the presenting one causing severe left orbital pain, ipsilateral abducens palsy , and hypoesthesia in the territory of the ophthalmic branch of the trigeminal nerve. Gadolinium MRI showed enhancement of tissue infiltrating the lateral wall of the carotid sinus. A relapse 21 months later caused involvement of left oculomotor and abducens nerves in addition to orbital pain which responded rapidly to steroids. Ensuing remission maintained for up to 11 years concluded in a new relapse again characterized by severe ipsilateral orbital pain and frontal numbness, but no ophthalmoplegia. A gadolinium-MRI did not show enhancement on this occasion despite prompt response to steroids. CONCLUSION: The risk of relapses in THS and its response to steroids may be maintained for as long as 13 years.

Adult↗

Tolosa-Hunt syndrome. Arteriographic evidence of improvement in carotid narrowing.

Diagnosis of Tolosa-Hunt syndrome was made in a 25-year-old woman on the basis of unilateral third and sixth nerve paresis and possible involvement of the first branch of the ipsilateral trigeminal nerve, accompanied by headaches and ocular pain, which responded promptly to corticosteroid administration. Irregular narrowing of the right carotid siphon and occlusion of the homolateral superior ophthalmic vein were observed. During steroid therapy this stenosis improved in association with almost complete clinical recovery, although the vein was not recanalized. Of ten reported cases with contrast radiographic abnormalities, including our own, only two showed pupillary involvement. We hypothesize that the third nerve paresis with pupillary sparing in this syndrome may be attributable to the same underlying mechanism as that of diabetic ophthalmoplegia.

Adult↗

Painful ophthalmoplegia. The Tolusa-Hunt syndrome.

A 36-year-old man had the Tolusa-Hunt syndrome, an unusual cause of painful ophthalmoplegia with a neurologic deficit that may not be distinguishable from the many other disease processes that involve the orbital apex. Diagnosis was made by excluding the other causes, and there was a prompt response to high-dose steroid therapy, which prevents or minimizes any permanent neurologic deficit. Because of this and the fact that the otolaryngologist is often involved in the diagnosis and treatment of the many causes of painful ophthalmoplegia, it is important to be aware of this syndrome.

Adult↗

Population densities of Sulawesi crested black macaques (Macaca nigra) on Bacan and Sulawesi, Indonesia: effects of habitat disturbance and hunting.

Population surveys of Sulawesi crested black macaques (Macaca nigra) were conducted on the Indonesian islands of Sulawesi and Bacan in 1992-1994 to assess the status of natural populations and determine habitat and anthropogenic factors affecting their population densities. We surveyed five sites for primates, including undisturbed and disturbed habitats. Data were collected on habitat structure and composition at two undisturbed and one disturbed forest site in which the primates were surveyed. The highest density of macaques was found in primary forest at Gunung Sibela Nature Reserve on Bacan (170.3 individuals/km2). Population density in logged forest on Bacan was high but significantly less than primary forest (133.4 individuals/km2). The high density of crested black macaques in primary forest on Bacan is best explained by the high carrying capacity found in primary forest. The lower food quantity and quality of food resources found in logged forest correlated with lower primate densities compared to primary forest. However, the large population of macaques in logged forest demonstrates the conservation value of such forest. Densities on Sulawesi at Tangkoko-Batuangas-DuaSudara Nature Reserve (TBDS) showed a continuing decline since earlier surveys. Primate densities were highest near the protected center of Tangkoko Reserve (66.7 individuals/km2). The peripheral areas of Batuangas and DuaSudara, even though adjacent and continuous, showed lower population densities of 46.4 and 23.5 individuals/km2, respectively. The best explanation for the continued decline of Macaca nigra populations at TBDS is hunting. Unless conservation measures are implemented immediately, M. nigra on Sulawesi risks extinction in the near future.

Animals↗

Tolosa-Hunt syndrome: a case with associated facial nerve palsy.

A 54-year-old man developed a painful right third nerve palsy with signs of involvement of the first two divisions of the trigeminal nerve and, later, ipsilateral peripheral facial nerve weakness. A cavernous sinus venogram demonstrated narrowing of the third portion of the right ophthalmic vein and poor filling of the adjacent cavernous sinus. Resolution occurred with steroid therapy. The Tolosa-Hunt syndrome may be a variant of a larger syndrome of recurrent multiple cranial polyneuropathies without identifiable cause.

Blepharoptosis↗

The hunters and the hunted revisited.

The dietary niches of extinct animals, including hominids and predators, may be constrained using stable carbon isotope ratios in fossil tooth enamel.(13)C/(12)C ratios of many of the primates abundant in the faunal assemblages of Members 1 and 2 at Swartkrans, including cercopithecoids and Australopithecus (Paranthropus) robustus, and a range of other possible prey species, have been reported previously. Resulting suggestions of a mixed, or omnivorous, diet for A. robustus raise questions about niche overlap with coeval, larger brained Homo. Here we present(13)C/(12)C data from Homo and several large predators including Panthera pardus, Dinofelis sp., Megantereon cultridens and Chasmoporthetes nitidula in Member 1, and P. pardus and P. leo in Member 2, in order to compare the two hominid species and to determine likely predators of the various primates and other macrovertebrates. Results for three Homo cf. ergaster individuals are indistinguishable from those of A. robustus, showing that proportions of C(3)- and C(4)-based foods in their diets did not differ. P. pardus, Megantereon and Crocuta are shown to be likely predators of the hominids and Papio baboons in Member 1, while the Dinofelis individual concentrated on prey which consumed C(4)grasses. The hunting hyaenid C. nitidula preyed on either mixed feeders or on a range of animals across the spectrum of C(3)and C(4)variation. The data from Members 1 and 2 confirm a shift in leopard diets towards animals that consumed C(4)grasses.

Animals↗

Gene hunting in hypoxia and exercise.

New technologies in genomics and proteomics are revolutionizing the study of adaptation to environmental stress. These approaches provide a comprehensive overview of the responses of thousands of genes/proteins to stress and enormously expand our view of the molecular and metabolic changes that underlie physiological responses. Several new technologies can help physiological labs to become gene hunters. DNA array screening is particularly effective for two purposes: (1) identifying coordinated responses by functional groups of gene/proteins such as multiple members of a signal transduction cascade or enzymes of a metabolic pathway, and (2) highlighting cell functions that have never before been linked with the stress under consideration. We have shown that heterologous screening of DNA arrays can be a highly effective method of gene hunting for the comparative biochemist provided that it is followed up by species-specific analyses including PCR to quantify transcript levels and Western blotting to analyze protein responses. Recent work in my lab has used cDNA array screening to evaluate responses to low oxygen by multiple hypoxia/anoxia tolerant systems, revealing common gene responses across phylogeny. Analysis of vertebrate facultative anaerobiosis in freshwater turtles reveals an interesting mixture of gene responses, including up-regulation of antioxidant enzymes, protease inhibitors, and proteins of iron metabolism; a few of these are coordinated by the hypoxia inducible factor in other systems but most are not. Array screening is also providing new insights into how exercise stimulates the growth of differentiated muscle cells and studies in our lab are identifying the gene responses associated with "anti-exercise"--gene up-regulation that aids hibernating mammals to maintain their muscle mass despite months of inactivity.

Animals↗

Altered gene expression in fetal Down syndrome brain as revealed by the gene hunting technique of subtractive hybridization.

Information on gene expression in brain of patients with Down Syndrome (DS, trisomy 21) is limited and molecular biological research is focussing on mapping and sequencing chromosome 21. The information on gene expression in DS available follows the current concept of a gene dosage effect due to a third copy of chromosome 21 claiming overexpression of genes encoded on this chromosome. Based upon the availability of fetal brain and recent technology of gene hunting, we decided to use subtractive hybridization to evaluate differences in gene expression between DS and control brains. Subtractive hybridization was applied on two fetal brains with DS and two age and sex matched controls, 23rd week of gestation, and mRNA steady state levels were evaluated generating a subtractive library. Subtracted sequences were identified by gene bank and assigned by alignments to individual genes. We found a series of up- and downregulated sequences consisting of chromosomal transcripts, enzymes of intermediary metabolism, hormones, transporters/channels and transcription factors (TFs). We show that trisomy 21 or aneuploidy leads to the deterioration of gene expression and the derangement of transcripts describes the impairment of transport, carriers, channels, signaling, known metabolic and hormone imbalances. The dys-coordinated expression of transcription factors including homeobox genes, POU-domain TFs, helix-loop-helix-motifs, LIM domain containing TFs, leucine zippers, forkhead genes, maybe of pathophysiological significance for abnormal brain development and wiring found in patients with DS. This is the first description of the concomitant expression of a large series of sequences indicating disruption of the concerted action of genes in this disorder.

Amino Acid Sequence↗

Intracranial pressure, cerebral perfusion pressure, and SPECT in the management of patients with SAH Hunt and Hess grades I-II.

The objective of our study was to examine the course of intracranial pressure (ICP) in patients with SAH Hunt and Hess grades I-II and to analyze the relationship between ICP, cerebral perfusion pressure (CPP) and cerebral blood flow (CBF). Twenty-three patients were studied. ICP, arterial blood pressure (ABP) and CPP were continuously recorded. The measurements of CBF with single-photon emission computed tomography (SPECT) were performed in fifteen patients, who showed TCD flow velocities exceeding 120 cnlJsec. In the first two days after SAH four patients (15%) showed a normal ICP, six (25%) patients had a moderate increase of ICP ranged from 15 to 25 mm Hg and thirteen (60%) patients had ICP values higher than 25 mm Hg. Seven of these patients, with ICP values higher than 40 mm Hg, showed clinical signs of delayed ischaemia. After the treatment with osmotic diuretic, ICP decreased and a clinical improvement was observed with the exception of one patient. In this patient, the SPECT study showed middle cerebral hypoperfusion concordant with the clinically ischaemic hemisphere. Our study showed the utility of the monitoring of these parameters in patients with lower grade SAH, because it allows the modulation of the therapeutic approach and defines the onset of neurological deficits secondary to cerebral ischaemia in all grades of SAH.

Adult↗

The Tolosa-Hunt syndrome.

The signs and symptoms of the Tolosa-Hunt syndrome are described and the results of orbital phlebography discussed. Emphasis is placed on the importance of systemic administration of corticosteroids, both as a diagnostic test and as a therapeutic measure. A case history is presented.

Adult↗

The tolosa-Hunt syndrome: further clinical and pathogenetic considerations based on the study of eight cases.

The Tolosa-Hunt syndrome (THS) is characterized by remittent and sometimes recurring episodes of painful ophthalmoplegia. The etiopathogenesis is still unclear and is an object of controversy. A non-specific granulomatous process of the wall of the cavernous sinus is claimed by many authors as the possible cause, on the basis of a few pathological studies. Other authors suggest the possible role of autoimmune or specific inflammatory processes localized in the retroorbital perineural tissues. The clinical, laboratory and radiological findings of the eight cases reported in the agreement with those previously described in the literature. However, the visual evoked potentials (VEP) were delayed in three of the four cases in which they had been studied. This finding, together with the observation that some analogies exist between THS and other well known neuritic processes of the cranial nerves, may suggest that at least in some cases THS may be related to an ocular polyneuritis.

Adult↗

Tolosa-Hunt syndrome versus recurrent cranial neuropathy. Report of two cases with a prolonged follow-up.

Two patients are described who had suffered for 12 years from episodes of painful ophthalmoplegia consistent with a Tolosa-Hunt syndrome (THS) alternating with palsies of cranial nerves other than the oculomotor (fifth motor and seventh on both sides). These two cases, as well as other similar ones previously reported in the literature, suggest that THS may sometimes be a variant of so-called recurrent cranial neuropathy, which is a benign and poorly understood clinical entity on an inflammatory or ischaemic basis.

Aged↗

Tolosa-Hunt syndrome, phlebographically controled after recovery.

A 27 year old patient, presenting clinically and phlebographically a Tolosa-Hunt syndrome was observed after recovery. Normal reappearance of a previously completely obstructed cavernous sinus was noted, leaving only a persisting irregularity of the superior ophthalmic vein.

Adult↗

Tolosa-Hunt syndrome with sellar erosion: case report.

The so-called Tolosa-Hunt syndrome consists of painful ophthalmoplegia caused by chronic nonspecific inflammation of the cavernous sinus and/or superior orbital fissure, responsive to steroid therapy. We present a case with the unusual feature of sellar erosion, in which angiography. CT and MRI suggested this idiopathic condition.

Cavernous Sinus↗

Radiological findings in gunshot wounds caused by hunting ammunition. An experimental study.

Experimental gunshots were made with hunting ammunition using a dummy model made of skin and foam rubber as the target. After penetration of intermediate targets of wood by the bullets, the characteristics of the wounds changed and their dimensions increased. The morphology of the wounds presented a very varied spectrum. When the gunshots had initially passed through wood 50 mm thick, radiographs of the skin showed a quantity of metallic residues between 10 microns and 1 mm. The metallic particles were wiped off the surface of the projectile by the target itself, whereby the best "wipe-off effect" was achieved with skin. The experimental findings suggest that the formation of the fine metallic residues is analogous to the development of the bullet wipe formed by lead bullets. Larger fragments flew into the target independently of the bullet and depending on the distance between the intermediate and final targets. A case example is documented.

Forensic Medicine↗

Normal muscle mitochondrial function in Ramsay-Hunt syndrome.

Mitochondrial encephalomyopathies may display clinical features similar to Ramsay-Hunt syndrome (RHS). We studied muscles mitochondrial function in 2 patients with RHS. Histochemical and ultrastructural studies of muscle biopsies and biochemical analysis of muscle mitochondrial enzymes were normal. There is no evidence for a disturbance of muscle mitochondrial function in RHS.

Adult↗

Sleep abnormalities in four cases of dyssynergia cerebellaris myoclonica of Ramsay-Hunt.

The nocturnal sleep of four patients with dyssynergia cerebellaris myoclonica (DCM) of Ramsay-Hunt was recorded with a polygraph. The following features were observed: a reduction of spindles, K complexes and vertex spikes; frequent arousals; rare rapid eye movements with a modification of their morphology and pattern; change in sleep stage percentages. In addition, myoclonus and polyspike-and-wave complexes appeared less frequently during sleep than during wakefulness. Three generalized convulsive and sixteen clonic seizures were recorded during stage 3/4 or on arousal. The clinical and physiopathological implications of these data are discussed.

Adolescent↗