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Acute profound deafness in Ramsay Hunt syndrome. Two case reports.

Two patients with sudden progressive profound hearing loss resulting from Ramsay Hunt syndrome are reported. Case 1: A 63-year-old woman was admitted to Jichi Medical School Hospital with sudden, progressing deafness of the left ear, vertigo, sore throat, and hoarseness. An otoscopic examination revealed the external ear and the tympanic membrane to be normal. Pure-tone audiometry revealed profound deafness in the left ear. A horizontal nystagmus in the non-affected direction was observed by gaze nystagmus test. An endoscopic examination revealed herpetic vesicles and shallow ulcers on the left side of the pharynx and the larynx. There was complete paralysis of the left recurrent nerve. Hearing acuity of the left ear did not recover at all with steroid hormone therapy. Case 2: A 75-year-old man was referred to the ENT Clinic by a dermatologist for hearing evaluation in Ramsay Hunt syndrome. The man had noticed severe otalgia and sudden progressive deafness of the right ear approximately 2 weeks prior to admission. Physical examination revealed herpetic vesicles and ulcers in the right external ear and lateral neck. Complete paralysis of the right facial nerve was noted. Profound hearing loss in the affected ear was observed by pure-tone audiometry. A gaze nystagmus test revealed a horizontal nystagmus in the non-affected direction. No recovery of the cochlear function was noted following administration of antiviral drug. The pertinent literature is briefly reviewed.

Aged↗

Enhanced MRI in patients with Ramsay-Hunt's syndrome.

Enhanced MRI was performed in 14 patients with Ramsay-Hunt's syndrome to investigate the pathogenesis of this syndrome. All MRI studies were performed on a 0.5T superconductivity MRI system using a head coil with Gd-DTPA. Enhancement was observed in the areas of the distal internal auditory canal and labyrinthine segment in many patients, and was especially prominent in patients suffering from vertigo, tinnitus, and hearing loss. In some patients it involved not only the facial nerve of the internal auditory canal but also the cochlear nerve and vestibular nerves. Since histological changes of the facial nerve in patients with Ramsay-Hunt's syndrome are assumed to occur in the distal internal auditory canal and labyrinthine segment, which is more proximal than the geniculate ganglion, and the possibility is suggested that inflammation may spread to the vestibular and cochlear nerve via the internal auditory canal.

Aged↗

[Conservative treatment of Hunt syndrome].

Based on the pathophysiology of Bell's palsy that edema as well as ischemia lead to both compression and hypoxia, Stennert employed high doses of cortisone and dextran and reported a high recovery rate. In the past 5 years, we have been treating patients with Bell's palsy and Hunt syndrome with a high dose of steroids or low-molecular dextran (SD therapy). SD therapy was administrated in 71 cases of Hunt syndrome, and the results were compared with those of a group of 36 patients who had been treated with orally administrated low-dose steroids. All patients with incomplete palsies recovered completely, regardless of the mode of treatment. In cases of complete palsy, 62% of patients recovered completely when treated with SD therapy. In contrast, 29% of the patients treated with orally administrated steroids recovered completely. These results indicate that for patients with complete palsy SD therapy is more effective than oral steroid therapy, while patients with incomplete palsy recover completely with oral steroids. On the basis of this study, oral steroids are best used in cases of incomplete palsy unless complete palsy develops. In these latter cases, we now believe that SD therapy should be started immediately.

Administration, Oral↗

[Clinical features and prognosis of facial palsy and hearing loss in patients with Ramsay Hunt syndrome].

Clinical studies were performed on 325 patients with Ramsay Hunt syndrome who were treated in the Facial Nerve Clinic at Ehime University Hospital between 1976 and 1995. The clinical manifestations of Ramsay Hunt syndrome were various. Three major symptoms, auricular vesicles, facial paralysis and vestibulo-cochlear dysfunction, were found in 57.6% of the patients although these symptoms did not always appear simultaneously. Auricular vesicles appeared before (19.3%), during (46.5%), or after (34.2%) the onset of facial paralysis. Hearing loss was observed subjectively in only 20% but objectively in 48.2% of the patients. Hearing loss appeared before (34.3%), during (34.3%), or after (31.3%) the onset of facial paralysis. Complete recovery from facial paralysis was achieved in 52.4% of the patients. Good recovery of the facial nerve function was achieved in patients who had zoster vesicles or vestibulo-cochlear dysfunction preceding the development of facial paralysis. Complete recovery of hearing was also achieved in 45.4% of the patients, and the recovery was better in patients having light hearing loss, less than 35dB. The patients younger than 16 years old showed better recovery from both facial paralysis and hearing loss than the patients older than 60 years. Glossopharyngeal nerve or vagal nerve paralysis concomitant with facial paralysis was found in 8 (2.5%) patients. The outcome of glossopharyngeal nerve paralysis was good but that of the vagal nerve was poor.

Adolescent↗

[Diagnosis and treatment of Ramsay Hunt syndrome (a report of 39 cases)].

Thirty-nine cases with Ramsay Hunt syndrome were presented, in which 23 cases were firmly diagnosed early, and others were misdiagnosed to be Bell's palsy in 9 cases, sudden sensorineural hearing loss in 5, herpes zoster pharyngitis in 1 and acute suppurative otitis media in 1, respectively. All patients were treated with prednisone or dexamethasone for 3 weeks. The results of treatment were as follows: complete recovery in 27 cases, residual facial paralysis in 12 patients, in which 11 had sensorineural hearing loss. We concluded that: 1. When patients present idiopathic facial paralysis associated with objective sensorineural hearing loss, Hunt syndrome should be suspected even in the absence of vesicles. 2. Treatment with steroid and antiviral agent is needed. There is no significant difference in the effects between oral and intravenous steroid therapy. 3. The poor prognosis may relate with severe facial paralysis accompanying severe hearing loss. 4. Acoustic stapedius reflex in patients with mild or no hearing loss is useful for defining the involved sites and evaluating the prognosis.

Adolescent↗

Who was... John A Hunt OBE?

Major John A (Anthony) Hunt OBE (1906 - 1986) was a professional geologist who spent part of his working life in the oil fields of the world and a further highly significant period of 28 years (1936 - 58) as a Colonial Administrative Service Officer in the former British Protectorate of Somaliland. In 1943, he was personally selected by the Governor to carry out a wide-ranging General Survey of the Protectorate. The report of this General Survey attained such repute that newly appointed colonial administrators were recommended to rely on three basic documents: the Koran, the Bible and Hunt's, A General Survey of British Somaliland.

England↗

The uncertainty of the toxic effect of stings from the Urtica nettle on hunting dogs.

This paper questions the effect of the sting from the Urtica species of nettle on hunting dogs, particularly in the US. Research in this area is limited and is reflected in the wide use of a particularly unsound literature reference on the subject. A general account is given of which types of "nettle" plant have a toxic sting, how the mechanism of the sting works, and the toxic substances it contains. The effects experienced by hunting dogs appear to represent a condition other than contact urticaria, which is normall the result of being stung by nettles (Urticas in particular). The possibility is discussed that the signs were caused by another plant, also commonly labelled a nettle, or that possibly they were caused by other than the direct stinging of soft tissues. Further research should be done on the toxic elements in the sting of Urtica chamaedryoides, indicated in some literature as the "guilty" plant.

Animals↗

Endovascular treatment of Hunt and Hess grade IV and V aneuryms.

BACKGROUND AND PURPOSE: Controversy still surrounds the question of when and how to manage cases of subarachnoid hemorrhage of Hunt and Hess grade IV and V aneurysms. Several authors are in favor of surgical treatment, reporting improved clinical outcomes and lower mortality rates. Considering that endovascular procedures are currently being increasingly used to treat aneurysms, we investigated their use in the management of subarachnoid bleeding in a retrospective review of 80 patients. METHODS: Eighty patients were admitted to our hospital between October 1992 and October 1998 with subarachnoid hemorrhage of Hunt and Hess grade IV and V aneurysms. Patients received standard resuscitation treatment, nimodipine to prevent vasospasm, CSF shunt when necessary, and selective occlusion with Guglielmi detachable coil. They were subsequently followed up for at least 1 year. Aneurysm occlusion was monitored with MR angiography and/or angiography at 6 months and at 1 year. RESULTS: Of the 80 patients, 42 (52.5%) did well (Glasgow Outcome Scale score of 1 or 2) (62% of the 56 patients with grade IV and 25% of the 24 patients with grade V aneurysms), seven (8.75%) presented with poor neurologic status (Glasgow Outcome Scale score of 3), and 30 (37.5%) died during the first 6 months (26.7% of the patients with grade IV and 62% of the patients with grade V aneurysms). One patient was lost to follow-up. The main causes of death were consequences of initial bleeding in the patients with grade V aneurysms and vasospasm in the patients with grade IV aneurysms. CONCLUSION: The results are at least as encouraging as the outcomes reported for the surgical series and suggest that early endovascular treatment of high grade hemorrhage is a feasible option, especially because endovascular maneuvers can be performed at any time, even during vasospasm.

Adolescent↗

Dimorphic foraging behaviors and the evolution of hominid hunting.

In contemporary foraging societies men typically hunt more than women. This observation has played an important role in many reconstructions of hominid evolution. The gender difference in human hunting, likely a product of both ecological and cultural factors, is mirrored by a similar sex difference among nonhuman primates. Existing explanations of such primate behavioral dimorphism are augmented by the recognition of an additional factor that may contribute to differences between males and females in the value of meat. Episodic female immunosuppression is a normal part of reproduction. Because meat is a source of pathogens, females can be expected to exhibit less constant attraction to meat. Sexual dimorphism in the attraction to meat may then contribute to dimorphic foraging specializations, a divergence that is likely augmented by the differential value of insectivory across the sexes. With the rise of cultural transmission of foraging knowledge, dimorphic foraging behaviors would have been reinforced, creating a more comprehensive gender-based division of labor.

Animals↗

[A case of Ramsay Hunt syndrome initiated with hoarseness and dysphagia: consideration on spreading mechanisms of cranial neuropathy].

A 85-year-old woman was admitted to our hospital because of progressive hoarseness and dysphagia of two days' duration. Neurological examination on admission revealed right pharyngeal and vocal cord palsies. After admission, gradual swelling of her right ear was noted, and on day 6, vesicular eruptions in her right geniculate zone, the right VII and the VIIIth cranial nerve palsies were added. On the basis of these findings, she was diagnosed as Ramsay Hunt syndrome. Varicella zoster virus (VZV) infection was confirmed by the elevation of serum anti-VZV-antibody titer, and detection of VZV DNA from cerebrospinal fluid. Ramsay Hunt syndrome associated with multiple cranial neuropathy is not frequently reported. Reviewing Japanese literatures, we found that the IX and the Xth cranial nerves were most frequently affected, and the half of these cases were initiated with cranial neuropathy other than the VIIth. Additionally, spreading mechanisms of cranial neuropathy, and the early diagnostic problems of these conditions were discussed.

Aged↗

[Witch hunt].

Owing to its sinister characteristics, as well as its temporal pertaining to the Modern Age, the Witch-Hunt historical episode has become an embarrassing affair for our supposedly rational beings' self-esteem. Long lastingly, therefore, Witch-Hunt has been either overpassed straight away or just attributed to violent and pathological a manifestation of collective craze as its own name indicates. Lately, though, many authors have met the challenge of finding out a rational background thereof, actually unearthing coldly calculated, relentlessly pursued outweighing political and economic interests. It is the author's contention that a serene, diligent consideration of all hypotheses that have been set before the connection with this episode--together with their very inherent disparities--could (apart from their own heuristic values) contribute eventually to both sharpening and broadening the understanding of our human condition, complex and fragile as it is.

Argentina↗

[A case of Tolosa-Hunt syndrome accompanied by facial and vestibular nerve damage].

A 37-year old man, who had repeatedly suffered from transient ophthalmoplegia in his left eye at the age of 29 and 36, developed left painful ophthalmoplegia accompanied by ipsilateral facial nerve palsy in August, 1991. Neurological examination revealed involvement of the left oculomotor, trochlear, ophthalmic division of the trigeminal, abducens, facial and vestibular nerves. Gadolinium-enhanced MRI which was taken at the acute phase of the illness demonstrated markedly enhanced left cavernous sinus and adjacent thickened dura mater in the middle cranial fossa. At the remission phase after starting corticosteroid therapy, these enhanced lesions were no longer observed even in enhanced MRI studies. We diagnosed him as suffering from Tolosa-Hunt syndrome presently accompanied by facial and vestibular nerve damage because of his history of illness, confined lesion in the left cavernous sinus and steroid-induced remission. We concluded that Tolosa-Hunt syndrome may be accompanied by damage of other cranial nerves in its course and that repeated gadolinium-enhanced MRIs are necessary for diagnosis and observation of the patients.

Adult↗

[Witch trials in the Salem as a medical error. Witch hunts in the XVII century and the medical art].

That article concerns with the influences of medical practitioners in witch hunts in the Salem (1692). Witch trials in England and English colonies in the XVII century were mainly criminal trials. Witchcraft had been there considered principally as a crime-tool rather then the crime itself. Witches were usually accused of crimes such as the murder and the disease sending. Physicians normally played in that situations the role of court experts. They decided if analyzed disease had normal or abnormal, artificial origin. In the Salem an medical practitioner judged that an illness which touched some children had come from the acts of sorcery. That was the beginning of a long and bloody witch hunt. Death sentences in the Salem trial were also justified with applying medical and physical categories to show the objectivity of a witchcraft. Salem judges were sure that by using "objective" categories they had been able to proof the existence of material relations between the witch and bewitched persons without the possibility of error.

Capital Punishment↗

[A fungal aneurysm in a patient with presumed Tolosa-Hunt syndrome].

A case of fungal aneurysm associated with presumed Tolosa-Hunt syndrome is reported. A 57-year-old man was admitted to our hospital with complaints of left blepharoptosis, headache and weight loss. Neurological examination revealed left ophthalmoplegia without facial hypesthesia. Visual acuity was normal. Laboratory studies showed raised ESR, 4+ glycosuria, and a blood sugar of 351mg/dl. Computerized tomography (CT) scan and left carotid angiography were considered normal. Left orbital venography showed no filling of the left cavernous sinus. Diabetic ophthalmoplegia was suspected by a neurologist. The patient was treated with insulin therapy, but visual acuity worsened, and hypesthesia was noted in the first and second divisions of the left trigeminal nerve. Subsequent CT scan demonstrated a high density lesion, which was homogeneously enhanced, in the left cavernous portion and the superior orbital fissure. The patient was presumed of Tolosa-Hunt syndrome, and prednine therapy (30mg/day) was started. On the second day after the administration of prednine, hypesthesia of the first and second division of the left trigeminal nerve improved. After 9 days of prednine therapy, the patient suddenly complained of severe headache, and lapsed into a coma. Massive hemorrhage with subarachnoid hemorrhage was recognized on the CT scan, with a marked midline shift to the right. The hematoma was immediately removed. A ruptured cerebral aneurysm was found at the bottom of the hematoma. The aneurysm was located in the distal portion of the left middle cerebral artery. Aneurysm clipping with external decompression and bilateral ventricular drainage was performed.(ABSTRACT TRUNCATED AT 250 WORDS)

Aneurysm, Infected↗

[Hodgkin's disease associated with Tolosa-Hunt syndrome].

A thirty-eight-year-old man developed gradually progressing right retro-orbital pain, diplopia on the left lateral gaze, and left ptosis. On examination paresthesia was present on the first division of right trigeminal nerve. Orbital venography revealed obstruction of right superior orbital vein on the entering portion to the cavernous sinus. A daily administration of 30 mg of prednisolone resulted in a rapid improvement of the symptoms. Diagnosis of Tolosa-Hunt syndrome was made on the basis of neurological symptoms, roentgenographic findings and responsiveness to prednisolone. One year later, weakness of right leg accompanied with left cervical and axillary lymphadenopathy was developed. A biopsy specimen of cervical lymph node was identical with the Hodgkin's disease, lymphocyte predominance type. He was treated with COPP regimen; lymphadenopathy decreased in size. We reported a rare case of Hodgkin's disease preceded by Tolosa-hunt syndrome which might be caused by the extranodal lesions.

Adult↗

[Variants of the Ramsay Hunt syndrome].

Out of 776 patients having polymorphic syndromes caused by herpes simplex or zoster infection 25 patients exhibited Ramsay Hunt syndrome. Altogether 5 clinical variants of the syndrome were recognized. Neurological symptoms and liquor investigations show that the process goes far beyond the ganglion of the facial nerve and therefore can be characterized as multiradiculoganglioneuritis. There are also frequent associated meningeal and encephalitic symptoms. Etiologically, some clinical variants of Ramsay Hunt syndrome arise from herpes simplex infection.

Adult↗

Rifle wounds from high velocity, center-fire hunting ammunition.

Wounds inflicted by high velocity, center-fire rifles firing hunting ammunition are radically different from wounds caused by handguns or .22 rim-fire rifles. Injuries from pistol or .22 rim-fire bullets are confined to tissue and organs directly in the wound track. In contrast, high velocity rifle bullets can injure structures without actually contacting them. This is due to the temporary cavity produced by such missiles with the resultant shock waves having pressures of up to 200 atmospheres (20 MPa). Organs struck by such high velocity rifle bullets may undergo partial or complete disintegration. Hunting ammunition, as it passes through the body, tends to shed fragments of lead from its core, producing a characteristic snowstorm picture on X-ray. The maximum range at which powder tattooing of the skin occurs from center-fire rifles depends on the physical form of smokeless powder used as propellant. In a test with a 30-30 rifle, cartridges loaded with ball powder produced powder tattooing out to a range of 30 in. (76 cm), while similar cartridges, loaded with the traditional cylindrical powder, produced tattooing out to only 12 in. (30 cm) of range.

Firearms↗

Isoelectrophoretogram of gazelle hemoglobin--a suggested tool for proving hunting offenses.

Hunting gazelle is an offense according to Israeli law. When comparative isoelectric focusing was performed on bloodstains made from gazelle, goat, sheep, and cow blood, the pattern obtained from gazelle hemoglobin differed from those of the other animals tested. The use of this difference in hemoglobin pattern is suggested as a means to identify gazelle blood in hunting offense cases.

Animals↗