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At least 199 records · Page 11Linked to original sources
PubMed1989

Dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome): a condition unrelated to mitochondrial encephalomyopathies.

Thirteen patients with dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome) had full clinical and neurophysiological study as well as muscle biopsy. The patients had action myoclonus, generalised epileptic seizures, and mild cerebellar syndrome. The disease was inherited in an autosomal recessive pattern in five patients, and occurred as isolated cases in the remaining eight patients. The age at onset of symptoms ranged from 6 to 15 years (mean, 10.4 years). The EEG and polygraphic findings included normal background activity in most patients, spontaneous fast generalised spike-and-wave discharges, photosensitivity, no activation during slow sleep, and vertex and rolandic spikes in REM sleep. Results of muscle biopsy, performed an average of 14 years after onset of the disease, were normal and showed no mitochondrial abnormalities. These findings suggest that Ramsay Hunt syndrome is a condition with distinctive clinical and neurophysiological features and unrelated to mitochondrial encephalomyopathies.

Adolescent↗
PubMed1989

Ramsay-Hunt syndrome in a preschool infant.

The Ramsay-Hunt syndrome affects mostly adults. The recovery of facial nerve function, according to recent literature, seems better than has been generally accepted. A small number of children with herpes zoster oticus have been reported. We describe a case of Ramsay-Hunt syndrome in a healthy 3 1/2-year-old girl. She still had an obvious facial palsy after 12 months. Three out of 6 reported children with herpes zoster oticus have had a slow recovery of the facial nerve function. This suggests a less favorable prognosis in children than in adults. Although not successful in this case, acyclovir should be tried early in the course of the disease.

Acyclovir↗
PubMed2004

Varicella-zoster virus DNA level and facial paralysis in Ramsay Hunt syndrome.

We have investigated whether the copy number of varicella-zoster virus (VZV) in saliva correlates with the clinical symptoms in patients with Ramsay Hunt syndrome. A real-time quantitative polymerase chain reaction assay was used to examine the VZV DNA copy number in saliva samples from 37 patients. We detected VZV DNA in 6 of the 7 patients with oropharyngeal zoster lesions (86%) and in 17 of the 30 patients who had zoster lesions only on the skin (57%). Patients with oropharyngeal zoster lesions had a high VZV load in their saliva, and the difference between the copy number in patients with oropharyngeal zoster lesions and those without was around 10,000 copies per 50 microL. In addition, patients with oropharyngeal zoster lesions showed worse recovery of facial function than those without. It seems that the VZV DNA level in saliva reflects the kinetics of viral reactivation in the facial nerve, as well as in the oropharyngeal epithelium, in patients with Ramsay Hunt syndrome.

DNA, Viral↗
PubMed1990

Detection of specific IgA antibodies to varicella zoster virus in serum of patients with Ramsay Hunt syndrome.

Varicella zoster virus (VZV)-specific IgG and IgA antibody titers were determined in serial serum samples of 23 patients with Ramsay Hunt syndrome by the immunoperoxidase assay. Varicella zoster virus-specific IgG antibodies were found in the first serum samples of all the patients. In 80% of 20 patients in whom a serum sample was available within 5 days after the onset of the disease. VZV-specific IgA antibodies were detected. The second serum sample was VZV-specific IgA-positive in all of the patients. While all the healthy age- and sex-matched control subjects had VZV-specific IgG antibodies, VZV-specific IgA antibodies were detected in a low titer (dilution = 2) in only three of the subjects. By using VZV-specific IgA antibody titers greater than or equal to 2 and greater than or equal to 4 by the immunoperoxidase assay as a "cutoff" for younger and older patients with Ramsay Hunt syndrome, respectively, an early diagnosis of the disease can be obtained in 89% of the younger and in 64% of the older patients by a single serum sample.

Adolescent↗
PubMed2005

TMB-Hunt: an amino acid composition based method to screen proteomes for beta-barrel transmembrane proteins.

BACKGROUND: Beta-barrel transmembrane (bbtm) proteins are a functionally important and diverse group of proteins expressed in the outer membranes of bacteria (both gram negative and acid fast gram positive), mitochondria and chloroplasts. Despite recent publications describing reasonable levels of accuracy for discriminating between bbtm proteins and other proteins, screening of entire genomes remains troublesome as these molecules only constitute a small fraction of the sequences screened. Therefore, novel methods are still required capable of detecting new families of bbtm protein in diverse genomes. RESULTS: We present TMB-Hunt, a program that uses a k-Nearest Neighbour (k-NN) algorithm to discriminate between bbtm and non-bbtm proteins on the basis of their amino acid composition. By including differentially weighted amino acids, evolutionary information and by calibrating the scoring, an accuracy of 92.5% was achieved, with 91% sensitivity and 93.8% positive predictive value (PPV), using a rigorous cross-validation procedure. A major advantage of this approach is that because it does not rely on beta-strand detection, it does not require resolved structures and thus larger, more representative, training sets could be used. It is therefore believed that this approach will be invaluable in complementing other, physicochemical and homology based methods. This was demonstrated by the correct reassignment of a number of proteins which other predictors failed to classify. We have used the algorithm to screen several genomes and have discussed our findings. CONCLUSION: TMB-Hunt achieves a prediction accuracy level better than other approaches published to date. Results were significantly enhanced by use of evolutionary information and a system for calibrating k-NN scoring. Because the program uses a distinct approach to that of other discriminators and thus suffers different liabilities, we believe it will make a significant contribution to the development of a consensus approach for bbtm protein detection.

Algorithms↗
PubMed2006

Searching for interpretable rules for disease mutations: a simulated annealing bump hunting strategy.

BACKGROUND: Understanding how amino acid substitutions affect protein functions is critical for the study of proteins and their implications in diseases. Although methods have been developed for predicting potential effects of amino acid substitutions using sequence, three-dimensional structural, and evolutionary properties of proteins, the applications are limited by the complication of the features and the availability of protein structural information. Another limitation is that the prediction results are hard to be interpreted with physicochemical principles and biological knowledge. RESULTS: To overcome these limitations, we proposed a novel feature set using physicochemical properties of amino acids, evolutionary profiles of proteins, and protein sequence information. We applied the support vector machine and the random forest with the feature set to experimental amino acid substitutions occurring in the E. coli lac repressor and the bacteriophage T4 lysozyme, as well as to annotated amino acid substitutions occurring in a wide range of human proteins. The results showed that the proposed feature set was superior to the existing ones. To explore physicochemical principles behind amino acid substitutions, we designed a simulated annealing bump hunting strategy to automatically extract interpretable rules for amino acid substitutions. We applied the strategy to annotated human amino acid substitutions and successfully extracted several rules which were either consistent with current biological knowledge or providing new insights for the understanding of amino acid substitutions. When applied to unclassified data, these rules could cover a large portion of samples, and most of the covered samples showed good agreement with predictions made by either the support vector machine or the random forest. CONCLUSION: The prediction methods using the proposed feature set can achieve larger AUC (the area under the ROC curve), smaller BER (the balanced error rate), and larger MCC (the Matthews' correlation coefficient) than those using the published feature sets, suggesting that our feature set is superior to the existing ones. The rules extracted by the simulated annealing bump hunting strategy have comparable coverage and accuracy but much better interpretability as those extracted by the patient rule induction method (PRIM), revealing that the strategy is more effective in inducing interpretable rules.

Amino Acid Sequence↗
PubMed2006

Oral contraceptives and increased headache prevalence: the Head-HUNT Study.

OBJECTIVE: To examine the prevalence of headache and migraine among women using oral contraceptives (OCs) in a large, cross-sectional population-based study. METHODS: In the Nord-Trøndelag Health Study in Norway 1995-1997 (HUNT 2), 27,700 (60%) out of 46,506 invited women responded to headache questions (Head-HUNT). Among 14,353 premenopausal women, 13,944 (97%) responded to questions regarding use of contraceptives. RESULTS: There was a significant association between headache and reported use of estrogen-containing OCs in premenopausal women, both for migraine (OR = 1.4, 95% CI = 1.2 to 1.7) and for non-migrainous headache (OR = 1.2, 95% CI = 1.0 to 1.4). A significant dose relationship between headache and the amount of estrogen in the OCs could not be demonstrated. No significant association between headache and OCs containing only gestagen was found. CONCLUSION: Headache, especially migraine, was more likely among premenopausal women using oral contraceptives containing estrogen.

Adult↗
PubMed1975

Arterial stationary wave phenomenon in Tolosa-Hunt syndrome.

In a patient with Tolosa-Hunt syndrome, serial cerebral angiograms showed arterial stationary wave phenomenon and persistent deformity of the carotid siphon. Stationary arterial waves are noted in only 0.3 percent of cerebral angiograms and have not been reported in Tolosa-Hunt syndrome. In accordance with theories proposed by Theander and New, we felt that the intense stenosis of the carotid siphon produced by the periarteritis in our patient caused relative obstruction and high resistance, giving rise to resonance of arterial pressure waves and creation of the arterial stationary waves.

Adolescent↗
PubMed1986

The "sinister" Tolosa-Hunt syndrome.

Four patients with presumed Tolosa-Hunt syndrome ultimately proved to have a parasellar tumor. All four had manifestations of a cavernous sinus syndrome, normal radiologic and medical investigations, and response to steroid therapy. The Tolosa-Hunt syndrome is a diagnosis of exclusion; many other lesions can simulate the clinical manifestations, including steroid responsiveness.

Adult↗
PubMed1994

[Orbital phlebography evaluation in 8 cases of Tolosa-Hunt syndrome].

Tolosa-Hunt syndrome (THS), or painful ophthalmoplegia is associated to a non-specific granulomatosis of unknown etiology, that involves the superior orbital fissure and its nervous and vascular structures. The clinical picture that responds to steroid therapy, is variable and is always associated with pain. Inflammatory conditions, tumors and aneurysms can produce similar symptoms. Computed tomography, cerebral angiography and orbital phlebography are the imaging methods of choice for making the diagnosis. We revised the results of these radiological examinations of eight patients seen at the Hospital São Paulo from 1989 to 1991, with the diagnosis of THS according to Hunt and Hannerz criteria. The analysis of orbital phlebographic changes based upon Hannerz et al. systematization showed non-specific features, but those were able to help the diagnosis.

Adult↗
PubMed1981

Cytological and immunological examination of cerebrospinal fluid in 9 patients with Ramsay Hunt's syndrome.

The purpose of the present study was to carry out the immunological and cytological examination of cerebrospinal fluid in 9 patients with Ramsay Hunt's syndrome. The results obtained were as follows: Among the serum immunoglobulins, IgG in 1 and IgM in 5 of 9 cases increased. The Varicella Zoster antibody titer was significantly elevated in sera of all 9 cases and in CSF of all 7 examined cases. There ws positive fluorescence to Varicella Zoster virus in 2 (Cases 8 and 9) of 4 cases in which CSF smears were examined. The cells showing fluorescence were 10.5% in the former and 1.5% in the latter. Pleocytosis was found in all cases and CSF cell counts ranged from 52 to 2,000/3 mm3 in the early stage. In 3 cases immunoglobulin containing cells (IgG and IgM) were seen about 2-20% within 2 weeks of onset. IgG contents in all and IgG% in 8 of 9 cases, IgA concentration in 7 and IgA% in 7 of 9 cases were increased. These results support the view that the Varicella Zoster virus spreads to the meninges and CSF from ganglions in patients with Ramsay Hunt's syndromes.

Adult↗
PubMed2001

Hunting practices increase the prevalence of Trichinella infection in wolves from European Russia.

From 1998 to 2000, 184 animals (82 wolves, 29 red foxes, 55 mustelids, 5 raccoon dogs, and 13 domestic dogs), mainly shot by hunters in the Tvier and Smoliensk regions of northwest European Russia, were tested for Trichinella larvae; 98 animals (53.3%) were found to be positive. The highest prevalence was detected in wolf (97.5%). Trichinella nativa was the most common species detected (98%). The diet of wolves was investigated by examining the stomach contents of 62 animals (75.6% of the total number of wolves examined for Trichinella). It consisted mainly of dog (36.4% of the total number of occurrences of all food items, PFO) and moose (31.2 PFO); however, during the hunting seasons of 1998-1999 and 1999-2000, skinned wolf carcasses were left in the forest as bait (567 carcasses, about 18,000 kg). This very high prevalence of Trichinella infection, the highest ever detected in a natural population of carnivores, could be explained by carnivore-carnivore transmission, influenced by the hunting practices adopted in the study area.

Animals↗
PubMed1989

[Tolosa-Hunt syndrome. Report of a surgical case].

A 58-year-old male presented with painful right ophthalmoplegia and was diagnosed as having Tolosa-Hunt syndrome. High-dose oral administration of a corticosteroid provided significant pain relief, but total ophthalmoplegia persisted. Computed tomography (CT) showed a tumor-like mass in the bilateral cavernous sinus. Angiography revealed occlusion of the right internal carotid artery. According to the literature, about 70% of cases of Tolosa-Hunt syndrome are caused by parasellar neoplasms. A transcranial biopsy of the patient's lesion revealed a nonspecific granulomatous process in the wall of the right cavernous sinus. He was again placed on high-dose corticosteroid therapy and within 1 week the right visual acuity improved slightly. The corticosteroid dose was gradually tapered over 3 months. After 2 months of therapy, the external ocular movement had almost fully recovered, although the light reflex was absent and the CT findings did not change.

Angiography↗
PubMed1995

Tolosa-Hunt syndrome with unusual clinical courses--two case reports.

Two cases of Tolosa-Hunt syndrome with unusual clinical courses are described. The clinical onset of the first patient was typical, but was unresponsive to steroid hormone treatment. The symptoms resolved spontaneously after 1 year. Computed tomography (CT) and magnetic resonance (MR) imaging revealed enlargement of the affected cavernous sinus, which did not change after the symptoms resolved. The clinical presentation in the second patient included marked pupillary dilatation, a sign which is absent in the classical criteria. CT showed no abnormal findings, but MR imaging revealed a mass located in the medial side of the cavernous sinus extending to the lateral wall, which might have caused the pupillary dilatation. After steroid therapy the symptoms disappeared and MR imaging showed remarkable reduction of the mass in the cavernous sinus. Tolosa-Hunt syndrome usually responds promptly to steroid therapy and rarely manifests marked pupillary dilatation. Such variation in presentation adds to the difficulties in diagnosis and treatment of this disease of unknown etiology.

Adult↗
PubMed2001

Racehorse injuries, clinical problems and fatalities recorded on British racecourses from flat racing and National Hunt racing during 1996, 1997 and 1998.

For improvements to the safety and welfare of racehorses to be possible, it is essential to have access to basic descriptive information about the veterinary incidents encountered during horseracing. A 3 year surveillance study (1996-1998) was conducted by The Jockey Club into racing injuries, other postrace clinical problems and fatalities from all 59 British racecourses (mainland Britain only) to identify risk factors. During the survey there were 222,993 racing starts: 106,897 starts in flat races on turf (47.9%), 26,519 starts in flat races on all-weather surfaces (11.9%), 30,932 starts in chases on turf (13.9%), 51,786 starts in hurdle races on turf (23.2%) and 6,859 starts in National Hunt flat races (3.1%). Information was recorded about age of horses, racing surfaces and clinical events observed or attended by a veterinary team of 2 clinicians and one veterinary surgeon employed by the racing authority. Of the 2358 clinical events reported (1.05% of all starts), 1937 involved the musculoskeletal system and 421 involved other body systems. Six hundred and fifty-seven incidents (0.29% of starts) resulted in death or euthanasia. Eighty-one percent of limb injury reports involved forelimbs and 46% involved flexor tendons/suspensory ligaments. Nonlimb problems included epistaxis (0.83/1000 starts), 'exhausted horse syndrome' (0.47/1000 starts) and paroxysmal atrial fibrillation (0.20/1000 starts). Incidents including fatalities per 1000 starts were 24.7 from chases, 19.45 from hurdle races, 8.46 from National Hunt flat races and 3.97 from flat races. The overall tendon injury was higher in chases than in hurdle races, even though age-specific rates of tendon injury were higher in hurdle races than in chases. The risk of injuries per start increased significantly with age, while softer racing surfaces were associated with fewer fatalities and injuries than firmer surfaces. The survey described in this paper has provided an up-to-date description of the fatal and non-fatal horseracing incidents under conditions on mainland Britain, enabling progress to be made towards improving the safety and welfare of racehorses.

Age Factors↗
PubMed1988

Electrodiagnostic findings in the early stages of Bell's palsy and Ramsay-Hunt's syndrome.

The incidence of denervation, as determined electrodiagnostically (nerve excitability test and electroneuronography), was analysed during the course of the disease in 150 patients with Bell's palsy and 45 with Ramsay-Hunt's syndrome. Of all 150 patients, 14.2% of those with Bell's palsy and 44% of the patients with Ramsay-Hunt syndrome either showed denervation at the time of the initial examination or developed denervation from neurapraxia within 3 weeks after the onset of paralysis.

Electrodiagnosis↗
PubMed1988

Neurotological findings in Bell's palsy and Hunt's syndrome.

Neurotological findings were analysed in 23 patients with Bell's palsy and in 25 patients with Hunt's syndrome. The incidence and extent of the auditory and vestibular pathology was high in Hunt's syndrome. Although patients had no subjective symptoms related to the auditory and/or vestibular pathology in the Bell's palsy cases, about one-third of the patients showed abnormal findings upon neurotological examination. Differentiating these two diseases is therefore considered difficult by means of neurotological examination in the same way as by serological testing as has been reported by other investigators.

Audiometry, Pure-Tone↗