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PubMed1988

The surgical one-stage management of combined cranio-maxillo-facial and frontobasal fractures. Advantages of the subcranial approach in 374 cases.

The aim of this paper is to emphasize the advantages of a one-stage, step by step repair of facial skeletal injuries as well as the dural tears and lesions of the anterior fossa, by one and the same team. The methods described permit optimal reduction of the pseudohypertelorism and major displacements in severe combined craniofacial injuries. The simultaneous subcranial exposure of the entire basal region of the anterior fossa obviates the intracranial approach or frontal lobe retraction thus making early stage surgical management feasible. The surgical treatment of these injuries as a single entity, as well as further modifications reduce complications such as recurrent CSF leakage, anosmia, mucocoele or secondary operations for inadequate fracture reduction. The significance of primary meticulous reconstruction of the combined cranial vault and midface fractures for the achievement of normal physiological function of the various systems is particularly highlighted.

Bone Plates↗
PubMed2002

GnRH neuronal development: insights into hypogonadotrophic hypogonadism.

Pulsatile secretion of the hypothalamic decapeptide gonadotrophin-releasing hormone (GnRH) regulates activity of the pituitary-gonadal reproductive axis. Defects of this neuroendocrine axis necessarily result in hypogonadotrophic hypogonadism. In many vertebrate species studied, the main population of GnRH neurones originates extracranially within the olfactory system. In humans, both olfactory and GnRH systems are affected in Kallmann's syndrome--resulting in isolated hypogonadotrophic hypogonadism (IHH) combined with anosmia (loss of sense of smell). Familial IHH is also caused by other genetic conditions, which prevent GnRH from activating luteinizing hormone/follicle-stimulating hormone release from pituitary gonadotrophs. However, many cases of IHH have no defined chromosomal abnormality and, in the absence of pedigree analysis, studying the biological mechanisms controlling migration of GnRH neurones through the olfactory system into the developing central nervous system might reveal additional genetic pathways that play a role in the pathogenesis of IHH.

Animals↗
PubMed1999

Posttraumatic smell loss: relationship of psychophysical tests and volumes of the olfactory bulbs and tracts and the temporal lobes.

RATIONALE AND OBJECTIVES: The purpose of this study was to define the primary sites of injury in patients with posttraumatic anosmia and hyposmia with magnetic resonance (MR) imaging and to determine if these sites correlated with the results of psychophysical olfactory tests. MATERIALS AND METHODS: Thirty-six patients with subjective loss in olfaction after head trauma underwent volumetric MR studies of the olfactory bulbs and tracts and temporal lobes. Pearson correlations were computed between olfactory bulb and tract and temporal lobe volumes and the patients' scores on tests of odor identification (including the University of Pennsylvania Smell Identification Test [UPSIT]), detection, and memory. Analysis of variance was used to compare volumes of the control subjects and the posttraumatic patients. RESULTS: The olfactory bulbs and tracts (32 [89%] of 36 patients), the subfrontal lobes (22 [61%] of 36 patients), and the temporal lobes (11 [31%] of 36 patients) showed the highest incidence of posttraumatic encephalomalacia. Left olfactory bulb and tract volumes showed a statistically significant correlation with left and total UPSIT scores. A statistically significant difference (P < .001) was found in the right and left olfactory bulb and tract volumes between anosmic and hyposmic patients and between posttraumatic patients and control subjects. CONCLUSION: Olfactory bulb and tract damage may correlate with deficits in odor identification. Olfactory bulb and tract and frontal lobe encephalomalacia coexist in many patients.

Adolescent↗
PubMed2000

Nasal polyposis: clinical course during 20 years.

BACKGROUND: The etiology of nasal polyposis is mainly unknown although it has been connected with many clinical conditions. The long-term clinical course of nasal polyposis is largely unknown, because long-term followup studies on the recurrence of nasal polyposis have rarely been reported. OBJECTIVE: The aim of the study was to find out the clinical course of nasal polyposis over a long period of time. PATIENTS AND METHODS: Our report describes a 20-year follow-up study of 41 patients with nasal polyps. These patients had surgery for nasal polyp disease 20 years previously and they were initially grouped according to occurrence of (1) acetylsalicylic acid (ASA) intolerance, (2) atopic allergy (AT), and (3) intrinsic allergy-like disease (INTR). Patients were now re-examined, sinus computed tomography (CT) scanning was made, and a biopsy from polyp or from mucosa of the middle turbinate was taken. RESULTS: Anterior rhinoscopy revealed polyps in 35 of 41 patients. Thus nasal polyposis was still active in 85% of patients after 20 years. Mucosal changes in paranasal sinuses were found in every patient. Anosmia or hyposmia was found in 61% (25/41) of the patients. Eight patients had had 11 or more surgical operations during the 20-year period. Of these, 88% (7/8) belonged to the ASA group. Bronchial asthma was found in all ASA intolerance patients (11/11), and in 36% (4/11) of AT and in 16% (3/19) of INTR patients, respectively. CONCLUSION: Because of the high recurrence tendency and insidious symptoms of nasal polyposis, patients will require followup for the rest of their lives.

Administration, Topical↗
PubMed2003

Kallmann's syndrome: molecular pathogenesis.

Kallmann's syndrome (KS) is a genetic condition characterised by hypogonadotrophic hypogonadism (HH) and anosmia; although these are the defining features of the condition, additional neurological and non-neurological sequel may also occur depending on the specific mode of inheritance. KS affects about 1 in 8000 males and 1 in 40,000 females, with most presentations being of the 'sporadic' type. Of the inherited forms, hitherto, only the gene responsible for the X-linked form (X-KS), namely KAL-1, has been identified and the encoded protein, anosmin-1, consists primarily of a whey acidic protein (WAP) and fibronectin-like type III (FnIII) domains which appear to mediate distinctly different protein functions. The WAP/FnIII combination is conserved in anosmins across species and recent studies in rodents and in Caenorhabditis elegans demonstrate that anosmin functions in both axonal targeting and branching. Screening for loci that modify these phenotypes in C. elegans has identified heparan-6-O-sulphotransferase as a key interactor mediating anosmin-1 function. Furthermore, over-expression and loss of function of the C. elegans Kal-1 gene disrupt epidermal morphogenesis, resulting in ventral enclosure and male tail formation defects. These findings provide novel insights into the molecular pathogenesis of X-KS.

Amino Acid Sequence↗
PubMed2005

Luffa operculata effects on the epithelium of frog palate: histological features.

UNLABELLED: Luffa operculata is the botanical name of buchinha-do-norte or cabacinha, which is a medicinal plant widely used for the treatment of rhinitis and rhinosinusitis. In Europe and USA, it is available in homeopathic medicines. In Brazil, Luffa operculata dry fruit infusion is inhaled or instilled into the nose releasing profuse mucous secretion, thus relieving nasal symptoms. Nevertheless, this often may cause irritation, epistaxis or anosmia. STUDY DESIGN: Experimental. MATERIAL AND METHOD: The effects of Luffa operculata were evaluated in different concentration infusions, in isolated frog palate preparation, testing 46 palates after immersion. Four groups (n = 10) were tested with the infusion prepared with frog Ringer (isotonic): control; 60 mg/L; 600 mg/L; and 1200 mg/L. An additional group was tested using the infusion with water (600 mg/L H2O, n = 6). Epithelial samples were harvested to be studied under light microscopy and electron transmission microscopy. RESULTS: In treated palates, light microscopy findings were dose-dependent standard toxic changes. Electron transmission microscopy showed enlargement of intercellular spaces and tight junctions disruption, pointing to ion-fluid transport abnormalities. CONCLUSIONS: Luffa operculata infusion in currently used doses can promote significant structural and ultrastructural changes in the epithelium of this ex vivo model of respiratory mucosa.

Animals↗
PubMed1980

Treatment of allergic and vasomotor rhinitis by the local application of silver nitrate.

A new, simple and effective local treatment of allergic and vasomotor rhinitis is presented. Using 15 per cent silver nitrate, bilateral chemical cautery of the anterior part of the nasal septum and inferior turbinates has been carried out at weekly intervals on 1 to 5 occasions, in 41 cases. No distinction was made between allergic rhinitis and vasomotor rhinitis. 92.6 per cent of cases belonged to the age group of 11 to 50 years. There was a slight predominance of female over male patients in this study. Only those cases were selected who had sneezing as the presenting symptom, while 17 per cent of these patients also suffered from asthma. The eosinophil count in the blood was below 5 per cent in 73.2 per cent of cases, and was above 10 per cent in 9.8 per cent of cases. Eosinophils were present in the nasal secretions of of 66.6 per cent of patients. Intestinal parasites were detected in the stools of 19.5 per cent of cases. No significant quantity of methaemoglobin was detected in the blood of any patient. Silver nitrate was applied after topical anaesthesia with 4 per cent lignocaine solution. The majority of patients had 2 to 4 applications. 68.3 per cent of patients had good relief, particularly from sneezing and watering of the nose. If patients treated with only a single application of silver nitrate are excluded from the series, 79.4 per cent patients reported relief. Significantly, out of 7 cases who were suffering from allergic rhinitis as well as asthma, 57.1 per cent of cases had freedom from the asthma. Among 3 patients who had no relief with earlier intranasal hydrocortisone injections, 66.6 per cent cases had good relief with silver nitrate. One patient who had good relief from allergic rhinitis developed anosmia after this treatment and some patients experienced transient sneezing and rhinorrhoea after the application of silver nitrate. Long-term results are awaited and it is suggested that further studies are necessary regarding the number of applications, the interval between applications, the strength of the silver nitrate solution, and the possible use of other chemical or physical agents for this mode of treatment.

Administration, Topical↗
PubMed1994

Ethmoidal sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease of unknown aetiology. Nasal sarcoidosis commonly affects the mucosa of the septum and the inferior turbinates. These patients may present with nasal discharge, crusting, obstruction, epistaxis or anosmia. We present an unusual case of nasal sarcoidosis involving the ethmoid sinus causing recurrent eyelid swelling and discuss its management.

Adult↗
PubMed1995

Occupational rhinitis--possible mechanisms of pathogenesis.

Occupational rhinitis has been a prescribed industrial disease in the UK since 1907. It has only relatively recently received significant attention from otorhinolaryngologists although numerous studies have been performed in the past by occupational and industrial health physicians. At the present time the precise mechanisms of pathogenesis are unclear and would appear to be multiple. Recently interest has arisen because of compensation claims. Diagnosis made on the basis of the clinical history is subject to two problems: firstly, there is difficulty in differentiating between occupational and nonoccupational rhinitis, and secondly, clinical histories can easily be feigned. Physical signs would be a more reliable indicator of occupational damage to the nasal mucosa if they differ from the signs normally found in allergic or vasomotor rhinitis. In a series of 100 shipyard workers dry atrophic nasal mucosa was found in 66 and septal ulceration in two. From their clinical histories 78 individuals complained of nasal obstruction, 28 of epistaxis, 42 of hyposmia, 10 of anosmia and 90 of rhinorrhoea. Possible pathogenesis is described.

Adult↗
PubMed1997

The endoscopic treatment of cerebrospinal fluid rhinorrhoea: the Nottingham experience.

The conventional neurosurgical management of cerebrospinal fluid (CSF) rhinorrhoea has been by intracranial access. Otorhinolaryngologists have undertaken extracranial approaches where access has been possible, and more recently an endoscopic approach has been advocated. We report 17 patients with confirmed CSF rhinorrhoea treated with endoscopic techniques between 1993 and 1996. Follow-up ranged from four to 32 months and showed an overall closure rate of 16 out of 17 patients with no complications. No patient undergoing primary endoscopic repair developed anosmia. The use of fluorescein helped localize the site of the CSF leak in four patients in whom the computed tomography (CT) scan had not identified the site of the defect. We conclude that the endoscope is a valuable tool in the evaluation and subsequent treatment of CSF rhinorrhoea. Where the skull base defect is accessible to the endoscope, we propose that an endoscopic approach should be considered as the preferred method.

Adult↗
PubMed1983

Pathobiology of neurosarcoidosis and clinicopathologic correlation.

In a retrospective study of 4,294 consecutive postmortem cases, there were 15 with histologically proved sarcoidosis, including 4 with CNS involvement. Whereas in previously reported autopsy series the prevalence of neurosarcoidosis was 15%, it is 27% in this review. Five cases with incidental, clinically inapparent sarcoidosis died at a mean age of 54.6 years; those six with systemic sarcoidosis, not involving the CNS, died at a mean age of 47.0 years; while those four with a variety of neurologic manifestations in addition to systemic sarcoidosis died at a mean age of 38.2 years. Thus, it appears that at one end of the spectrum of morbidity, sarcoidosis may cause no or only trivial symptoms and permit prolonged survival. At the other extreme, sarcoidosis may affect a younger population more severely. CNS involvement occurs relatively early in the course and its rather rapidly progressive, accounting for the poor prognosis, despite appropriate medical and neurosurgical management. In its turn, neurosarcoidosis may present with a variety of signs and symptoms, depending on the site of involvement along the craniospinal axis. This feature is illustrated by the manifestations of compression myelopathy, hydrocephalus with dementia, hydrocephalus with seizures and ataxia, and anosmia, blindness, seizures, and diabetes insipidus. In addition, one patient developed a Nocardia brain abscess as a complication of the altered immune system in sarcoidosis.

Adult↗
PubMed2002

Aroma extract dilution analysis. Precision and optimal experimental design.

The odor thresholds of 12 different compounds have been determined in an AEDA experiment using a panel composed of 8 individuals. Only in one case is the distribution of thresholds among judges significantly different from the log-normal. The cause of that departure from normality seems to be a cross adaptation rather than anosmia. The standard deviations (SD) range from 2(0.7) to 2(4.1), with 2(1.8) as average. If the AEDA is carried out at a dilution rate, R, and dilution R(P) (where P = 0, 1, 2.) is the last one at which a compound was detected by a judge, the flavor dilution (FD) factor that should be provided for that judge is R(P+0.5). In the case where several judges carry out the AEDA, the FD for a given compound should be the geometric mean of the FD provided by all the judges. The SD of the distribution of FDs is related to that of the distribution of odor thresholds, but is strongly influenced by the dilution rate, R, being higher with higher R values. The relationship between both SDs can be used to determine the expected precision (measured as the geometric length of the 95% confidence interval) of the result of an AEDA experiment as a function of the dilution rate, the number of judges, and the SD of the distribution of thresholds. Different simulations have shown that in most cases, a dilution rate of 10 is the best option, and that lower dilution rates are advantageous only if the analyzed solution contains compounds with a very narrow distribution of thresholds.

Chromatography, Gas↗
PubMed2004

Expression of expanded polyglutamine protein induces behavioral changes in Drosophila (polyglutamine-induced changes in Drosophila).

Spinocerebellar ataxia type-3 or Machado-Joseph disease (SCA3/MJD) is an autosomal dominant neurodegenerative disease caused by triplet nucleotide expansion. The expansion of the polyglutamine tract near the C terminus of the MJD1 gene product, ataxin-3, above a threshold of 40 glutamine repeats causes neuronal loss and degeneration. The expanded ataxin-3 forms aggregates, and nuclear inclusions, within neurons, possibly due to the misfolding of mutant proteins. Here we report upon the behavioral test changes related to truncated and expanded forms of MJD protein (MJDtr) in Drosophila, and show that expanded MJDtr, when expressed in the nervous system, causes characteristic locomotor dysfunction and anosmia. This phenomenon has not been previously reported in humans or in transgenic Drosophila models. In addition, the in vivo expression of the antiapoptotic gene bcl-2 showed no evidence of ameliorating the deleterious effect of MJDtr-Q78s, either in the eye or in the nervous system. The study shows that such Drosophila transgenic models express olfactory dysfunction and ataxic behavior as observed in human patients.

Animals↗
PubMed1999

N-isobutyrylcysteine, a donor of systemic thiols, does not reduce the exacerbation rate in chronic bronchitis.

N-isobutyrylcysteine (NIC), a new thiol compound that is not rapidly hydrolysed to give higher levels of free thiols in the body than N-acetylcysteine (NAC), was used to test if the effect of NAC on exacerbations in chronic bronchitis was an effect of the unhydrolysed thiol compound. Smokers or exsmokers with chronic bronchitis forced expiratory volume in one second (FEV1) >40% and reversibility < or = 10% predicted were treated with oral NIC 300 mg b.i.d. or placebo for 24 weeks. Steroids, NAC, antibiotics, and nonsteroid anti-inflammatory drugs use were restricted. Exacerbations were recorded by a respiratory symptom diary card and the time to onset of the first exacerbation after the start of treatment was measured using life-table analysis. Spirometry was performed at each visit. Six hundred and thirty-seven patients were randomized to treatment with NIC (n=316) or placebo (n=321). NIC did not prolong the time to first exacerbation (life-table analysis, p=0.59) and no increase in FEV1 or forced vital capacity was observed. Altered taste perception, taste loss and anosmia occurred more often in the NIC group (p<0.001). In conclusion, N-isobutyrylcysteine, a N-acetylcysteine-like drug with a greater bioavailability has, contrary to N-acetylcysteine, no effect on exacerbations in chronic bronchitis. This suggests that the effect of N-acetylcysteine on exacerbations in chronic bronchitis is not due to the relatively low free thiol levels (other than glutathione) produced by N-acetylcysteine therapy.

Administration, Oral↗
PubMed1991

Olfactory sensitivity: reliability, generality, and association with aging.

Thirty-two Ss between 22 and 59 years of age yielded detection thresholds for 4 odorants over 4 sessions. The thresholds decreased and reliability increased over the course of testing. High intercorrelations between odorants and the stability of an S's relative position within the threshold distributions showed that a general factor of sensitivity dominated the outcome. Age contributed strongly to intersubject variation. Even among these nonelderly individuals, it accounted for up to 2 orders of magnitude in threshold performance. Other important factors included superiority of the right nostril and a negative correlation between the mean and variance of threshold distributions. Scant attention to the correlation may have contributed to overestimation of the frequency and specificity of specific anosmia. A clinically relevant outcome was that measurement of threshold for diagnostic purposes can generally rely on just 1 odorant.

Adult↗
PubMed1983

Role of the infraorbital nerve in retrieving behavior in lactating rats.

Injecting the local lidocaine into the mystacial pads of primiparous rats rendered the snout insensitive to touch and abolished the dams' ability to retrieve. Injecting the drug into the masseter muscles or intraperitoneally did not render the snout anaptic or abolish retrieval, results indicating that the effect of intramystacial lidocaine treatment could not be attributed to systemic toxicity or to the drug's spreading from the mystacial pads to affect the nearby masseter muscles. Cutting the intraorbital nerves produced a temporary retrieval impairment that was indistinguishable from that produced by intramystacial lidocaine injection. Surgical deafferentation did not affect the latency of dams to approach pups that had been displaced from the nest site, which indicates that the retrieval deficit was not due to postoperative debilitation. In addition, infraorbital section did not interfere with the ability to locate and consume a piece of cheese that had been buried in the home cage, which indicates that the operation did not produce anosmia or interfere with the muscles used to grasp pups. Cutting the facial nerves abolished vibrissal movement but did not disrupt retrieval, results indicating that the effect of infraorbital lesions could not be attributed to the loss of vibrissal cues or to nonspecific effects of nerve section. The possibility was tested that infraorbital deafferentation has a profound effect on retrieval because anaptic dams, in their initial attempts at picking up pups, elicit distress vocalizations from their offspring. Dams injected with lidocaine in the mystacial pads failed to retrieve pups that had been anesthetized with a barbiturate to abolish their distress vocalizations. Thus pup-produced vocalizations are not responsible for the retrieval impairment exhibited by anaptic mothers. It is concluded that perioral tactile sensation plays an important role in the ability of lactating rats to retrieve.

Animals↗
PubMed2002

Stimulus control of maternal responsiveness to Norway rat (Rattus norvegicus) pup ultrasonic vocalizations.

Mother rats (Rattus norvegicus; 6 to 8 days postpartum) approach and maintain proximal orientation to a pup that is emitting ultrasonic vocalizations (USVs) far more than do virgin females (W. J. Farrell & J. R. Alberts, 2002). We used a playback regimen to examine the roles of acoustic and nonacoustic cues in regulating maternal proximal orientation toward vocalizing pups. When presented with recorded USVs, mothers of 6- to 8-day-old pups and nulliparous virgin females exhibited equivalent levels of proximal orientation toward the playback speaker. Mothers did show enhanced proximal orientation toward recorded USVs, however, if a silent pup was positioned below the speaker. Pup odors appear to be crucial for the maternal response to vocalizing pups, as peripherally induced anosmia attenuated maternal proximal orientation toward a vocalizing pup. Furthermore, spatial contiguity between olfactory and auditory stimuli was required for a maximal maternal response.

Animals↗
PubMed1977

Evaluation of intranasal zinc sulfate treatment on olfactory discrimination in rats.

The effects of intranasal syringing with 5% zinc sulfate were assessed in rats trained in a wind-tunnel olfactometer to detect the presence of an odor or to discriminate odors of graded intensities. The syringing produced a severe but brief interference in olfactory performance. Full recovery of detection performance occurred within 72 hr after treatment. Initial recovery of odor intensity discrimination occurred in 2-8 days and full recovery within 8-10 days. These results suggest that intranasal syringing with ZnSO4 is not an adequate control for the effects of anosmia produced by olfactory bulbectomy.

Administration, Intranasal↗