More on X-linked ichthyosis, steroid sulfatase deficiency, and hypogonadism and anosmia.
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Odor identification ability among patients with Sjögren's syndrome.
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Adult↗
Twenty-four-hour-old lambs rely more on maternal behavior than on the learning of individual characteristics to discriminate between their own and an alien mother.
Lambs can discriminate their own mother from an alien dam on the first day of life, suggesting the recognition of individual physical characteristics of the mother. Alternatively, their choice may depend on behavioral differences existing between the ewes because of their maternal selectivity. To clarify this, the ability of 24-hr-old lambs to discriminate between their own and an alien mother, that were either intact and accept only their own lamb at nursing (i.e., selective, n = 19) or anosmic, which nurse indiscriminately alien lambs as well as their own (i.e., nonselective, n = 24), was assessed by a 5-min, two-choice test. With intact dams, lambs spent significantly more time next to their own mother whereas this was not so in the presence of anosmic dams. Furthermore, in the intact group, the vocal activity by their own mother differed from that by the alien dam while this was not so in anosmic ewes. We conclude that 24-hr-old lambs rely more on the behavior of the ewes to select their dam than on their individual physical characteristics.
Animals↗
Olfaction and the homing ability of pigeons raised in a tropical area in Brazil.
Several workers have investigated the effect of anosmia on pigeon navigation in different geographical locations because it has been suggested that homing behavior is based on different cues, such as olfactory cues, the Earth's magnetic field or infrasound, and that in the absence of one cue another would be used. In this situation, no cue is universally indispensable, including olfactory ones. In order to extend such observations to a novel biome, we observed the behaviour of 192 young inexperienced birds raised in southeastern Brazil, a tropical area where olfactory tests had never been run before. The birds were released from eight symmetrically distributed sites 17 to 44 km from the loft. Half of these birds (experimentals) had been made temporarily anosmic by washing their olfactory mucosae with 4% solution of ZnSO4 the day before release, while controls were treated with Ringer solution. The results of release tests showed that anosmia totally impaired the navigational performance of experimental birds, which were unable to home from sites at relatively short distances from home (34-44 km) and whose pooled initial bearings produced a (negative) homeward component not significantly different from 0. Homing performance of controls was significantly better, and their pooled vanishing bearings had a significant homeward component, in spite of much scatter in individual releases. We conclude that pigeon homing in the study area depends on olfactory information, even though local environmental conditions in the interior of the State of Sao Paulo, as in several other parts of the world, do not appear to be as favorable as Italy for the development of efficient olfactory navigation.
Animals↗
Pineal-induced alterations in reproductive function and pituitary prolactin in the female rat: the effects of bilateral superior cervical ganglionectomy and nervi conarii transection.
The reproductive organs of female rats subjected to blinding and and anosmia were hypotrophic while pituitary prolactin stores were markedly depressed in these animals. All of the effects of dual sensory deprivation were reversed by either superior cervical ganglionectomy or nervi conarii transection. It was concluded that both of these surgical procedures were as effective as pinealectomy in reversing the pineal-induced alterations in the reproductive physiology of the blind-anosmic female rat.
Animals↗
Postoperative taste and smell deficit after upper gastrointestinal cancer surgery--an unreported complication.
BACKGROUND AND OBJECTIVES: Patients undergoing upper gastrointestinal cancer surgery were noted to suffer loss of taste and/or smell, a previously unreported problem. Our aim was to investigate the extent of this phenomenon, quantify recovery time, and identify potentially associated factors. METHODS: In this retrospective study, a postal questionnaire was sent to all patients still alive after oesophagectomy or gastrectomy, with a minimum 1-year follow-up and no clinical or radiological evidence of recurrence. Data were analysed for prevalence of deficit in relation to operation, age, sex, respiratory complications, and disease stage. RESULTS: A total of 109/119 (92%) patients completed the questionnaire: 50 gastrectomies and 69 oesophagectomies. Ten patients were excluded with prior sensory deficit. Overall, 45/99 patients (45%) suffered deficit (M:F = 1.6:1). No association was found with type of surgery: deficits for subtotal gastrectomy, total gastrectomy, and oesophagectomy were 44, 46 and 46% respectively (chi(2) = 0.355, 2 df P > 0.5). No other parameter was associated, and full recovery occurred in 30 patients (67%) within a mean of 6 months. CONCLUSIONS: Loss of taste and smell occurs in nearly one-half of all cases after upper gastrointestinal surgery. The pathophysiology is unknown, but it resolves in most patients within 6-12 months. This complication should be discussed as part of informed consent for patients undergoing oesophagogastric cancer surgery.
Adult↗
Olfaction in essential tremor patients with and without isolated rest tremor.
An olfactory deficit is present in patients with essential tremor (ET), but it is often milder than that in patients with Parkinson's disease (PD). In both, the deficit occurs early in the disease. Isolated rest tremor without other signs of parkinsonism can occur in patients with ET. If the rest tremor in these patients represents a manifestation of ET rather than early PD, we hypothesized that their University of Pennsylvania Smell Identification Test (UPSIT) scores would be similar to those of ET patients without rest tremor. The mean UPSIT score in 13 ET patients with isolated rest tremor did not differ from that of 58 ET patients without rest tremor (29.3 +/- 4.3 vs. 29.4 +/- 6.4; P = 0.69). Several ET patients with rest tremor had UPSIT scores that fell outside of the range that is seen in 95% of patients with PD. These data raise the possibility that some ET patients with isolated rest tremor may not have early PD and that the pathological process that is responsible for their ET is also involving the basal ganglia.
Aged↗
A 100% increase of dopaminergic cells in the olfactory bulb may explain hyposmia in Parkinson's disease.
Hyposmia is one of the most prevalent symptoms of Parkinson's disease. It may occur even before the motor symptoms start. To determine whether the olfactory dysfunctions, like the motor symptoms, are associated with a loss of dopamine, the number of dopaminergic cells in the olfactory bulb of Parkinson's disease patients was studied using tyrosine hydroxylase immunohistochemistry. The quantitative analysis reveals that the total number of tyrosine hydroxylase-immunoreactive neurons in the olfactory bulb is twice as high in Parkinson patients compared to age and gender-matched controls. Because dopamine is known to inhibit olfactory transmission in the olfactory glomeruli, we suggest that the increase of dopaminergic neurons in the olfactory bulb is responsible for the hyposmia in Parkinson patients. The increase of dopamine in the olfactory bulb explains why olfaction does not improve with levodopa therapy.
Aged↗
Detection of presymptomatic Parkinson's disease: combining smell tests, transcranial sonography, and SPECT.
Olfactory loss is among the early signs of Parkinson's disease (PD). We investigated whether "idiopathic" olfactory dysfunction might relate to signs of nigral degeneration. Olfactory tests were combined with transcranial sonography of the substantia nigra and single photon emission computed tomography (SPECT) imaging. Thirty patients diagnosed with idiopathic olfactory loss participated. Eleven of these patients exhibited an increased echogenicity of the SN in the transcranial sonography. In 10 of these 11 patients, SPECT scans with (123)I-FP-CIT were performed. Median uptake ratios in the basal ganglia were pathological in 5 patients, 2 patients exhibited borderline findings, and 3 patients had normal results. Considering patients with idiopathic olfactory dysfunction, noninvasive transcranial sonography seems to be helpful in identifying patients potentially at risk to develop PD. Longitudinal follow-up studies are necessary to estimate the ratio of patients with dopaminergic cell loss in the basal ganglia who will develop PD in the future.
Adult↗
Olfaction testing in patients with tremor-dominant Parkinson's disease: is this a distinct condition?
There is considerable controversy regarding the relationship between essential tremor (ET) and Parkinson's disease (PD), especially when tremor is the dominant feature of PD or there is a family history of tremor. Reduced olfaction function is one of the initial signs of PD. In contrast, ET has relatively preserved olfaction. To infer whether the tremor-dominant subgroup of PD is intrinsically different from mainstream PD, we tested olfaction using the University of Pennsylvania Smell Identification Test-40 (UPSIT) in this group and compared the findings with those of patients with non-tremor-dominant "regular" PD. We then evaluated predictors of reduced UPSIT scores within the tremor-dominant group. Overall, olfaction did not differ between tremor-dominant PD and regular PD; however, the subgroup of tremor-dominant PD with a family history of tremor had less olfaction loss than those without a family history (P = 0.0007) or those with regular PD (P = 0.0350). Other clinical features of this tremor-dominant PD with a family history of tremor group mostly resembled those without a family history. This finding suggests that patients with a family history of tremor may represent a different disease process even though, aside from differences in olfaction, they are clinically similar to other patients with tremor-dominant parkinsonism. It additionally suggests phenotypic overlap between PD and ET.
Aged↗
Smell identification ability in twin pairs discordant for Parkinson's disease.
Olfactory dysfunction has been proposed to be a sign that may precede the motor features of Parkinson's disease (PD). To determine whether smell identification deficits predict subsequent PD, we studied smell identification ability using the University of Pennsylvania Smell Identification Test (UPSIT) in 62 members of male twin pairs discordant for PD at baseline. Smell identification ability was reduced at baseline in the twins with PD compared to their unaffected brothers (23 vs. 31 of 40; P = 0.001). UPSIT scores were not reduced in the twins without PD when compared to age- and gender-specific normal values. After a mean interval of 7.3 years, 28 unaffected twins were still alive and 19 agreed to a second evaluation. Two had newly developed PD. Neither twin had impaired smell identification at baseline. The average decline in UPSIT percentile scores in these 2 twins was greater than in the 17 twins who did not develop PD (-68% vs. -24%; P = 0.01). In subjects who did not meet Core Assessment Program for Intracerebral Transplantations diagnostic criteria for PD at baseline, the presence of cardinal signs of parkinsonism was not associated with lower baseline UPSIT scores nor with a subsequent decline. Smell identification ability may not be a sensitive indicator of future PD 7 or more years before the development of motor signs, even in a theoretically at-risk population.
Aged↗
Association of olfactory dysfunction with incidental Lewy bodies.
Olfactory dysfunction is found in early Parkinson's disease (PD) and in asymptomatic relatives of PD patients. Incidental Lewy bodies (ILB), the presence of Lewy bodies in the brains of deceased individuals without a history of PD or dementia during life, are thought to represent a presymptomatic stage of PD. If olfactory dysfunction were associated with the presence of ILB, this would suggest that olfactory deficits may precede clinical PD. The purpose of this study was to determine the association of olfactory dysfunction during late life with ILB in the substantia nigra or locus ceruleus. Olfaction was assessed during the 1991-1994 and 1994-1996 examinations of elderly Japanese-American men participating in the longitudinal Honolulu-Asia Aging Study. Among those who later died and underwent a standardized postmortem examination, brains were examined for Lewy bodies in the substantia nigra and the locus ceruleus with hematoxylin and eosin stain. Lewy bodies in the brains of individuals without clinical PD or dementia were classified as ILB. There were 164 autopsied men without clinical PD or dementia who had olfaction testing during one of the examinations. Seventeen had ILB. The age-adjusted percent of brains with ILB increased from 1.8% in the highest tertile of odor identification to 11.9% in the mid-tertile to 17.4% in the lowest tertile (P = 0.019 in test for trend). Age-adjusted relative odds of ILB for the lowest versus the highest tertile was 11.0 (P = 0.02). Olfactory dysfunction is associated with ILB. If incidental Lewy bodies represent presymptomatic stage of PD, olfactory testing may be a useful screening tool to identify those at high risk for developing PD.
Aged↗
The anterior olfactory nucleus in Parkinson's disease.
Impaired olfaction occurs in patients with idiopathic Parkinson's disease (PD), and Lewy bodies have been found in the olfactory bulb and tract. We now confirm the latter finding and show that this presence of Lewy bodies is associated with significant neuronal loss. A quantitative study of the anterior olfactory nucleus (AON) was performed in tissue obtained postmortem from seven patients with PD and seven age-matched controls. Neuronal loss was seen in the PD bulb and tracts (p < 0.01), and a strong correlation of neuronal loss with disease duration was detected (R = -0.87). The presence of Lewy bodies was confirmed with immunocytochemical staining for ubiquitin in all the PD cases.
Aged↗
Clinical assessment of olfactory dysfunction in Parkinson's disease.
We used two simple tasks to test the capacities of patients with Parkinson's disease to discriminate and identify olfactory stimuli. The patients presented defective odor identification abilities whereas their capacity to discriminate between odors was apparently unaffected. This raises a question about the nature of olfactory dysfunction in Parkinson's disease. Further clinical data is required for analysis of this dysfunction. We therefore propose simple and rapid tests appropriate for clinical use with Parkinson's disease patients.
Adult↗
Distribution of GluR1 is altered in the olfactory bulb following neonatal naris occlusion.
The olfactory system is well suited for studies of glutamate receptor plasticity. The sensory neurons are glutamatergic, and they turn over throughout life, and the olfactory bulb neurons that process their inputs express many of the known glutamate receptor subunits. Neonatal naris occlusion alters olfactory bulb development and the expression of certain neuroactive substances and receptors, at least in part due to loss of the sensory inputs. We therefore postulated that neonatal naris occlusion might alter glutamate receptor expression during postnatal development. Single nares of newborn mice were occluded on postnatal days 1-2, and the distribution of glutamate receptor subunits was evaluated using immunoperoxidase methods. Light microscopic examination on postnatal day 6 failed to reveal adult-like staining of neuronal cell bodies in the olfactory bulbs. By day 12, cell bodies that were immunoreactive (-IR) for the GluR1 subunit were visible in the external plexiform layer (EPL) of both sides. By day 18, many of the GluR1-IR cell bodies could be identified as cell types that had previously been reported to express homomeric GluR1 receptors. Analysis of single, mid-dorsal sections from 18-25-day-old mice showed that the medial EPL of the occluded side had a significantly lower density of these cell bodies. The GluR1 staining of the adjacent mitral cell layer (MCL) was also heavier on the occluded side, but no gross differences in staining for other glutamate receptor subunits were observed. Neonatal naris occlusion therefore appears to provide a new model for studying expression of GluR1 receptors during the development of a discrete population of olfactory bulb neurons.
Animals↗
On the trigeminal percept of androstenone and its implications on the rate of specific anosmia.
Specific anosmia is a term that describes an inability to perceive a particular odorant in the context of an otherwise normal olfactory acuity. The most common example, for the odor of androstenone, has been ascribed a prevalence ranging from 2 to 45%. In two experiments we sought to determine whether this wide range could be explained by the difference in steroid concentrations used, and by the degree to which the trigeminal system contributes to perception of androstenone. Experiment 1 demonstrated that high concentrations of androstenone stimulated the trigeminal system, as indicated by electrophysiological recordings. Experiment 2 demonstrated that conscious detection of androstenone is possible based solely on the trigeminal system. Interestingly, detection seems to interact with olfactory acuity in that subjects with a low olfactory sensitivity to androstenone were better able to detect its trigeminal component. The agreement between conscious experience and behavioral discrimination was not well calibrated, in that subjects demonstrated a clear overconfidence in their abilities. Altogether, the current study suggests that androstenone is an odorant that produces a concentration-dependent degree of trigeminal stimulation. This trigeminal component explains the diversity of the reported prevalence of specific anosmia for androstenone and might have implications on future use of specific anosmia as a tool to understand odor processing.
Adult↗
Olfactory disorder in motor neuron disease.
In Parkinson's disease and Alzheimer's disease there is profound disorder of olfaction. The extent to which this modality is involved in motor neuron disease (MND) has been studied little. To address this further we assessed olfaction by three methods-a smell identification test ("UPSIT") in 58 patients and 135 controls; olfactory-evoked response (OEP) to H2S in 15 patients, and pathological examination of olfactory bulbs obtained from 8 cadavers. It was found that smell identification compared with the controls was slightly worse overall in the MND group as a whole, but only the bulbar patients scored significantly less on the UPSIT. Patients displayed a subtle defect in cheese odor recognition. OEPs were normal in 9 subjects and delayed in 1 subject. The remaining 5 OEPs were unsuccessful. Histopathological studies of olfactory bulbs showed excess lipofuscin deposition in all 8 cases examined, indicating subclinical neuronal damage. Olfactory neurons with a degree of antioxidant defect may be more susceptible to cellular damage than other neuronal groups because of their direct relationship to environmental agents. Overall we found the degree of olfactory dysfunction in MND to be mild and in contrast with the marked changes described by others.
Adolescent↗
Characterization of olfactory deficits in the rat following administration of 2,6-dichlorobenzonitrile (dichlobenil), 3,3'-iminodipropionitrile, or methimazole.
The histopathology of the olfactory mucosal lesion associated with ip administration of 2,6-dichlorobenzonitrile (dichlobenil) and 3,3'-iminodipropionitrile (IDPN) has been well documented. Whether there is an olfactory deficit associated with the partial loss of the olfactory mucosa (localized around the dorsal medial meatus of the nasal cavity) has yet to be determined. Dichlobenil (100 mg/kg) or IDPN (200 mg/kg) was administered ip to adult male Long-Evans rats previously trained in an olfactory task to find a food pellet buried in approximately 7.5 cm of bedding in a 0.61 x 1.2 x 0.61-m Plexiglass chamber. As a positive control, another group received 300 mg/kg ip of 1-methyl-2-mercaptoimidazole (methimazole), a dosing regimen which destroys nearly all of the olfactory mucosa. All three compounds caused a transient increase in the mean latency to find the pellet, with the magnitude of the effect positively correlated with the extent of the olfactory lesion. In order to determine whether these deficits resulted from olfactory dysfunction or impaired cognitive function (a deficit previously attributed to IDPN exposure), another group of rats was dosed as above and tested in another spatial memory task, the Morris water maze (MWM), which is less dependent upon olfactory function. No performance deficit was detected in the MWM. These data suggest that the transient olfactory deficit in the dichlobenil-, IDPN-, and methimazole-treated rats is attributable to defective olfactory function.
Animals↗