Hyposmia after paranasal sinus exenteration of laryngectomy.
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Many people experience some degree of olfactory dysfunction during the acute stages of an upper respiratory infection (URI). The olfactory function usually returns to the preinfection level in few days. In some cases, the dysosmia remains even after the reopening of the air passageways. The olfactory loss after URI associates an hyposmia and odor distortions. We report a rare case of odor distortions after URI treated by surgery.
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BACKGROUND: Deficit syndrome (DS) schizophrenia patients have smooth pursuit eye movement (SPEM) dysfunction. We examined if they also had smell identification deficits, since social affiliation is related to olfaction in other mammals. METHODS: Sixty-seven patients had DS assessments: 31 patients had SPEM and 50 had Smell Identification Test (SIT) assessments, and 14 patients had both measurements. RESULTS: DS patients had worse SPEM and SIT performance than the non-DS patients. Areas under the receiver-operator characteristic (ROC) curves for SIT and SPEM were both fairly accurate in identifying the DS. The odds ratio (OR) for the DS for impaired versus normal SPEM was 6.21 (95% confidence interval [CI]: 1.21, 32.25) and for microsmia versus normosmia was 10.4 (95% CI: 1.23, 88.18). Further analyses showed that the association of SIT with both SPEM and the DS could account for the SPEM-DS association. CONCLUSIONS: We found a strong association between the DS and SIT scores suggesting that the neural substrates of olfaction may be related to social affiliation in humans, as they are in other mammals. These data further support the notion that the DS defines a homogeneous subgroup of schizophrenia patients and further suggest that dysfunction in the neural circuitry of olfaction may contribute to its pathophysiology.
The staggerer mutation is characterized by the disruption of a single recessive gene encoding for the nuclear hormone receptor RORalpha. In addition to the well-studied gene expression in the cerebellum causing massive primary Purkinje cell loss, the RORalpha gene is also expressed in the thalamus and the olfactory bulb. A quantitative histochemical study of cytochrome oxidase activity was performed in staggerer mutants and their respective controls in order to determine whether olfactory bulb neuropathology leads to neuronal metabolic alterations in olfactory and related limbic regions. In the staggerer olfactory bulb, the core and the shell of the glomeruli had lower levels of cytochrome activity, whereas higher levels were found in the external plexiform and granular layers. Other olfactory and limbic regions were unchanged, except for a higher level in the accessory olfactory bulb and a lower level in the most ventral part of the medial orbital cortex. These results are discussed with regard to the olfactory deficits and changes in social interactions previously observed in this mutant.
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Magnetic resonance imaging (MRI) of the pituitary, hypothalamus and olfactory sulci was performed in 40 patients with idiopathic hypogonadotropic hypogonadism (IHH). Twelve of these patients had an impaired sense of smell (Kallmann's syndrome). Sagittal and coronal imaging revealed no morphological abnormalities in the hypothalamic-pituitary region. On axial imaging rudimentary, hypoplastic or aplastic olfactory sulci were found in eight cases. All patients with olfactory sulcal abnormalities had associated hyposmia (one case) or anosmia (seven cases). Structural defects identifiable on MRI were present only in 20 per cent of patients with IHH.
Olfactory function was evaluated by olfactory event-related potentials and standardized psychophysical measures including the Smell Identification Test and odor detection threshold tests for 3 chemosensory stimulants in 9 subjects with isolated congenital anosmia and 9 age- and gender-matched normosmic controls. There was a significant difference in Smell Identification Test scores (P < 0.001) and odor detection thresholds for phenylethyl alcohol (P < 0.001) and isoamyl acetate (P < 0.001) between the anosmic and normosmic subjects. Detection thresholds for chloracetyl phenone, a trigeminal stimulant, did not differ between the 2 groups. Olfactory evoked potentials were recorded in response to amyl acetate and air control stimuli presented at volume flow rate of 5 l/min, stimulus duration of 40 ms, and randomized interstimulus intervals of 6-30 s. In the control subjects, evoked potentials to amyl acetate were characterized by 4 reproducible components (P1, N1, P2, and N2). In the subjects with congenital anosmia, no reproducible evoked potential components were identified in response to amyl acetate. No reproducible evoked potential components were seen in response to the air control stimulus in either the anosmic or normosmic groups. These data suggest that olfactory evoked potentials provide a specific measure of olfactory function.
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Four men with hypogonadotropic hypogonadism and anosmia were tested with acute intravenous injections of luteinizing hormone-releasing hormone (LH-RH) and D-leucine-6-LH-RH-ethylamide (D-L eu-6-LH-RH-EA) with a 1-week interval. Each patient was then treated with this drug for 60 days and tested again after this period with an intravenous injection of D-L eu-6-LH-RH-EA. The administration of LH-RH resulted in a significant increase in the LH level in only one patient and in follicle-stimulating hormone (FSH) and testosterone increases in none. The analog D-Leu-6-LH-RH-EA resulted in significant increases in LH levels in two patients, in FSH levels in three, and in testosterone levels in one. Results obtained after treatment were closely similar to those observed before treatment. Clinical improvement in terms of increased libido, erection, pubic hair growth, and testicular size was observed. D-Leu-6-LH-RH-EA could be useful in the treatment of patients with hypogonadotropic hypogonadism, a possibility deserving further studies.
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Pituitary cytology was studied in a man with untreated Kallmann's syndrome who died at the age of 61. All the adenohypophyseal cells were normal except the gonadotrophs. The identifiable gonadotrophs were scanty and had few and small secretory granules, which gave a weak positivity for beta -follicle-stimulating hormone (FSH) and beta -luteinizing hormone (LH). The tests showed marked hypogenesis, while the adrenals and the thyroid were normal. In the hypothalamus, the lateral tuberal nuclei were undeveloped, and the nucleus subventricularis exhibited nerve cell hypertrophy. The morphologic findings are in accord with the results of endocrine investigations, which showed that the hypogonadism was due to a failure in the secretion of luteinizing hormone-releasing hormone.
Hypogonadotropic hypogonadism with anosmia was found in a 38 year old female. Cardiac abnormalities were manifested by a second degree heart block as well as a conduction delay below the AV node. Striking T wave inversions developed in the absence of any symptoms. These changes probably reflect a central nervous system defect.
Because there has been no suitable diagnostic instrument for evaluation of olfaction in children, we designed an odorant identification test for that purpose. We screened 40 microencapsulated odorants ("scratch 'n' sniff" cards) by randomly grouping them into 40 overlapping sets of five odorants each. Forty-one children, 4 and 5 years of age, tried to identify each test odorant, selecting their responses from among five photographs depicting the substances in the set of odorants. We used the results to select a subset of five odorants (baby powder, bubble gum, candy cane, fish, and orange). To determine how well these odorants could be identified by normal children, we tested another 134 subjects, 3 1/2 to 13 years of age. For children 3 1/2 years to 5 years 4 months of age, the mean (+/- SEM) percentage of correct responses increased from 66% +/- 8% to 92% +/- 2%. Thereafter the mean percentage of correct responses remained at a plateau of about 90%. The 10th percentile for the percentage of correct responses tended to be higher for girls than for boys throughout childhood. We concluded that this set of five odorants can be correctly identified by most normal children 5 years of age or older. The performances of three older subjects with Kallmann syndrome were all subnormal, but the overall efficacy of the test for evaluating children with olfactory deficits needs to be determined.
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