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[Olfactory disorders in hypogonadism].

On the basis of the study and particularly of the examination of the ofactive functions in three patients with hypogonadismus the relationship between the olfactive and the endocrine lesions is established. This syndrome, described by Kalmann and by De Morsier is not well known, but it deserves attention since it is indicative of pathologic changes in the region which lies between the hypothalamus and the hypophysis.

Humans↗

Identifying normosmics: a comparison of two populations.

An olfactory function test developed by the Connecticut Chemosensory Clinical Research Center (CCCRC) was administered to 65 normal subjects to determine its ability to identify normosmic subjects. The CCCRC test categorizes individuals into five distinct levels of olfactory functioning according to composite score ranges derived from odor identification and butanol threshold subtests. These categories are: normosmic (normal); mildly, moderately, and severely hyposmic (impaired functioning); and anosmic (no sensation). Comparing score distributions from CCCRC with ours revealed that a greater percentage of our subjects were categorized as hyposmic because of higher butanol thresholds. The butanol threshold subtest was studied further to determine test-retest reliability and normal day-to-day variability in scores. Norms are the basis for interpreting test scores and are important in diagnosing and treating smell disorders.

Adolescent↗

Cilia-related diseases.

There are at least eight categories of cilia in the human body and malfunctioning of any one or several of them will have different consequences for the patient. A genetic error of the respiratory cilia (9 + 2) is the cause of the airways disease named immotile-cilia syndrome (or PCD), whereas defective ependymal cilia (9 + 2) carries an increased risk of hydrocephalus. When the so-called nodal cilia (9 + 0) of the early embryo are malfunctioning, there is a random determination of asymmetry of the heart and visceral organs ('a 50% risk of situs inversus'). Some genes are responsible for the synthesis, transport, and assembly of the cilia, and mutations in these genes may lead to progressive degeneration of ciliary structures, such as the connecting cilium (9 + 0) of the photoreceptor cells-this is the cause of retinitis pigmentosa. Ciliary malfunctions due to genetic errors tend to be systemic and life-long, whereas acquired diseases are local and may be temporary only.

Cilia↗

Development of the University of Pennsylvania Smell Identification Test: a standardized microencapsulated test of olfactory function.

The development of the first standardized "scratch 'n sniff" olfactory test is described. Over 1600 subjects participated in five experiments. In Experiment 1, 50 microencapsulated odorants were rated as to their intensity, pleasantness, irritation, coolness, and familiarity, and two procedures for releasing them were compared. In Experiment 2, the results of the first experiment and other data were used in the development of the test, which was administered to a large number of subjects. Using multiple regression analysis, scores on this test were shown to be significantly related to the subjects' gender, ethnic background, and smoking behavior. Average test scores decreased as a function of age, with the greatest decline occurring between the sixth and tenth decades of life. These age-related changes were not correlated with scores on the Wechsler Memory Scale. Women performed better than men within all age categories. In Experiment 3, the test was shown to differentiate between subjects with known olfactory disorders (e.g., Kallmann's syndrome; Korsakoff's syndrome) and normal controls, and to reliably detect persons instructed to feign total anosmia. In Experiment 4, the test-retest reliability was established (6-month interval; r = 0.918, p less than 0.001), and in Experiment 5 the test was shown to correlate thresholds with odor detection (r = -0.794, p less than 0.001). This self-administratered test now makes it possible to rapidly and accurately assess general olfactory function in the laboratory, clinic, or through the mail without complex equipment or space-consuming stores of chemicals.

Adolescent↗

Rating of different olfactory judgements in Alzheimer's disease.

Using simple successive tasks we assessed the influence of Alzheimer's disease on the processing of different odours. Fifteen patients with Alzheimer's disease, 15 old control subjects and 15 young control subjects were tested. The experiment included two sessions. Initially 12 odorants were presented, one odorant every minute. For each odour the subjects were asked to rate intensity, pleasantness, familiarity and edibility using linear rating scales. The odorants were then presented a second time and the subjects were asked to identify them. The results show that the intensity scores were lower in old control subjects and Alzheimer patients than in the young control subjects and that familiarity and identification scores were lower in Alzheimer patients than in old control and young control subjects. When we compared the five olfactory tasks the impairment of performance in Alzheimer patients was relatively higher for identification than familiarity, itself higher than the intensity judgement. No difference was observed between the three groups of subjects for pleasantness and edibility judgements.

Adult↗

[Parkinson's disease and anosmia in monozygotic twin sisters (author's transl)].

Monozygotic twin sisters developed Parkinson's disease and anosmia at the age of 39. The disease was kept under control and regressed with L. Dopa. Two families with the same association had been previously reported. An anomaly of the metabolism of dopamine, genetically determined, is probably responsible for these disorders.

Adult↗