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Olfactory evoked potentials and contingent negative variation simultaneously recorded for diagnosis of smell disorders.

Objective diagnosis of olfaction can be performed by registration of cortical olfactory evoked potentials (OEP) and of contingent negative variation (CNV). The CNV is a negative voltage developing at the vertex after discrimination of one of two smells while the patient is expecting a second stimulus. By an adequate procedure, including a long time window for averaging (2.56 seconds) with appropriate filters, the two tests can be performed simultaneously in a single session of less than 10 minutes. Anosmia is determinable by both OEP and CNV, although CNV shows less variability. On the other hand, CNV requires attention and some cooperation of the patient. Parosmia is accessible by CNV only; two odor qualities presented in random order have to be distinguished. Hyposmia can also be detected; just above the discrimination threshold, CNV amplitudes tend to be large--even enhanced--whereas OEP amplitudes may still be undetectable.

Contingent Negative Variation↗

Hereditary bimanual synkinesis combined with hypogonadotropic hypogonadism and anosmia in four brothers.

A new genetic syndrome of the combined occurrence of hypogonadotropic hypogonadism, anosmia (Kallmann syndrome) and congenital mirror movements in four brothers is presented. Mirror movements were manifest only within the distal parts of the upper extremities and resembled congenital mirror movements described for isolated or familial cases or those occurring in combination with other genetic defects. The hypothesis is supported, that a midline fusion disorder with preponderance of uncrossed pyramidal tract fibers is a major pathogenetic factor for the occurrence of congenital mirror movements.

Adult↗

Early diagnosis of Parkinson's disease.

In idiopathic Parkinson's disease (IPD) approximately 60 % of the nigrostriatal neurons of the substantia nigra (SN) are degenerated before neurologists can establish the diagnosis according to the widely accepted clinical diagnostic criteria. It is conceivable that neuroprotective therapy starting at such an 'advanced stage' of the disease will fail to stop the degenerative process. Therefore, the identification of patients at risk and at earlier stages of the disease appears to be essential for any successful neuroprotection. The discovery of several genetic mutations associated with IPD raises the possibility that these, or other biomarkers, of the disease may help to identify persons at risk of IPD. Transcranial ultrasound have shown susceptibility factors for IPD related to an increased iron load of the substantia nigra. In the early clinical phase, a number of motor and particularly non-motor signs emerge, which can be identified by the patients and physicians years before the diagnosis is made, notably olfactory dysfunction, depression, or 'soft' motor signs such as changes in handwriting, speech or reduced ambulatory arm motion. These signs of the early, prediagnostic phase of IPD can be detected by inexpensive and easy-to-administer tests. As one single instrument will not be sensitive enough, a battery of tests has to be composed measuring independent parameters of the incipient disease. Subjects with abnormal findings in this test battery should than be submitted to nuclear medicine examinations to quantify the extent of dopaminergic injury and to reach the goal of a reliable, early diagnosis.

3-Iodobenzylguanidine↗

Rating of olfactory judgements in migraine patients.

The aim of this study was to evaluate olfactory hypersensitivity (OHS) between attacks in migraine patients. Seventy-four migraine patients and 30 controls were enrolled. The presence of OHS was evaluated using an oral questionnaire and a chemical odour intolerance index. Subjects had to rate the intensity and hedonicity of 12 odourants using a linear rating scale. Twenty-six patients (35.2%) but no control subjects reported an interictal OHS (P<0.001). Logistic regression analysis showed that patients with OHS presented a greater attack frequency, a higher number of odour-induced migraines and visual hypersensitivity when compared with other patients. Disease duration, age, gender and auditory hypersensitivity were not associated with OHS. OHS patients judged odours less pleasant than did other patients and controls, whereas the intensity scores were identical in both groups. OHS between attacks was significantly associated with odour-triggered migraine and an alteration of hedonic judgement.

Adult↗

Aging and life quality: an otorhinolaryngological review.

The increase in the number of people over the age of 65 and the current rise in the proportion of old people represents an important change in the demographic patterns in the world. Since some structural and physiological changes generally occur in elderly patients, maintaining function is surely an important goal. The most devastating disabilities affecting elderly patients are those that diminish the capacity to function independently and those that have a serious impact on the quality of life. Otolaryngologists play a major role within the braod field of geriatrics. Our involvement in caring for the elderly requires us to know how to improve their quality of life. This article provides an overview of many problems that arise within our specialties.

Aged↗

Olfaction and psychiatry.

Recent clinical studies have identified significant olfactory deficits in several neuropsychiatric disorders, notably Alzheimer's disease and Parkinson's disease. These have correlated with neurochemical and neuropathological studies of the olfactory system. The presence of a specific sensory deficit may be related to the localisation of pathology within the brain. There is a need for incorporation of olfactory testing into routine clinical examination.

Brain↗

Olfactory impairment after chronic occupational cadmium exposure.

Disorders of olfaction affect millions of Americans, but the extent to which occupational and environmental exposures contribute to these disorders is unknown. We examined 55 workers with chronic occupational exposure to cadmium fumes in a brazing operation. We estimated cadmium body burden using urinary cadmium levels and assessed cadmium-induced renal damage by urinary beta 2-microglobulin levels. We quantified olfactory function using a standardized test that measured two components of olfaction, butanol detection threshold and odor identification, and compared workers with a reference group. Forty-four percent of the cadmium-exposed workers were mildly hyposmic, and 13% were either moderately or severely hyposmic. In the reference group, 31% were mildly hyposmic, and the rest were normosmic. The workers with both high urinary cadmium levels and tubular proteinuria had the most significant olfactory dysfunction, with a selective defect in odor detection threshold. Our findings suggest that chronic occupational cadmium exposure sufficient to cause renal damage also is associated with impairment in olfactory function.

Adult↗

Neuroimaging in patients with olfactory dysfunction.

This review summarizes neuroimaging findings in patients with olfactory dysfunction. Neuroimaging techniques offer a valuable means for evaluating and distinguishing disorders of olfaction. The new or refined techniques make it possible to pinpoint the anatomic and pathologic changes of many disorders of the sinonasal cavity and brain that cause olfactory deficits. From an anatomic point of view, the causes of olfactory deficits generally can be classified as peripheral or central (intracranial). In the assessment of the peripheral causes, CT and MR imaging reveal anatomic information and structural changes, enable a differential diagnosis, and provide a road map for surgical intervention. In the evaluation of the central causes, MR imaging, positron emission tomography, or single-photon emission computed tomography can provide the links between olfactory dysfunction and structural or functional changes in the brain.

Brain Diseases↗

Neuropsychological functioning associated with high-altitude exposure.

This article focuses on neuropsychological functioning at moderate, high, and extreme altitude. This article summarizes the available literature on respiratory, circulatory, and brain determinants on adaptation to hypoxia that are hypothesized to be responsible for neuropsychological impairment due to altitude. Effects on sleep are also described. At central level, periventricular focal damages (leuko-araiosis) and cortical atrophy have been observed. Frontal lobe and middle temporal lobe alterations are also presumed. A review is provided regarding the effects on psychomotor performance, perception, learning, memory, language, cognitive flexibility, and metamemory. Increase of reaction time and latency of P300 are observed. Reduced thresholds of tact, smell, pain, and taste, together with somesthetic illusions and visual hallucinations have been reported. Impairment in codification and short-term memory are especially noticeable above 6,000 m. Alterations in accuracy and motor speed are identified at lower altitudes. Deficits in verbal fluency, language production, cognitive fluency, and metamemory are also detected. The moderating effects of personality variables over the above-mentioned processes are discussed. Finally, methodological flaws found in the literature are detailed and some applied proposals are suggested.

Adaptation, Physiological↗

Preclinical diagnosis of Parkinson's disease: are we there yet?

Preclinical Parkinson's disease (PD) can be defined as a state that precedes the diagnosis of PD but without the presence of the characteristic motor features of the disorder. In such a situation, subtle non-motor features may be present or subclinical abnormalities may exist that can be detected only by physiologic testing. Alternatively, lifelong traits such as genetic mutations may predict the onset of PD in the absence of any clinical or physiologic abnormalities. A number of diagnostic technologies are currently available that can detect both preclinical states and traits that will lead to PD in the future. The current challenges are to refine these technologies and to determine how they should be employed so that their use is ethical, practical, and meaningful to at-risk individuals.

3-Iodobenzylguanidine↗

A review of olfactory dysfunctions in man.

Although a large number of individuals experience olfactory disorders following accidents, disease states, medical interventions, aging, and exposure to environmental chemicals and pollutants, few medical practitioners have the expertise or staff to provide appropriate clinical evaluation, treatment, counseling, or referral for such patients. The present review examines studies associated with the diagnosis and treatment of olfactory disorders, as well as ones noting olfactory signs as diagnostic markers for brain tumors and other serious problems. A basic taxonomy of smell dysfunctions is presented, along with a review of etiologic factors, including local diseases and mechanical obstruction of the airways, viral infections, trauma, congenital anomalies, endocrine disorders, tumors, psychiatric disorders, aging, drugs, environmental and industrial pollutants, iatrogenic factors, and miscellaneous diseases. A discussion of current disability compensation guidelines in the United States and Britain is also presented.

Aging↗

Early community outreach intervention in children with acquired brain injury.

Ten patients with acquired brain injury were recruited over an 18 month period in the south-western health care region of Sweden in order to evaluate the costs and effectiveness of a multidisciplinary community outreach intervention programme. An experienced multidisciplinary project team was involved and patients underwent detailed functional, cognitive and motor assessments following initial contact within two weeks of injury, within six weeks of injury and at a 12-month follow-up. An individualized counselling programme was also offered. Of an expected recruitment number of 50 patients (based on epidemiological and population based figures) 10 children were reached, evaluated and followed; eight patients with traumatic brain injury (five severe, two moderate and one mild), and two patients with non-traumatic brain injury (both severe). At follow-up there was a significant improvement in motor function. No significant changes were seen in other areas of functional assessment or on neuropsychological measures although there were mild improvements in communication and behaviour functions. The financial costs per patient in the programme were deemed relatively modest compared with cost estimates of shorter-term in-patient rehabilitation. Time intensive interventions included supporting caregivers and school staff and the direct and indirect patient interventions were shown to enhance support and promote active involvement of local services.

Adolescent↗