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Biomedical subjects

B Neundörfer

Publications and source records attributed to B Neundörfer.

At least 181 records · Page 10Linked to original sources

[Friedreich disease. A neurocardiologic syndrome with uncertain nosologic classification of heart involvement].

Friedreich's ataxia (FA) represents a degenerative, genetically determined disease of the nervous system in combination with myocardial affection and in some cases endocrinological disturbances. Manifestation of myocardial involvement usually follows symptoms of nervous system degeneration later in the course, but seems not to be secondary. These cardiac disturbances are the main cause of death in FA-patients. Therapeutic management of heart disease is possible and interdisciplinary neurologic-cardiologic cooperation should start early in the course of FA.

Adult↗

Neurotoxicity of long-term low-level exposure to carbon disulphide: results of questionnaire, clinical neurological examination and neuropsychological testing.

OBJECTIVE: Carbon disulphide (CS2) is highly neurotoxic. There is ample evidence of damage to the peripheral and central nervous system. The air concentration at which such adverse effects can first be observed is presently a subject of controversy. METHODS: In a cross-sectional study of CS2-exposed workers from the viscose industry and healthy controls, data on neurological complaints, basic laboratory diagnosis, clinical neurological examination and neuropsychological testing were evaluated. Data were from 222 workers in the viscose industry exposed to CS2 and 191 employees from the same factory with similar physical and psychological stress factors but without occupational contact with neurotoxic substances. Multiple linear or multiple logistic regression analysis was used to check for statistical differences. RESULTS: The median of the CS2-measurements using personal air sampling was below the current maximum concentration permissible (MAK value) in Germany (10 ppm) in all departments. The threshold limit value was, however, exceeded in almost 10% of the persons investigated. Exposure fluctuated between < 0.2 and 65.7 ppm (median of all departments was 4.02 ppm). As a parameter of internal exposure, CS2-metabolite 2-thio-1,3-thiazolidine-4-carboxylic acid (TTCA) concentrations in the urine of the exposed persons were between < 0.16 and 10.9 mg/g creatinine (median 1.43 mg/g). CONCLUSIONS: Neither an increase in subjective complaints nor an increase in pathological findings in clinical-neurological and neuropsychological examination could be found in persons exposed to CS2 at the exposure levels described.

Adult↗

Electrophysiological investigation of central, peripheral and autonomic nerve function in workers with long-term low-level exposure to carbon disulphide in the viscose industry.

OBJECTIVE: Neurotoxicity of carbon disulphide (CS2) is well known. The air concentration at the workplace at which such adverse effects can first be observed is the subject of controversial discussion. METHODS: In a cross-sectional study on CS2-exposed workers peripheral motor and sensory nerve conduction studies, somatosensory evoked potentials, thermotesting and investigation of forced respiration sinus arrythmia have been carried out. The data from 222 workers exposed to CS2 in the viscose industry were evaluated and compared with data from 191 employees from the same factory with similar physical and psychological stress factors but without detectable occupational contact to neurotoxic substances. Median exposure to CS2 was below the currently valid occupational-medical threshold limit value (MAK-value) of 10 ppm. Multiple linear or multiple logistic regression analysis was used to check for statistical differences. RESULTS: Binary evaluation (comparison of exposed persons versus controls after multiple linear regression) revealed a slightly lower value in the exposed group for the motor nerve conduction velocity (MNCV, -0.76 m/s, median 48 m/s), but a long way from pathological thresholds. No dose-response relationship could be found within the exposed group for any evaluation criteria of CS2-exposure. Somatosensory evoked potentials, thermotesting and analysis of heart rate variability yielded no indication of a neurotoxic effect of CS2. CONCLUSION: Isolated decrease of MNCV in binary evaluation is, with regard to the known mechanism of CS2-neurotoxicity and the lack of a dose-response relationship, obviously not due to toxic effects. We interpret our results as showing that an adverse effect of carbon disulphide at the exposure ranges found was not detectable in the exposed group.

Adult↗

Sudomotor function in sympathetic reflex dystrophy.

Sudomotor functions were studied in 27 patients suffering from reflex sympathetic dystrophy (RSD) according to the criteria established by Bonica (18 women, 9 men; mean age 50 +/- 12.3 years; median duration of disease 8 weeks, range 2-468 weeks). To measure local sweating rates, two small chambers (5 cm2) were affixed to corresponding areas of hairy skin on the affected and unaffected limbs. Dry nitrogen gas was passed through the chambers (270 ml/min) and evaporation was recorded at both devices with hygrometers. Thermoregulatory sweating (TST) was induced by raising body temperature (intake of 0.5 1 hot tea and infra-red irradiation). Local sweating was also induced through an axon reflex (QSART) by transcutaneous iontophoretic application of carbachol (5 min, 1 mA). In addition, skin temperature was measured on the affected and unaffected side by infra-red thermography. Mean skin temperature was significantly higher on the affected side (P < 0.003). In spite of the temperature differences, there was no difference in basal sweating on the affected and unaffected side. However, both methods of sudomotor stimulation lead to significantly greater sweating responses on the affected compared to the unaffected side (TST: P < 0.05, QSART: P < 0.004). Latency to onset of sweating was significantly shorter on the affected side under both test conditions (P < 0.04 and P < 0.003, respectively). Sweat responses were not correlated to absolute skin temperature but were probably related to the increased blood flow on the affected side. Our findings imply a differential disturbance of vasomotor and sudomotor mechanisms in affected skin. Whereas vasoconstrictor activity is apparently lowered, sudomotor output is either unaltered or may even be enhanced.

Axons↗

Clinical and morphological phenotype of HMSN 1A mosaicism.

Clinical, neurophysiological and morphological studies on a patient with mosaicism of the 17p11.2 duplication were performed in detail for the first time. Since duplication occurs during paternal meiosis, a somatic reversion is suggested, leading to mosaicism. The proportion of nuclei with duplication varied markedly between 49% in blood cells and 74% in tissue from the sural nerve. Clinically, mild symptoms of a motor and sensory neuropathy were present. However, neurophysiological changes and findings in the sural nerve biopsy were consistent with a typical hereditary motor and sensory neuropathy type 1 (HMSN 1). Differing clinical findings in patients with mosaicism of the 17p11.2 duplication may be explained by a varying degree and/or time of reversion.

Adult↗

Cutaneous norepinephrine application in complex regional pain syndrome.

Patients with complex regional pain syndrome (CRPS) (n=20) were examined in order to evaluate cutaneous reactions to norepinephrine (NE) on both the affected and the unaffected limb in comparison to healthy controls. Sixteen female and four male patients suffering from very acute and therefore untreated CRPS with a mean duration of 5.5 weeks were included in this study. Two groups of healthy volunteers served as control groups: the first group (n=18) according to the same study protocol as CRPS patients, and the second group (n=10) after warming up one limb. Norepinephrine was iontophoresized (0.2 mA, 120 s) and vasoconstriction was recorded by laser-doppler flowmetry. Pain sensations were simultaneously rated on a visual analogue scale (VAS). Five patients underwent a second trial with higher intracutaneous NE concentrations in order to study possible dose-dependent effects of NE on pain sensation. After acclimatization, skin temperature was recorded by infra-red thermography. The NE-induced reduction of skin blood flow was significantly higher in the affected limb in the patient group (33.0 vs 11.2%, p<0.005). None of the patients reported pain or hyperalgesia. The skin temperature of CRPS patients was significantly higher in the affected limb (34.7 vs 32.5 degrees C, p<0.001). The first control group did not show any difference between left and right sides concerning NE-induced vasoconstriction or skin temperature. The second control group had an increased unilateral skin temperature after warming up (35.0 vs 34.3 degrees C, p<0.006) and demonstrated a significantly increased vasoconstriction on the warmer side (52.0 vs 20.2%, p<0.03) corresponding to findings in patients with acute CRPS. The present study proves that there are signs of decreased sympathetic activity in the affected limb in very acute CRPS. However, no indication was found for increased sensitivity of vascular alpha-receptors in the very acute stages of CRPS, and there was also no indication for a significant direct contribution of the sympathetic nervous system to pain in very acute CRPS.

Journal Article↗

[Critically ill polyneuropathy].

"Critically ill" polyneuropathy occurs in patients with artificial respiration. It is more frequent in men than in women. The most frequent underlying diseases, in the course of which CIP was found, were pneumonias, traumas and major surgery. In most cases sepsis and/or multiple organ dysfunction syndrome can be observed. Symmetrical motor polyneuropathy is the typical clinical pattern. In many cases the thoracic muscles are also involved. In some cases facial pareses and ophthalmoplegia may occur. Sensory disturbances are rather rare. The EMG often shows a myopathic pattern in addition to the neuropathic pattern. Morphologically, CIP is an axonal degeneration. The rate of lethality is high. In surviving patients there is a 50% chance of full remission.

Axons↗

[The use of alternative medicine by multiple sclerosis patients--patient characteristics and patterns of use].

UNLABELLED: The use of alternative medicine is growing in all Western countries. Little is known about the modalities and patterns of use of alternative medicine by patients suffering from multiple sclerosis. PATIENTS AND METHODS: We analysed an anonymous questionnaire that was sent to and answered by 129 former inpatients who had multiple sclerosis diagnosed by typical clinical and laboratory findings. RESULTS: 82 of 129 patients (63.6%) have been using alternative therapies. They were treated with a total of 87 different alternative healing methods or substances. Some patients used up to 9 different methods. The mean duration of the alternative treatment was 2.6 (0-20) years. Most patients used homoeopathy (n = 35), herbs (29 different substances, 32 users), different relaxation methods like yoga (n = 38) and various diets (n = 21). The most important motivation to look for alternative medicine was the aim to participate actively in the healing process. Most patients thought that there was some positive effect from the alternative treatment but did not inform their general practitioner or neurologist about it. DISCUSSION: Like in other chronic diseases many MS-patients use alternative medicine. The experiences of these treatments forms part of the patient's coping with the disease.

Adult↗

Transmission of Creutzfeldt-Jakob disease via a corneal transplant.

A 45 year old woman is reported who initially presented with a cerebellar syndrome, severe ataxia, and dysarthria. She rapidly deteriorated to coma vigile with bilateral myoclonic jerks, flexion rigidity, and immobility necessitating complete nursing. Her EEG showed generalised slow activity and periodic biphasic and triphasic waves. The CSF concentration of neuron specific enolase was very high. Consequently the diagnosis of Creutzfeldt-Jakob disease was established. Eight months later she died of respiratory complications. Thirty years earlier the patient had undergone corneal transplantation for keratoconus. Review of the organ donor's hospital records showed that death was caused by intercurrent pneumonia subsequent to subacute spongiform encephalopathy confirmed by necropsy. In view of two previous case reports in the literature it is presumed that the cadaveric cornea was the source of transmission of Creutzfeldt-Jakob disease in this patient.

Corneal Transplantation↗

[Urine test strips for cerebrospinal fluid diagnosis of bacterial meningitis].

BACKGROUND: Diagnosis of bacterial meningitis is due to granulocytic pleocytosis of CSF. In developing countries and in hospitals without continuous availability of a clinical laboratory a bedside-method of diagnosing meningitis is desired. METHODS: 75 CSF samples were tested for granulocytes and protein with reagent strips and the results were compared with those obtained from the clinical laboratory. RESULTS: All 48 samples with normal cell count were correctly diagnosed negative using reagent strips. Twenty-five samples of 27 samples with granulocytic pleocytosis were correctly recognised as right positive (sensitivity 92.6%; specificity 100%). All 48 samples with normal protein were correctly tested normal. Twenty-one samples of 27 samples with elevated protein were correctly diagnosed right positive (sensitivity 77.8%; specificity 100%). CONCLUSION: Testing CSF for granulocytes and protein with urine reagent strips is a bedside-method which allows a rapid and reliable decision whether CSF is normal or granulocytically pleocytotic.

Cerebrospinal Fluid Proteins↗

[Diagnosis of brain death in the admission department of the Neurologic Clinic of Erlangen University from 1984 to 1994].

OBJECTIVE: The reliable diagnosis of brain death is a precondition of organ removal for subsequent transplantation. As demographic data, information on the underlying cause and written consent to organ removal from brain-dead patients are largely unknown; protocols relating to the determination of brain death were analysed to obtain these data. PATIENTS AND METHODS: The study was based on an examination of the protocols of 547 consecutive and unselected patients who between 1984 and 1994 had been examined for possible brain death by experienced members of the Neurological Department of Erlangen University, in consequence of the establishment of a consultation service for this purpose to hospitals in the region of North Bavaria. The criteria of brain death were those established by the Federal German Doctors' Chamber, all the data in the written protocol being analysed retrospectively. RESULTS: Brain death was confirmed in 521 patients (319 males, 202 females; mean age 40.1 [1.5 - 84] years). 473 patients (86.5%) had been examined at the Erlangen University Clinic or the Municipal Hospital in Nuremberg, the remainder in regional hospitals. The most common causes were trauma to the head/brain (43.5%), subarachnoid haemorrhage (18.6%), generalised hypoxaemia (9.5%), cerebral infarction (7.3%) or other conditions (4.5%). 33 patients (6%) had committed suicide. Organs were removed in 244 of 413 patients (59.1%) for whom there data on possible organ removal: seven patients had carried donor cards, relatives' consent was obtained in 237. No consent was given in 90 cases (21.8%), while 79 (19.2%) were excluded for medical reasons. CONCLUSION: If brain death is suspected, an experienced neurological consultant should be called in as soon as possible to assess the patients survival chances and evaluate possible organ removal for subsequent transplantation.

Admitting Department, Hospital↗

[Survey of alcohol drinking and results of studies of alcoholism biological markers within the scope of a neurologic occupational medicine study of industrial workers].

As part of a combined neurological and occupational-medical study about potentially neurotoxic agents consumption of alcohol of the probationers as possible influential factor on the nervous system was evaluated. Therefore an injury of industrial workers of different nationalities was performed about the individual alcohol consumption combined with an investigation about so-called alcoholism-markers [gamma-Glutamyltranferase (gGT), mean corpuscular erythrocytes volume (MCV) and Carbohydrate-deficient Transferrin (CDT)]. It was the aim of the study, to give an overview of the amount of alcohol consumption in male industrial workers - and to examine, to what extent connections exist between alcoholism markers and the amount of individual alcohol consumption. We found a high percentage of probationers with alcohol abstinence (53%). The test persons with anamnestic alcohol consumption indicated a mean alcohol consumption of 33,7 g per day (6 to 240 g/d). A significant correlation between changes of laboratory markers and amount of alcohol consumption could only be found for CDT. In addition there was a correlation of CDT with gGT and MCV and of gGT with MCV, but with very small correlation coefficients. After multiple logistic regression and establishing of a prediction model of the consumption of alcohol under knowledge of laboratory results an assignment of the probationers to the respective group (abstinents and/or consumers of alcohol) was not successful with sufficient safety. According to our data in a normal collective a determination of laboratory parameters, which are discussed as alcoholism markers, does not contribute to better delimitation of abstinent living and alcohol drinking persons and to a better knowledge of the amount of alcohol consumption.

Alanine Transaminase↗

Multifocal motor neuropathy: clinical and electrophysiological findings.

Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stimulation technique. In 8 patients, the diagnosis of MMN was confirmed by electrophysiological evidence of conduction block or temporal dispersion. The typical clinical picture of MMN with chronic progressive, asymmetrical, marked distal weakness was observed in our patients. Electrophysiological routine tests of distal nerves were usually normal except in nerve segments with conduction block. In 4 patients, conduction block could be recorded only in proximal nerve segments or spinal roots. All patients showed rapid improvement of clinical features and parallel reduction of conduction block during or after high-dose intravenous immunoglobulin (ivIG) therapy, supporting the diagnosis of an immune-mediated neuropathy. Three of them are now in remission without any therapy, whereas 5 still receive a regular ivIG course every 2-12 weeks as long-term treatment. In all patients with pure or predominantly motor syndromes and normal findings in electrophysiological routine tests of distal nerve segments, there should be proximal conduction block studies to avoid overlooking a treatable disorder such as MMN.

Adolescent↗

[Immunoglobulin therapy of chronic inflammatory neuropathies].

High-dose intravenous immunoglobulins (ivIg) are an effective therapy in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In both diseases, data regarding ivIg long-term treatment are sparse. Therapy with ivIg was performed in 18 patients with CIDP or MMN. Sixteen patients responded to ivIg therapy; they were treated for more than 6 months. Two of them had not previously shown any positive response to other immunosuppressive treatments. Response to ivIg therapy could be observed even after a long disease duration (maximum of 19.5 years). All 16 therapy responders now have no or only mild clinical symptoms. Treatment could eventually be completely stopped in 6 patients; they have now been in complete remission without therapy for a maximum of 63 months. Ten patients still receive regular ivIg infusions in different dosages. No severe side effects were observed in the whole group. IvIg therapy is an effective, safe and easily applicable treatment regimen even in the long-term course of CIDP and MMN.

Adolescent↗

[Follow-up and prognosis of patients of a neurologic intensive care unit with special reference to age].

To evaluate risk factors effecting course and prognosis of neurological intensive care (ICU) patients with special respect to age, 422 patients (235 male, 187 female, mean age 56.7 years, standard deviation +/- 18.8 years) admitted to the ICU of the Department of Neurology, University Erlangen-Nürnberg, were retrospectively studied. The status at the time of ICU discharge was compared to that assessed 18-30 months later using the Barthel-Index, a five grade scale of independence, and the Glasgow Outcome Scale. At the time of reexamination, 203 of the 422 patients (48.2%) were still alive. The fatality rate increased with age. However, approximately 70% of the patients above the age of 70 years were still alive two years after ICU treatment with the majority of patients describing their life as satisfying. Multivariate analysis demonstrated that age by itself does not determine the course of disease. Age affects the prognosis only in combination with other variables such as preexisting diseases (e.g. stroke, carotid surgery, occlusive arterial disease), secondary complications (e. g. pneumonia), and specific ICU treatment (e.g. mechanical ventilation, nasogastric tube), and the patient's state at the time of ICU discharge (bedriddenness, aphasia, dementia).

Activities of Daily Living↗