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Biomedical subjects

B Neundörfer

Publications and source records attributed to B Neundörfer.

At least 91 records · Page 5Linked to original sources

Interictal cardiac autonomic dysfunction in temporal lobe epilepsy demonstrated by [(123)I]metaiodobenzylguanidine-SPECT.

We studied the post-ganglionic cardiac sympathetic innervation in patients with chronic temporal lobe epilepsy (TLE) by means of [(123)I]metaiodobenzylguanidine-single photon computed tomography (MIBG-SPECT) and evaluated the effects of carbamazepine on cardiac sympathetic innervation. TLE is frequently associated with dysfunction of the autonomic nervous system. Autonomic dysregulation might contribute to unexplained sudden death in epilepsy. Anticonvulsive medication, particularly with carbamazepine, might also influence autonomic cardiovascular modulation. MIBG-SPECT allows the quantification of post-ganglionic cardiac sympathetic innervation, whereas measuring the variability of the heart rate provides only functional parameters of autonomic modulation. Antiepileptic drugs, especially carbamazepine (CBZ), can affect cardiovascular modulation. We determined the index of cardiac MIBG uptake (heart/mediastinum ratio) and heart rate variability (HRV) using time and frequency domain parameters of sympathetic and parasympathetic modulation in 12 women and 10 men (median age 34.5 years) with a history of TLE for 7-41 years (median 20 years). Myocardial perfusion scintigrams were examined to rule out deficiencies of MIBG uptake due to myocardial ischaemia. To assess the possible effects of CBZ on autonomic function, we compared MIBG uptake and HRV in 11 patients who had taken CBZ and 11 patients who had not taken CBZ, and in 16 healthy controls. In order to identify MIBG uptake defects due to myocardial ischaemia, all patients had a perfusion scintigram. Cardiac MIBG uptake was significantly less in the TLE patients (1.75) than in the controls (2.14; P = 0.001), but did not differ between subgroups with and without CBZ treatment. The perfusion scintigram was normal in all patients. Time domain analysis of HRV parameters suggested the predominance of parasympathetic cardiac activity in the TLE patients, but less parasympathetic modulation in the patients treated with CBZ than in those not treated with CBZ (P < 0.05), whereas frequency domain parameters showed no significant difference between the subgroups of patients or between patients and controls. MIBG-SPECT demonstrates altered post-ganglionic cardiac sympathetic innervation. This dysfunction might carry an increased risk of cardiac abnormalities.

3-Iodobenzylguanidine↗

Acute stroke management in the local general hospital.

BACKGROUND AND PURPOSE: The majority of stroke patients are treated in local general hospitals. Despite this fact, little is known about stroke care in these institutions. We sought to investigate the status quo of acute stroke management in nonspecialized facilities with limited equipment and resources. METHODS: Four general hospitals located in smaller cities of a rural area in Germany participated in this study. The 4 hospitals were similar in structure and technical equipment; none had a CT scanner in-house. We reviewed the medical records of every stroke patient hospitalized in 1 of the 4 hospitals within a period of 8 weeks within 1 year. RESULTS: We collected data of a total of 95 patients at all 4 hospitals. The frequency of diagnostic tests was low: at least 1 CT scan was obtained in only 36.8% of all cases, whereas diagnostic methods available in-house were used more frequently, such as Doppler ultrasound (49.0%), echocardiography (42.3%), and 24-hour ECG registration (48.4%). Each hospital had a different therapeutic approach. Main therapeutic options were the use of pentoxyfilline (0% to 90.5%), osmodiuretics (0% to 90%), piracetam (0% to 93.3%), and hydroxyethylstarch (4.8% to 30%). Medication for long-term secondary prevention was given to 69.8% of all patients. CONCLUSIONS: This study provides one of the few data samples reflecting stroke care in smaller general hospitals. The findings demonstrate a partially suboptimal level of care in these institutions. To achieve future improvements, extended human and technical resources as well as research for stroke care should not be restricted to academic stroke centers.

Diuretics↗

Neuropathy is a major contributing factor to diabetic erectile dysfunction.

Erectile dysfunction (ED) in diabetes is multifactorial. So far, the impact of neuropathy has not been well determined. This study was performed to assess the frequency of abnormal neurophysiological tests in patients with ED due to diabetes compared to patients with ED due to nondiabetic neuropathies in order to estimate the contribution of neuropathy in diabetic ED. Forty-nine men with ED were studied. We classified ED as 'diabetic', 'neuropathic' or 'ED of other origin'. 26.6% of the men fulfilled the criteria of diabetic ED, 42.9% had neuropathic ED. In every patient history taking, a questionnaire focusing on autonomic symptoms other than ED, clinical examination, nerve conduction studies (NCS), sphincter ani electromyography (EMG), heart rate variability testing (HRV) and quantitative sensory testing (QST) was performed. Vascular function was assessed by the intracavernosal prostaglandin E1 (PGE1) injection test. The frequency of abnormal results in diabetic and neuropathic patients was compared. Vascular function was abnormal in only one patient with diabetic ED and three patients with neuropathic ED. Both groups had similar frequencies of autonomic symptoms other than ED (64% in diabetic vs. 64% in neuropathic patients), abnormal EMG (33% vs. 40%) and abnormal QST (vibratory perception 83% vs. 84%, cold perception 9% vs. 19%, warm perception 42% vs. 43%). Abnormal clinical findings (50% vs. 33%), NCS (75% vs. 50%) and HRV (39% vs. 25%) were slightly, but not significantly more frequent in men with diabetic ED than neuropathic ED. The tests indicating neuropathy showed abnormalities in men with diabetic ED as frequently as in men with neuropathic ED. Some tests even suggested neuropathy more often in diabetic than in neuropathic ED. The findings support the hypothesis that neuropathy contributes significantly to the pathophysiology of ED in diabetes mellitus.

Adult↗

A second locus for an axonal form of autosomal recessive Charcot-Marie-Tooth disease maps to chromosome 19q13.3.

Autosomal recessive Charcot-Marie-Tooth disease (CMT) represents a heterogeneous group of disorders affecting the peripheral nervous system. The axonal form of the disease is designated as "CMT type 2" (CMT2), and one locus (1q21.2-q21.3) has been reported for the autosomal recessive form. Here we report the results of a genomewide search in an inbred Costa Rican family (CR-1) affected with autosomal recessive CMT2. By analyzing three branches of the family we detected linkage to the 19q13.3 region, and subsequent homozygosity mapping defined shared haplotypes between markers D19S902 and D19S907 in a 5.5-cM range. A maximum two-point LOD score of 9.08 was obtained for marker D19S867, at a recombination fraction of.00, which strongly supports linkage to this locus. The epithelial membrane protein 3 gene, encoding a PMP22 homologous protein and located on 19q13.3, was ruled out as being responsible for this form of CMT. The age at onset of chronic symmetric sensory-motor polyneuropathy was 28-42 years (mean 33.8 years); the electrophysiological data clearly reflect an axonal degenerative process. The phenotype and locus are different from those of demyelinating CMT4F, recently mapped to 19q13.1-13.3; hence, the disease affecting the Costa Rican family constitutes an axonal, autosomal recessive CMT subtype (ARCMT2B).

Adult↗

Alteration of the somatosensory cortical map in peripheral mononeuropathy due to carpal tunnel syndrome.

Substantial plasticity of the mature mammalian somatosensory cortex was demonstrated after deprivation of sensory input produced by amputation or somatosensory deafferentation. Following transection of the median nerve, adult owl and squirrel monkeys exhibit extensive reorganization in the cortical representation of the hand in areas 3b and 1. In the present study we investigated the possible effect of incomplete median nerve damage on sensory cortex somatotopy in a patient with unilateral carpal tunnel syndrome. We assessed interhemispheric differences of the hand representation in SI by means of magnetic source imaging. Additional intersubject data comparison was performed for specific results on the basis of available normal data from the literature and from own investigations in five healthy volunteers. Our results demonstrated a decreased extension of the cortical zone representing the injured median nerve and suggested invasion of the deprived area by cortical sectors receiving inputs from the little finger (supplied by the ulnar nerve) and from the dorsum of the thumb (innervated by the radial nerve). The study indicates topographic rearrangement of the hand representational zone in the human primary somatosensory cortex in a case of chronic median nerve injury.

Aged↗

German open label trial of riluzole 50 mg b.i.d. in treatment of amyotrophic lateral sclerosis (ALS).

UNLABELLED: Riluzole is currently the only drug that holds any hope of prolonging life in amyotrophic lateral sclerosis (ALS) by slowing the rate of disease progression. METHODS AND RESULTS: Between 1995 and 1997 a total of 7916 ALS patients in 39 countries, were given 100 mg riluzole per day for a mean of 7.2 months. The present report focuses on the German results in comparison to the total population. Nine hundred and nineteen patients were treated in 25 German centres; 162 (17.6%) died from the disease during the course of the study. Serious adverse events attributed to the study medication occurred in 16 patients (1.7%). Most frequently these were reversible changes in liver enzymes (0.9%) occurring during the first 3 months, none resulted in death. In all, 413 patients (44.9%) reported an adverse event. The most frequent were reduced lung function (7.3%), nausea (7.1%), asthenia (5.8%), pneumonia (2.5%) and abdominal pain (2.5%). CONCLUSION: The results of the study allow the conclusion that riluzole is well tolerated. The majority of adverse events were symptoms of the underlying disease and were not attributed to riluzole. Overall the safety profile found in the German centres was very similar to the profile seen in the total patient population and was more favourable than in the two published double-blind studies [New Engl J Med 330 (1994) 585; Lancet 347 (1996) 1425].

Amyotrophic Lateral Sclerosis↗

Increased skin lactate in complex regional pain syndrome: evidence for tissue hypoxia?

To investigate oxygen metabolism in complex regional pain syndrome (CRPS), the authors measured skin lactate via dermal microdialysis performed on patients with CRPS (n = 11) and healthy control subjects (n = 11). In addition, they measured blood lactate. Although venous lactate was unaltered, skin lactate was increased in patients with CRPS (2.95 mmol/L; control subjects 1.74 mmol/L; p < 0.005). These results suggest enhanced anaerobic glycolysis, probably as a result of chronic tissue hypoxia.

Adult↗

Transcranial doppler sonography-ergometer test for the non-invasive assessment of cerebrovascular autoregulation in humans.

Cerebrovascular hemodynamics during physical stress have been sparsely investigated, mostly through risky invasive techniques. The aim of this study was to determine the effect of ergometer stress on cerebrovascular hemodynamics in humans using the non-invasive and thus clinically-applicable method of transcranial Doppler sonography (TCD) combined with simultaneous non-invasive measurements of cardiovascular parameters. In eighteen healthy subjects (six women, twelve men; 29.3+/-4.6 years old) left midcerebral artery blood flow velocities (CBFVs) were continuously monitored using TCD during 3 min at rest, 3 min during ergometry and 3 min recovery. Simultaneously, systolic, diastolic, mean CBFVs, pulsatility index (PI), heart rate, beat-to-beat blood pressure (BP) and transcutaneous p(CO(2)) were measured. The subjects were supine with elevated trunk. Ergometry was performed by pedalling a Mühe-ergometer. In eight volunteers, the procedure was repeated within the next day to test the repeatability of the results. Heart rate increased significantly during ergometry (from 65.2+/-11 to 105. 3+/-12.3/min; P<0.05). The systolic BP increased significantly slightly later during ergometry (from 118.9+/-8.6 to 141.6+17.9 mmHg; P<0.05). Transcutaneous p(CO(2)) was initially within physiological ranges, but increased significantly after a delay during the 3rd min of cycling (from 39.7+/-3.7 to 41.1+/-4.7 mmHg; P<0.05). MFV started to rise significantly after 1 min of the exercise period (from 59.6+10.9 to 68.3+13.9 cm/s; P<0.05). PI increased immediately and significantly at the start of exercise (PI at rest 0.93+0.11; PI ergometry 1.1+0.13; P<0.05). The results were found to be reproducible in the eight volunteers. The cerebrovascular changes during ergometer exercise may reflect the combined activation of the cerebrovascular autoregulative mechanisms (neurogenic, myogenic and metabolic). The TCD-ergometer test presented here is non-invasive and would seem to present a low risk for patients who are judged fit enough for mild exercise. The test may contribute to the detection of cerebrovascular abnormalities in various diseases.

Adult↗

Differentiation of Parkinson's disease and multiple system atrophy in early disease stages by means of I-123-MIBG-SPECT.

BACKGROUND: Differential diagnosis between idiopathic Parkinson's disease (PD) and multiple system atrophy (MSA) is often difficult in early disease stages. Since MSA is misdiagnosed as PD in more than 20% of the early stages, there is need for methods refining the differentiation of the two disease entities. In PD postganglionic involvement of the autonomic nervous system (ANS) predominates whereas in MSA the ANS is mainly affected in its preganglionic structures. The functional integrity of postganglionic cardiac sympathetic neurons can be investigated using I-123-metaiodobenzylguanidine-single photon emission computed tomography (MIBG-SPECT). OBJECTIVES: We investigated whether I-123-MIBG-SPECT allows to differentiate between early stages of PD and MSA in patients not yet requiring L-dopa therapy. METHODS: Thirty patients (10 PD and 20 MSA patients) underwent MIBG-SPECT and evaluation of heart rate variability (HRV). Patients on any medication interfering with MIBG-accumulation were excluded from the study. Cardiac perfusion was evaluated by myocardial scintigraphy. RESULTS: The median cardiac MIBG uptake was significantly decreased in PD as well as MSA patients compared to controls (P<0.001). However, in the PD group MIBG uptake was significantly lower than in MSA (P=0.03). Even in PD patients without clinical signs of autonomic failure, MIBG uptake was significantly lower than in MSA patients (P=0.03). Analysis of heart rate parameters did not differentiate between PD and MSA patients. The median coefficient of variation was significantly smaller in PD and MSA patients compared to control subjects. CONCLUSIONS: Our study shows that MIBG-SPECT identifies autonomic cardiac dysfunction in very early stages of both, PD and MSA. More importantly, the technique facilitates differentiation of MSA and PD in the early stages. The different pathology with prominent peripheral, postganglionic sympathetic dysfunction in PD and primarily central and preganglionic lesions in MSA accounts for a lower MIBG uptake in PD compared to MSA patients.

Adult↗

Longitudinal study of neurotoxicity with occupational exposure to aluminum dust.

Two cross-sectional studies were conducted at a German aluminum (Al) powder plant to evaluate possible nervous system effects from occupational Al exposure. The investigation included biological monitoring, a neuropsychological test battery, and event-related P300 potentials. Neurophysiologic findings in workers chronically exposed to Al dust did not differ from non-Al-exposed controls from the same plant. The authors suggest that chronic exposure to Al dust, at the levels documented in this study, does not induce measurable cognitive decline.

Adult↗

Peripheral neuropathy in chronic venous insufficiency.

Chronic venous insufficiency (CVI) of the lower legs may cause tissue damage, but involvement of peripheral nerves is uncertain. We examined 30 patients with CVI and 20 healthy controls using motor and sensory nerve conduction studies, vibration testing and thermotesting, quantitative sudomotor axon-reflex test, and laser Doppler flowmetry. Subjects with possible confounding factors for peripheral neuropathies were excluded. Prolongation of distal motor latency of the peroneal nerve (median, 5.4 versus 4.5 ms; P = 0.02), increased limits for warm (9.60 degrees C versus 5.20 degrees C; P = 0.016) and cold detection (3.45 degrees C versus 1.55 degrees C; P = 0.016) and reduced vibration sense (2.8925 versus 1.1075; P < 0.008) were found. The results demonstrate a disturbance of A-alpha fibers, A-beta fibers, A-delta fibers, and thermoafferent-C fibers, possibly induced by ischemia due to venous microangiopathy and increased endoneurial pressure. Analogous to neuropathic ulcers in diabetes, the CVI-associated neuropathy may also be a cofactor in the development of venous ulcers.

Adult↗

Cell death in vasculitic neuropathy.

Vasculitic neuropathy shows cluster of differentiation (CD)4+ and CD8+ perivascular infiltrates. CD8+ cells kill the target by different ways. Some mechanisms require the release of granzymes and T-cell restricted intracellular antigen (TIA-1) which induce cell death by apoptosis. A nonsecretory ligand-mediated mechanism has been proposed for apoptosis and requires the interaction of Fas and Fas-ligand. We studied apoptosis by immunohistochemistry and terminal deoxynucleotidyl transferase-mediated nick end labeling (TUNEL) in nerve biopsy specimens in 19 cases of vasculitic neuropathy and 8 controls. In vasculitic neuropathy, perivascular mononuclear cells expressed TIA-1, granzyme A, and granzyme B. Regarding the ligand-mediated mechanism, we observed Fas+ and Fas-ligand+ mononuclear cells in an epineurial-perivascular distribution. Cells undergoing apoptosis, as demonstrated by TUNEL, represented mononuclear cells. In vasculitic neuropathy, apoptosis by both a secretory and a nonsecretory mechanism is suspected. However, apoptosis seems to be restricted to inflammatory mononuclear cells, suggesting that it may play a critical role in recovery from vasculitic neuropathy.

Apoptosis↗

Hypoglycemic sensorimotor polyneuropathy associated with insulinoma.

Hypoglycemia-induced peripheral neuropathy due to insulinoma is unusual and, as far as we know, has previously been reported in only 34 patients. In this case report, we describe the clinical features, electrophysiological features, and pathological findings in a 37-year-old patient with polyneuropathy from repeated hypoglycemic episodes over a 9-year period that related to an insulinoma. The literature is discussed. The reported case is of special interest because the peripheral neuropathy led to the correct diagnosis.

Adult↗