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Biomedical subjects

A Müller

Publications and source records attributed to A Müller.

At least 235 records · Page 13Linked to original sources
PubMed1999

Rare occurrence of intracerebellar colloid cyst. Case report.

Colloid cysts are rare intracerebral lesions that are preferentially encountered within the third ventricle. There are only a few reports in which colloid cysts are described in other locations such as the fourth ventricle. A symptomatic intracerebellar colloid cyst in a 45-year-old woman is described. The patient presented with headache, gait disturbance, and nausea. Neuroradiological imaging revealed compression of the fourth ventricle, hydrocephalus, and an intracerebellar cystic lesion measuring 4 x 5 cm that had a small peripheral solid portion. The cyst was successfully removed via a paramedian suboccipital approach. Postoperatively, the patient recovered quickly. The findings in the present report represent an additional example of the broad spectrum of cystic lesions encountered in the cerebellum.

Cerebellar Diseases↗
PubMed1999

Persistent Klüver-Bucy syndrome after bilateral thalamic infarction.

OBJECTIVE: To describe a patient who exhibited a partial Klüver-Bucy syndrome after small bilateral ischemic lesions in the thalami. BACKGROUND: Previously reported patients with Klüver-Bucy syndrome had very large, mostly bilateral lesions in the limbic system and could not provide sufficient information about its anatomo-functional correlate. METHOD: Behavioral assessments and clinical examinations, including magnetic resonance imaging and positron emission tomography, were conducted. RESULTS: The patient was severely amnestic, distractible, hyperoral, and affectively dyscontrolled, and she behaved socially inappropriately. Magnetic resonance imaging showed bilateral infarctions in the territories of both thalamoperforating arteries, and positron emission tomography revealed bilaterally decreased fluorodeoxyglucose uptake in the anterior parts of the ventral thalami and, to a lesser extent, in the fronto-temporal cortices. CONCLUSIONS: This behavioral syndrome has not yet been reported with isolated diencephalic lesions, but it has been observed after bilateral temporal lobe lesions. The authors conjecture that this syndrome resulted from a disruption of the pathways connecting the dorsomedial thalami with the prefrontal cortices and with other limbic areas, systems essential for memory and the regulation of impulses and emotions.

Aged↗
PubMed1999

Immunopathology of ANCA-associated vasculitis.

During the past few years remarkable progress has been achieved in the understanding of the pathogenic mechanisms leading to vascular inflammation and injury in ANCA-associated vasulitides (AAV): Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg Strauss syndrome (CSS). In this paper we review the immunopathology of these diseases by describing the role of autoantibodies (ANCA), dysregulation and abnormalities at the cellular level, genetic background and environmental factors that predispose to autoimmune response.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1998

Regulation of polar auxin transport by AtPIN1 in Arabidopsis vascular tissue.

Polar auxin transport controls multiple developmental processes in plants, including the formation of vascular tissue. Mutations affecting the PIN-FORMED (PIN1) gene diminish polar auxin transport in Arabidopsis thaliana inflorescence axes. The AtPIN1gene was found to encode a 67-kilodalton protein with similarity to bacterial and eukaryotic carrier proteins, and the AtPIN1 protein was detected at the basal end of auxin transport-competent cells in vascular tissue. AtPIN1 may act as a transmembrane component of the auxin efflux carrier.

Amino Acid Sequence↗
PubMed1998

AtPIN2 defines a locus of Arabidopsis for root gravitropism control.

The molecular mechanisms underlying gravity perception and signal transduction which control asymmetric plant growth responses are as yet unknown, but are likely to depend on the directional flux of the plant hormone auxin. We have isolated an Arabidopsis mutant of the AtPIN2 gene using transposon mutagenesis. Roots of the Atpin2::En701 null-mutant were agravitropic and showed altered auxin sensitivity, a phenotype characteristic of the agravitropic wav6-52 mutant. The AtPIN2 gene was mapped to chromosome 5 (115.3 cM) corresponding to the WAV6 locus and subsequent genetic analysis indicated that wav6-52 and Atpin2::En701 were allelic. The AtPIN2 gene consists of nine exons defining an open reading frame of 1944 bp which encodes a 69 kDa protein with 10 putative transmembrane domains interrupted by a central hydrophilic loop. The topology of AtPIN2p was found to be similar to members of the major facilitator superfamily of transport proteins. We have shown that the AtPIN2 gene was expressed in root tips. The AtPIN2 protein was localized in membranes of root cortical and epidermal cells in the meristematic and elongation zones revealing a polar localization. These results suggest that AtPIN2 plays an important role in control of gravitropism regulating the redistribution of auxin from the stele towards the elongation zone of roots.

Amino Acid Sequence↗
PubMed1998

[Megacolon and megarectum in adults. Abdominal proctectomy with colo-anal anastomosis].

Severe constipation in adults with megarectum may be a manifestation of Hirschsprung's disease or idiopathic megarectum. The differential diagnosis is discussed in the light of two case histories. Total abdominal proctectomy with coloanal anastomosis eliminated the aganglionic rectal segment in Hirschsprung's disease and the adynamic distal colorectum of idiopathic megarectum respectively. The surgical and functional results of a hand-sewn transanal end-to-end-anastomosis were excellent in both patients.

Adult↗
PubMed1998

[Active vaccination against early summer meningoencephalitis].

TBE is a rather rare disease (incidence in Switzerland 0.46/1,000,000 population per year). Acquisition occurs only in well known endemic areas through bites of infected ticks. The course of the disease is not trivial: 0.5-2% of cases are fatal, 2.7% have persistent serious paralysis and some 33% a postencephalitic syndrome. There is no specific therapy. An active vaccine is commercially available which is reliably immunogenic (99% of vaccinated persons develop protective antibodies) and involve only a few mild side effects. Mass vaccination seems not to be cost-effective but would be epidemiologically efficient, as a vaccination campaign in Austria has shown. Because of the increasing importance of leisure-acquired TBE infections, the Swiss recommendations could be formulated more liberally: active vaccination is recommended for everyone who regularly spends time in forests in an endemic area, be it for work or leisure.

Encephalitis Viruses, Tick-Borne↗
PubMed1998

[Differential indication for coronary stent implantation. Comparative study of acute cardial and vascular complications in relation to the indication].

BACKGROUND AND OBJECTIVE: Coronary stents are used nowadays not only for the reduction of restenosis and for treating acute vessel occlusions after PTCA but also after acute myocardial infarction. This study was undertaken to determine whether widening the indications has affected the incidence of acute complications and to compare acute cardiac and vascular complications. PATIENTS AND METHODS: The data on 197 consecutive patients (155 men, 42 women; mean age 62 +/- 9 [37-85] years) with coronary stents were analysed retrospectively, divided into 5 groups depending on the indications for the stent implantation: 1) acute or threatened vessel occlusion after elective PTCA ("bail-out"); 2) acute myocardial infarction (AMI); 3) unstable angina with threatened vessel occlusion; 4) suboptimal primary results (angiographically assessed) after PTCA; 5) elective stent implantation to prevent restenosis. Acute or subacute stent thrombosis, side-branch occlusion, intra- and transmural infarction, death and emergency aortocoronary bypass operation were classified as acute cardiac complications. Haemorrhage in the inguinal region requiring blood transfusion, false aneurysm and operative vascular reconstruction were classified as vascular complications. RESULTS: An intended stent implantation was impossible in 18 patients (primary success rate 91%). Independent of indication an acute or chronic stent stenosis occurred in three (1.6%) and seven (3.9%) patients, respectively. Side-branch occlusion was observed in 12 patients (6.7%), transmural infarction in nine (5.6%). No emergency bypass operation had to be performed. Comparing the different indication groups there was a significantly increased rate of "non-Q" infarctions in patients with unstable angina pectoris (P < 0.014). Among acute vascular complications (10 [5%] inguinal haemorrhages requiring transfusion and 5 [2.5%] operative vascular reconstructions), false aneurysm was significantly more common in patients with AMI (P < 0.014). Comparing emergency and elective stent implantations, side-branch occlusions were significantly more common in the former (12% vs. 0%; P < 0.08), as were also "non-Q" infarcts (10% vs. 0%; P < 0.002). CONCLUSION: Coronary stent implantation for these indications, including AMI, can be taken as firmly established. Stent thrombosis was not significantly increased after "bail out". Implantation in an acute ischaemic episode led to a significantly higher incidence of side-branch occlusion and "non-Q" infarction.

Acute Disease↗
PubMed1998

[Neurosarcoidosis].

Signs and symptoms of neurosarcoidosis are variable and depend on location and size of granulomas. Clinical studies suggest a rate of 5% and autopsy results a rate of more than 25% of central nervous system (CNS) involvement in sarcoidosis. Statistical analysis of 57,789 patients admitted to the Department of Medicine in Lucerne over an 11-year period revealed 51 patients (0.9/1000) with the diagnosis of sarcoidosis. Six of these (12%) had sarcoidosis affecting the CNS. Neurosarcoidosis presented as: leptomeningeal granulomas, cranial nerve palsy, hypothalamic-pituitary syndrome, diabetes insipidus, pareses, paresthesia, pyramidal signs, dementia, urine retention, and asymptomatic granulomas. Neurosarcoidosis has predilections for the base of the brain, cranial nerves (facial nerve palsy is the most common) and meninges, but any part of the CNS may be affected. Therefore, the diagnosis of neurosarcoidosis may be extremely difficult, especially when it occurs as an isolated finding. Positive findings in transbronchial biopsy and lavage may demonstrate asymptomatic pulmonary involvement in as many as 50% of patients with neurosarcoidosis. Angiotensin-converting enzyme levels may be raised in the blood or cerebrospinal fluid in some 50% of cases. Kveim test has a low sensitivity in neurosarcoidosis and thus is of little use. Gallium uptake may demonstrate extracranial granuloma available for biopsy. All these tests, and also computed tomography and magnetic resonance imaging, may be helpful. However, when in selected cases with isolated CNS disease standard investigations are not conclusive, meningeal or cerebral biopsy may be required in order to exclude other causes such as other granulomatous disorders, tumor metastasis, lymphoma, vasculitis, Sjögren syndrome, infection, neurologic disease such as multiple sclerosis, or systemic diseases such as Whipple's disease. CNS involvement in the acute phase of the disease has a favorable prognosis, while chronic courses respond less well to therapy. Treatment is initiated most frequently with corticosteroids (0.5-1 mg/kg body weight/day or pulses of 1 g/day of methylprednisolone in severe cases). Improvement is seen within 1-2 months. Side effects of corticosteroids, aggressive disease or frequent recurrences may require other immunosuppressive drugs (methotrexate, azathioprine, chlorambucil, cyclosporine A). Cerebral irradiation may be successful in some cases when other treatments fail.

Adult↗
PubMed1998

[Progress of acute liver failure and indications for liver transplantation].

OBJECTIVE: To analyse the course of acute liver failure and the indications for liver transplantation. PATIENTS AND METHODS: In 16 patients who developed acute liver failure between July 1992 and July 1997 the indications for liver transplantation and total survival time were analysed retrospectively. RESULTS: Intensive medical care (ventilation, dialysis) was necessary in 60% of the patients to bridge organ failure until liver transplantation. In 14 patients liver transplantation was indicated. Eleven of the patients, with a minimal Quick value of less than 10%, presented with the prognostically poorest London criteria. In two patients the combination of three additional criteria of the London classification led to liver transplantation being indicated. In one patient with Wilson's disease the transplantation was undertaken despite good clotting factor synthesis, because an encephalopathy occurred. In two patients liver transplantation was not undertaken even though indicated, in one because liver functions improved, in the other because necrotizing pancreatitis had occurred. In another patient, who had been poisoned by eating the mushroom Amanita phalloides, the attempted transplantation was cut short, because intestinal necrosis had developed, and the patient died during the operation. Nine of the eleven patients who had a transplantation and three of the four treated conservatively survived. CONCLUSION: The 60-day survival rate in this series, achieved in a recently established transplantation programme, is high (12 of 16; 75%), being comparable to those in larger transplantation centres.

Adult↗
PubMed1998

[Tsutsugamushi fever. Rare rickettsiosis after a stay in the Philippines].

HISTORY AND CLINICAL FINDINGS: After returning to his native Germany from a holiday in the Philippines a 37-year-old man was admitted because of high fever, cervical lymphadenopathy, pharyngitis and conjunctivitis, transient skin rash, nausea and vomiting, leukocytosis with shift to the left, atypical lymphocytes, as well as increased transaminases, LDH and cholestasis-indicating enzymes. INVESTIGATIONS: Stool, sputum and urine cultures were negative. The chest radiogram showed bilateral mild interstitial infiltration. Antibody titres against Rickettsia tsutsugamushi were markedly raised (IgG 1:128, IgM 1:2048). DIAGNOSIS, TREATMENT AND COURSE: Empirical antibiotic treatment with ciprofloxacin (200 mg twice daily intravenously) had no effect. As the mild signs of interstitial pneumonia progressed, clarithromycin (500 mg twice daily orally) was substituted with rapid fall in fever and gradual improvement. Tsutsugamushi infection was diagnosed serologically and the antibiotic changed to doxycycline (100 mg twice daily orally), continued for 14 days. Full remission occurred. CONCLUSIONS: Tsutsugamushi fever should be included in the differential diagnosis if, in addition to a history of a visit to an endemic area, there is the clinical triad of skin necrosis at the site of a mite bite, regional lymphadenopathy and skin rash (in this case, no skin lesion). The infection can be lethal without adequate treatment. Tetracyclines and possibly also macrolide antibiotics are effective against the causative organism.

Adult↗
PubMed1998

[Strongyloides stercoralis infection in a patient with AIDS and non-Hodgkin lymphoma].

HISTORY AND CLINICAL FINDINGS: The patient, now 50 years old, an immigrant miner from the former Yugoslavia who was known to have AIDS, was in 1992 found to have non-Hodgkin lymphoma of the oesophagus and given five cycles of multiple chemotherapy (CHOP) with complete remission. Subsequently he complained of retrosternal pain, dysphagia, dry cough and upper abdominal discomfort. On admission he had slight fever of 39.1 degrees C, but physical examination was unremarkable. LABORATORY TESTS: Blood count revealed an eosinophilia of 41%. IgE concentration was raised to 432IU/ml. The CD4+ T-cell count was reduced to 10/microliter, that of CD8+ to 89/microliter. ADDITIONAL TESTS: Blood culture, fecal and sputum samples and bronchoalveolar lavage demonstrated Mycobacterium avium intracellulare. TREATMENT AND COURSE: Treatment of the disseminated atypical mycobacterial infection was started with clarithromycin, rifabutin, ciprofloxacin and ethambutol. There was no improvement of the upper abdominal discomfort, but the fever subsided. Oesophagogastroscopy excluded recurrence of the lymphoma. Biopsy and examination of the duodenal juice revealed worms and larvae of Strongyloides stercoralis. Stool samples contained no mycobacteria, but strongyloides larvae were demonstrated. Albendazole was given (2 x 400 mg daily for 6 days, followed by a maintenance dose of 1 x 400 mg daily). Repeat endoscopy and stool sample after a month no longer showed any parasites. CONCLUSION: Even in Western Europe, persons coming from endemic areas who, as this patient, have various risk factors that may facilitate the occurrence of strongyloidiasis. With early diagnosis and treatment albendazole is an efficacious drug.

AIDS-Related Opportunistic Infections↗
PubMed1998

New aspects of electron transfer revealed by the crystal structure of a truncated bovine adrenodoxin, Adx(4-108).

BACKGROUND: Adrenodoxin (Adx) is a [2Fe-2S] ferredoxin involved in steroid hormone biosynthesis in the adrenal gland mitochondrial matrix of mammals. Adx is a small soluble protein that transfers electrons from adrenodoxin reductase (AR) to different cytochrome P450 isoforms where they are consumed in hydroxylation reactions. A crystallographic study of Adx is expected to reveal the structural basis for an important electron transfer reaction mediated by a vertebrate [2Fe-2S] ferredoxin. RESULTS: The crystal structure of a truncated bovine adrenodoxin, Adx(4-108), was determined at 1.85 A resolution and refined to a crystallographic R value of 0.195. The structure was determined using multiple wavelength anomalous dispersion phasing techniques, making use of the iron atoms in the [2Fe-2S] cluster of the protein. The protein displays the compact (alpha + beta) fold typical for [2Fe-2S] ferredoxins. The polypeptide chain is organized into a large core domain and a smaller interaction domain which comprises 35 residues, including all those previously determined to be involved in binding to AR and cytochrome P450. A small interdomain motion is observed as a structural difference between the two independent molecules in the asymmetric unit of the crystal. Charged residues of Adx(4-108) are clustered to yield a strikingly asymmetric electric potential of the protein molecule. CONCLUSIONS: The crystal structure of Adx(4-108) provides the first detailed description of a vertebrate [2Fe-2S] ferredoxin and serves to explain a large body of biochemical studies in terms of a three-dimensional structure. The structure suggests how a change in the redox state of the [2Fe-2S] cluster may be coupled to a domain motion of the protein. It seems likely that the clearly asymmetric charge distribution on the surface of Adx(4-108) and the resulting strong molecular dipole are involved in electrostatic steering of the interactions with AR and cytochrome P450.

Adrenodoxin↗
PubMed1998

[Iatrogenic disease and self-injury: significant factors in hospital admission?].

UNLABELLED: The frequency of adverse effects caused by doctors may serve as a quality control. We therefore assessed the number of adverse effects leading to a hospital admission and occurring during a six-month period in the medical department of a district hospital. We compared these figures with the number of admissions caused by substance-abuse by the patient himself. RESULTS: At admission to hospital 35 patients (6.4%) suffered from adverse effects. In 89% of these, the adverse effect was the leading factor to admit the patient; in the remaining 4 patients (11%) the adverse effect was detected by routine-screening upon admission. Further 8.6% developed adverse effects in the hospital which in 12.5% had consequences upon course and duration of the hospitalization. Self-afflicted hospital-admissions (substance-abuse, suicide-attempts) were found in 13%. Iatrogenic adverse effects brought 3.5% of the patients to the hospital. CONCLUSIONS: Adverse effects caused by diagnostic and therapeutic activities of doctors considerably contribute to hospital-admissions and course of hospital-stay, but even more often the patient himself is responsible by his self-damaging behavior.

Adult↗
PubMed1998

Paralysis of B7 co-stimulation through the effect of viral IL-10 on T cells as a mechanism of local tolerance induction.

The Epstein-Barr virus (EBV) encodes an open reading frame with significant homology to the cellular IL-10 gene. This viral IL-10 (vIL-10) might enable EBV to evade antiviral T cells. We employed transfectants of a murine tumor cell line (P815) to investigate whether vIL-10 interferes with the first (antigenic) or second (co-stimulatory) signal of T cell activation. Untransfected P815 cells caused tumors in syngeneic DBA/2 mice after s.c. inoculation. In contrast, transfectants that provided either a strong antigenic stimulus (P815-Kb cells) or a strong co-stimulatory signal (P815-B7 cells) were rejected. Injection of double-transfected P815 cells expressing Kb and secreting high levels of vIL-10 (P815-Kb-vIL-10) did not result in tumor growth. We then investigated whether vIL-10 could paralyse co-stimulation by B7 under the same conditions. Therefore P815-B7 cells were mixed with vIL-10-secreting P815-Kb cells and co-injected into DBA/2 animals. Most of these mice developed a tumor. Explanted tumor cells expressed the B7 molecule but not the Kb antigen. These observations in vivo were mirrored by experiments in vitro: vIL-10 could induce T cell tolerance towards P815-B7 cells but not P815-Kb cells. Taken together our results suggest that vIL-10 acts directly on T cells to inhibit co-stimulatory signals mediated via B7 receptors such as CD28 or CTLA-4.

Animals↗
PubMed1998

[Semiologic classification of epileptic seizures].

Recent advances in epileptology and epilepsy surgery require revision of the currently used International Classification of Epileptic Seizures, which was published 1981. We present a classification of epileptic seizures which is based purely on the clinical seizure semiology. The advantages of a semiological seizure classification are stressed.

Brain Mapping↗