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Biomedical subjects

R Zschenderlein

Publications and source records attributed to R Zschenderlein.

At least 19 recordsLinked to original sources

[Adenocarcinoma-associated nonbacterial thrombotic endocarditis as the cause of recurrent strokes].

We describe a 60-year-old female patient without vascular risk factors diagnosed with cardioembolic ischemic stroke due to an atrial septal aneurysm with a right-to-left shunt. However, further investigation after recurrent strokes revealed a nonbacterial thrombotic endocarditis (NBTE) caused by a metastatic adenocarcinoma. The presented case illustrates the difficulties in establishing the diagnosis of NBTE premortally and points out the importance of repeated echocardiographic evaluations of cardiac valves and serological examination of tumor markers in patients with recurrent strokes of unknown origin.

Adenocarcinoma↗

[Sporadic cerebral amyloid angiopathy with ischemic leukoencephalopathy and microbleeds. Cause for a rapidly progressive dementia syndrome].

We describe a 72-year-old patient with rapidly progressive dementia and a complex focal seizure. Magnetic resonance (MR) imaging revealed leukoencephalopathy with the involvement of the U-fibers as well as cortical and subcortical microbleeds. Brain biopsy confirmed the diagnosis of cerebral Abeta amyloid angiopathy (CAA). The presented case illustrates the significance of CAA as a cause of rapidly progressive dementia and leukoencephalopathy and points out the importance of T2-weighted MR imaging in the evaluation of dementia.

Aged↗

Multiple sclerosis coinciding with Machado-Joseph disease.

Although inflammatory demyelination is considered to be the key feature in multiple sclerosis (MS) pathogenesis, histopathological investigations and MRI studies recently highlighted the extent of neuronal damage that occurs even in the early stages of the disease. We report the unusual case of a patient with Machado-Joseph disease (MJD; spinocerebellar ataxia (SCA) III) and discuss this coincidence in light current pathogenetic paradigms of CNS autoimmunity.

Adult↗

Escalating immunotherapy of multiple sclerosis--new aspects and practical application.

Recent clinical studies in multiple sclerosis (MS) provide new data on the treatment of clinically isolated syndromes, on secondary progression, on direct comparison of immunomodulatory treatments and on dosing issues. All these studies have important implications for the optimized care of MS patients. The multiple sclerosis therapy consensus group (MSTCG) critically evaluated the available data and provides recommendations for the application of immunoprophylactic therapies. Initiation of treatment after the first relapse may be indicated if there is clear evidence on MRI for subclinical dissemination of disease. Recent trials show that the efficacy of interferon beta treatment is more likely if patients in the secondary progressive phase of the disease still have superimposed bouts or other indicators of inflammatory disease activity than without having them. There are now data available, which suggest a possible dose-effect relation for recombinant beta-interferons. These studies have to be interpreted with caution, as some potentially important issues in the design of these studies (e. g. maintenance of blinding in the clinical part of the study) were not adequately addressed. A meta-analysis of selected interferon trials has been published challenging the value of recombinant IFN beta in MS. The pitfalls of that report are discussed in the present review as are other issues relevant to treatment including the new definition of MS, the problem of treatment failure and the impact of cost-effectiveness analyses. The MSTCG panel recommends that the new diagnostic criteria proposed by McDonald et al. should be applied if immunoprophylactic treatment is being considered. The use of standardized clinical documentation is now generally proposed to facilitate the systematic evaluation of individual patients over time and to allow retrospective evaluations in different patient cohorts. This in turn may help in formulating recommendations for the application of innovative products to patients and to health care providers. Moreover, in long-term treated patients, secondary treatment failure should be identified by pre-planned follow-up examinations, and other treatment options should then be considered.

Clinical Trials as Topic↗

[Immunotherapy of chronic inflammatory demyelinating polyneuropathy].

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated disease of the peripheral nervous system with an estimated prevalence of 1-2/100,000. The clinical presentation is heterogeneous, but the most common form causes symmetrical progressive or relapsing weakness affecting proximal and distal muscles. CIDP is among the most treatable peripheral nerve disorders and corticosteroids, plasmapheresis and intravenous immunoglobulin have been shown to be effective in short-term prospective, randomized controlled trials. Data however indicate that approximately one-third of patients do not respond to these treatment modalities, nor do they provide equivalent evidence for a durable clinical response. There is a lack of good quality controlled trials of any other immunosuppressive agent, but cyclophosphamide and cyclosporin may be of value in patients with poor response to first-line modalities. Alternatively, the use of combination therapy may increase the efficacy in unresponsive patients. This review highlights the current status of CIDP treatment trials and discusses the significance of any therapeutic option in terms of efficacy, tolerability and cost-effects.

Chronic Disease↗

Downregulation of transforming growth factor-beta1 in interferon-beta1a-treated MS patients.

The authors report an intense downregulation of transforming growth factor-beta1 (TGF-beta1) serum levels 4 weeks from start of interferon-beta1a (IFN-beta1a) treatment at 44 microg/week in 271 patients with relapsing-remitting MS, which was still present after 1 year (p < 0.001). In line with previous data, interleukin-10 serum levels did not vary significantly. These results indicate that the immunomodulatory effects of IFN-beta might not be restricted to the postulated anti-inflammatory mechanisms and address the role of TGF-beta in the pathogenesis of MS.

Adult↗

Polyspecific immunoglobulins (IVIg) suppress proliferation of human (auto)antigen-specific T cells without inducing apoptosis.

Polyspecific immunoglobulins (IVIg) have been shown to reduce disease activity in multiple sclerosis (MS). To investigate the mechanisms of action of IVIg, we studied the impact of IVIg on growth and death (apoptosis) of human (auto)antigen-specific T cells. We observed a substantial suppression of proliferation of specifically activated T cells, in absence of caspase activation or DNA fragmentation. Further, neither susceptibility of T cells to undergo CD95-mediated apoptosis nor expression of apoptosis-blocking bcl-2 was modulated by IVIg. We conclude that IVIg may inhibit the reactivity of antigen-specific T cells in MS through suppression of proliferation rather than modulation of apoptosis.

Apoptosis↗

Adult-onset Leukoencephalopathy with vanishing white matter presenting with dementia.

We report on a case of dementia and extensive cerebral white matter abnormalities seen on magnetic resonance-images which meet the criteria for leukoencephalopathy with vanishing white matter. This is an inherited condition that was first thought to occur only in children. Our patient shows that vanishing white matter should be considered in adult patients with early-onset dementia and extensive white matter changes seen on magnetic resonance images.

Adult↗

Chronic inflammatory demyelinating polyradiculoneuropathy with histologically proven optic neuritis.

Although patient series of clinical, electrophysiological, or magnetic resonance imaging evidence for involvement of the central nervous system in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been published, the histological proof has never been reported. We present the case of a 46-year-old male patient who developed CIDP in his early 20s and who died of relapsing severe pneumonia. In late stages of the disease the patient presented visual loss and bilateral atrophy of the optic nerve. Neuropathological examination revealed severe peripheral neuropathy consistent with CIDP and central involvement with bilateral optic neuritis. This is the first case reporting CIDP and histologically proven optic neuritis.

Axons↗

Rapid typing of Borrelia burgdorferi sensu lato species in specimens from patients with different manifestations of Lyme borreliosis.

To further investigate the pathogenic potential of different Borrelia burgdorferi genospecies, specimens from 27 patients with different manifestations of Lyme borreliosis were analyzed by PCR and reverse line blotting (RLB). In samples from Lyme arthritis patients, B. burgdorferi sensu stricto was predominantly identified, while in patients with neuroborreliosis or acrodermatitis, Borrelia garinii and Borrelia afzelii, respectively, were exclusively detected. The results demonstrate that PCR-RLB is a valuable tool for epidemiological and pathogenetic studies of Lyme borreliosis.

Adult↗