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Biomedical subjects

R Zschenderlein

Publications and source records attributed to R Zschenderlein.

At least 37 records · Page 2Linked to original sources
PubMed2000

Partial synergy of bisindolylmaleimide with apoptotic stimulus in antigen-specific T cells--implications for multiple sclerosis.

In multiple sclerosis (MS), induction of T cell apoptosis constitutes a promising therapeutic strategy. Recently, bisindolylmaleimide has been shown to be an effective treatment of experimental autoimmune encephalomyelitis, presumably due to enhancement of CD95-mediated T cell apoptosis. Therefore, we studied the effects of bisindolylmaleimide on human (auto)antigen-specific T cells. We observed a synergistic effect of bisindolylmaleimide with apoptotic stimulus assessed via caspase activity and annexin V-binding, but no potentiation of DNA fragmentation or cell death. Thus, bisindolylmaleimide might be useful for modulating T cell apoptosis, yet more potent substances have to be generated re-establishing immunological control over auto-reactive T cells.

Annexin A5↗
PubMed2000

No induction of apoptosis by IFN-beta in human antigen-specific T cells.

Interferon (IFN)-beta, the most effective immunomodulatory treatment for MS, inhibits the proliferation of myelin-specific T cells. We report that IFN-beta moderately enhances the expression of the death receptor, CD95, at the surface of human antigen-specific T cells. However, T-cell apoptosis was not induced by IFNbeta-1a or IFNbeta-1b as assessed by caspase activity or DNA fragmentation. Immunomodulation mediated by IFN-beta does not directly involve apoptotic pathways in human T cells.

Adjuvants, Immunologic↗
PubMed2000

[Apoptosis in multiple sclerosis. Etiopathogenetic relevance and prospects for new therapeutic strategies].

Apoptosis, or programmed cell death, is a physiological cell suicide program mainly leading to selective elimination of useless cells. This mechanism is important for the homeostasis of the immune system and presumably plays a two-sided role in the pathogenesis of multiple sclerosis (MS). On the one hand, evidence has been provided that impaired apoptosis might result in increased numbers or persistence of activated myelin-specific T cells, thus inducing the pathophysiologic processes in MS. On the other hand, local tissue damage might involve apoptosis of glial and neuronal cells and lead to the clinical symptoms. Here, an overview is presented on the current knowledge of the role of apoptosis in the pathogenesis of MS, and implications for related therapeutic strategies are discussed.

Apoptosis↗
PubMed2000

Clinicopathological study of atypical motor neuron disease with vertical gaze palsy and ballism.

The case of a 38-year-old patient with rapidly progressing motor neuron disease, complicated by major dysfunction of the extrapyramidal system and of vertical gaze is described. Neuropathological examination revealed a degenerative process that severely affected the lower motor neurons, as well as the neurons of the pars compacta of the substantia nigra, the nucleus of Darkschewitsch, the nucleus interstitialis of Cajal, the colliculi superiores, and the pallidum. The long tracts were unaffected at all levels of the brain stem and spinal cord. There was no convincing evidence for the presence of a multiple system atrophy or progressive supranuclear palsy; the results rather revealed a pattern of vulnerability characteristic of a variant of motor neuron disease.

Adult↗
PubMed1998

Changes in perfusion pattern using ECD-SPECT indicate frontal lobe and cerebellar involvement in exercise-induced paroxysmal dystonia.

The clinical features of exercise-induced paroxysmal dystonia (EPD) are delineated in a pedigree including two affected members (both male) showing an autosomal-dominant inheritance trait. Gait analysis using kinematic electromyography during the motor attacks revealed coactivation of antagonistic calf muscles characteristic of dystonia. In the interval, impaired muscular alternation was observed. To characterize further the pathophysiological basis of the condition, ictal and interictal cerebral perfusion SPECT studies using technetium 99m-ethyl cysteinate dimer (ECD) were performed to establish whether cortical hyperactivity indicative of epilepsy is present during the motor attacks and to identify regional changes in the ictal perfusion pattern that could indicate an anatomic structure relevant to the disease. During the motor attacks, decreased ictal perfusion of the frontal cortex was found in both patients. In contrast, increased cerebellar perfusion was observed. The perfusion of the basal ganglia also decreased. No cortical hyperperfusion indicative of an epileptic nature was seen. Cerebellar hyperactivity in connection with prominent frontal hypoactivity has also been described in both the idiopathic and the symptomatic forms of dystonia. Our findings therefore suggest that EPD represents a paroxysmal movement disorder rather than epilepsy. It is concluded that changes in frontal and in cerebellar function are relevant to the pathophysiology of EPD.

Adolescent↗
PubMed1998

Atypical stiff-person syndrome with spinal MRI findings, amphiphysin autoantibodies, and immunosuppression.

In an atypical case of stiff-person syndrome (SPS), spinal T2-weighted MRI revealed a hyperintense lesion extending from C2 to C7 that corresponded with the clinical symptoms and signs. CSF showed lymphocytic pleocytosis and oligoclonal bands. Amphiphysin autoantibodies were detected in serum and CSF; however, unlike other reported cases, no malignancy occurred during a 3-year observation period. Methylprednisolone and cyclophosphamide pulse therapy led to a marked reduction of symptoms.

Anti-Inflammatory Agents↗
PubMed1997

A new variant of progressive encephalomyelitis with rigidity associated with cerebellar ataxia and dementia: correlation of MRI and histopathological changes. A case report.

A 27 year-old patient developed a progressive neurological multisystem disorder. Initial signs were cerebellar ataxia and dementia, followed by rigidity and oculomotor dysfunction. Myoclonus was not present. MRI showed a marked atrophy of the spinal cord, the cerebellum, and mild (sub)cortical atrophy. CSF contained oligoclonal bands, but no anti-glutamic acid dehydrogenase antibodies. He died 33 months after onset of symptoms. Autopsy revealed widespread neuropathological alterations including perivascular lymphocytic cutting, neuronal cell loss, and micro/astrogliosis the distribution of which corresponded to the changes seen in MRI. The diagnosis of progressive encephalomyelitis with rigidity was pathohistologically confirmed. Brain samples were negative for neurotrophic viruses tested by polymerase chain reaction. A new variant of this rare disorder is described initially presenting with ataxia and dementia, but without myoclonus.

Adult↗
PubMed1993

[Clinico-genetic diagnosis of ataxia telangiectatica (Louis-Bar syndrome)].

We describe the possible difficulties in getting the diagnosis "ataxia telangiectasia" using the example of a 16 years old girl. If cases of cerebellar ataxia in childhood present without classical symptoms, the diagnosis of ataxia telangiectasia should not be excluded before chromosome analysis. In our case, first signs of cerebellar ataxia were observed from the age of 11 years and we found only mild, atypical located and late onset telangiectasis. Other signs of the syndrome, such as elevated alpha-fetoproteine and deficiency of IgA or IgE could not be detected. Chromosome analysis, however, demonstrated a breakage syndrome with chromosome 14 to 7 translocation and established a firm diagnosis of ataxia telangiectasia. Patients with chromosome breakage syndromes including the Louis-Bar-syndrome have an increased risk for malignomas. Therefore chromosome analysis should be undertaken in cases of children with cerebellar ataxia, and frequent radiological examination avoided.

Adolescent↗
PubMed1989

[Nosologic classification of Fazio-Londe disease].

The observation of a progressive bulbar paralysis with lethal exit in a 20 years old patient, whose mother had died in the age of 29 years after a similar course of disease, is coordinated as Fazio: Londe-disease. But peculiarities are mentioned, that refer to traits of the special form of progressive bulbar paralysis described by Kennedy and of Kugelberg-Welander-disease. A genetic basis of variability is supposed.

Adult↗
PubMed1989

[Local intravasal fibrinolysis in acute cerebrovascular occlusions].

The development of local intravasal fibrinolysis in acute cerebrovascular obturations is lined out and the precautions, indications and contraindications as well as our results from 10 patients are described. The requirements of dosage, procedure and of a close cooperation and combination with further clinical treatment are given. Furthermore close cooperation with hemostaseology is stressed.

Acute Disease↗