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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.

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.

High titers of anti-Hu antibodies indicate the paraneoplastic genesis of neurologic syndromes with high specifity.1,2⇓ In most cases, the primary tumor is identified as small cell lung cancer (SCLC); reports of other tumors are rare.2,3⇓ Here, we report a patient positive for anti-Hu antibodies who developed a sensory neuropathy and Holmes–Adie syndrome (HAS). The only tumor that was detected was a unilateral seminoma. After tumor removal, anti-Hu antibodies eventually vanished. The then 31-year-old patient was first admitted to the hospital in June 2000 with a 6-week history of hypoesthesia and paresthesia of his feet. He was a nonsmoker and had no relevant past or family history. Neurologic examination revealed missing Achilles tendon reflexes, marked decrease in proprioceptive and kinesthetic sensation in both feet, and mild sensory gait ataxia. CSF showed elevated protein levels (1,747 mg/L); all other findings, including oligoclonal bands and tests for borreliosis and syphilis, were normal, as were serum tests for autoantibodies, diabetes, folic acid, …

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.

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.

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.

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.

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.

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.

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.

As a contribution to the question of participation of the central nervous system in paraneoplastic syndromes an unusual case of hypernephrotic carcinoma with heavy immunopathological disturbances and clinical symptoms like Harada's syndrome is reported. The development of the 63 years old patient's disease, the clinical, haematological, CSF-, biochemical and other laboratory as well as neuroradiological and electrophysiological findings are discussed in detail. Immunosuppressive treatment after nephrectomy repeatedly led to a distinct improvement.

A form is described for recording the results obtained when monitoring the brain function in cases of severe acute brain damage. The form follows the principles introduced in other countries for recording the results of similar standard examinations, but the symptoms and scalings have been modified to suit the author's requirements. The state consciousness as shown by the opening of the eyes, verbal response and motor reactions to various stimuli is considered essential for the continuous monitoring of the brain function, as are also the dilation of the pupile and their response to light and the vestibuloocular reaction. The form also contains entries for respiration, blood pressure, heart rate and body temperature. The neurological symptoms have been selected on the basis of their diagnostic value, consistency and simplicity of examination and interpretation. The advantages of a standard examination scheme are explained. Advice is given regarding the examination of the different symptoms.

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.

12 patients, whose neurological findings had induced the procedure of brain death determination, and further 4 young children were examined correspondently by special CT methods. In conclusion cranial computerized tomography as a non-invasive method seems to be useful alternatively beside cerebral angiography for the documentation of cerebral circulation arrest.