Fortschritte Der Neurologie Psychiatrie最新文献

The present report describes a patient with Alexander's disease occurring in late adulthood. The genetic mutation reported in this case has not been known or reported before. The 47-year-old patient presented in our clinic with various neurological symptoms. A microsurgical partial resection was performed and showed the diagnosis stated above. Postoperatively, the symptoms continued to persist. The patient underwent interdisciplinary rehabilitation.

Myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are rare autoimmune-mediated neuromuscular diseases characterized by muscle weakness. Although various treatment options are available for MG, a subset of patients may be considered treatment-refractory, with differing definitions of this status present in the scientific literature. This monocentric, retrospective cohort study investigated 200 patients with MG and 15 patients with LEMS. Data on clinical characteristics, antibody status, treatment response, and concomitant diseases were collected. MG patients who received at least three different immunosuppressants during their disease history were categorized as treatment-refractory. Statistical analyses were conducted using SPSS.11.5% of MG patients (n=23) were classified as treatment-refractory. According to the MGFA classification, these patients were more likely to exhibit severe disease, have a higher prevalence of MuSK antibodies, and experience increased cardiovascular comorbidities. The results indicate that patients in the treatment-refractory group exhibited severe disease progression more frequently according to the MGFA classification and required escalation therapies more often. However, a standardized definition of therapy-refractory MG is not yet available.

Primary progressive aphasia (PPA) is a neurodegenerative disease that leads to progressive impairment of language and communication, with serious consequences for the daily lives of affected patients.As part of an international research collaboration (https://www.comet-initiative.org/Studies/Details/1871), important endpoints for investigating the success of (speech and language) therapy for people with PPA were developed (Core Outcome Set for PPA, COS-PPA). Here we report the results from Germany, discuss them in the context of the overall project, and compare them with the core outcomes for post-stroke aphasia.Six small group interviews were conducted with n=9 patients with PPA and n=13 caregivers using the nominal group technique to ask about wishes and goals regarding communication and speech-language therapy as well as their importance.PPA patients emphasised linguistic goals, while care partners focused on communication relevant to everyday life and psychological aspects. The international COS-PPA results were largely consistent with the findings in Germany and also showed a focus on language and communication. A comparison of the German survey results with the COS for aphasia after stroke revealed particular overlap regarding the improvement of language, communication, and quality of life.Taking into consideration the wishes and treatment goals in PPA can contribute to the planning of therapy and to the standardization of research methods in clinical trials. However, the individual character of the treatment should be preserved.

Whipple's disease is a rare systemic disease caused by a chronic infection with Tropheryma whipplei, which can often only be diagnosed years after the first symptoms appear. The symptoms are varied and sometimes unspecific. In addition to cachexia, recurrent joint swelling and abdominal complaints, neurological symptoms with dementia, myoclonus and motor neuron disease also occur. In the reported case, symptoms such as joint swelling and spasms of the legs occurred during neurological rehabilitation following a paramedian pons infarction. The close collaboration between the neurological rehabilitation physicians and rheumatology colleagues made it possible to diagnose Whipple's disease. Guideline-compliant treatment with ceftriaxone followed by sulfamethoxazole-trimethoprim was initiated. This improved the joint effusions and, surprisingly, also the spasms of the legs within a few days.

Telepsychiatry is shifting the focus of psychiatry and psychotherapy from personal interaction and relationship building to communication with technological mediators in the form of telecommunication, virtual reality (VR), social robots or artificial intelligence (AI). This article discusses the opportunities and risks of new technologies in psychiatric treatment, taking into account the principles of medical ethics. Telepsychiatric treatments can promote self-determination in the home environment as opposed to institutionalisation but carry the risk of uncontrolled data sharing. They harbour risks of potential harm such as social isolation, negative effects on the doctor-patient relationship and long-term changes in patient behaviour through, for example, VR. In terms of justice, demographic and psychopathological factors could result in unequal access to telepsychiatry, with questionable sustainability effects. The anonymity of telepsychiatry threatens alienation, while moral considerations cannot be replaced by AI for the time being. It is obligatory to carefully weigh up the benefits and risks of telepsychiatry, but especially in rural areas they could optimise care.Precisely because of ethical concerns, further intensive research is necessary to weigh up the risks and benefits. The development of telepsychiatric systems requires transdisciplinary co-operation. At a societal level, the significance of technologies as a supplement or replacement for the doctor-patient relationship needs to be discussed.

Since 2020 physicians and psychotherapist in Germany can prescribe digital interventions. The interventions have to meet safety and data privacy requirements and must prove their effectiveness. Eight interventions are available in the field of neurology: Two for multiple sclerosis (elevida, levidex), three for insomnia (HelloBetter Schlafen, somnio, somnovia), one for aphasia (neolexon Aphasie), one for mild cognitive impairment (NeuroNation MED), and one for migraine headache prophylaxis (sinCephalea - Migräneprophylaxe). In Germany, digital interventions can be prescribed to outpatients and as a post-hospital treatment.

Background:   B-cell targeted therapies are highly effective in multiple sclerosis (MS). Most of these therapies are administered intravenously at long intervals. Ofatumumab, an anti-CD20 antibody that is administered subcutaneously at low doses on a monthly basis due to its high affinity to the target structure, became available for the treatment of MS in 2021.

Methods:   An overview of practice-relevant immunological and clinical data on ofatumumab is provided.

Results:   The high affinity of ofatumumab to the target structure allows low dose and low volume administration, with the release and absorption profile after subcutaneous application allowing for high concentrations in the lymph nodes and gradual depletion of B-cells. Rapid onset of action is achieved as well as B-cell repletion within a few months in case of discontinuation of therapy. Long-term data show stable IgG levels over up to four years and high efficacy with respect to relapse rate, progression, and cognition. According to current study data, the effect compared to teriflunomide is greater the earlier therapy is initiated. Ofatumumab has a specific B-cell depletion pattern. CD20 expressing B-cell progenitor cells in the bone marrow are preserved and therefore also the inducibility and differentiation of plasma cells. The formation of a humoral immunological memory is therefore possible. Four-year study data showed no abnormalities in the rate of severe infections or malignancies.

Conclusions:   Ofatumumab is an innovative B-cell targeted therapy. It is highly effective with a good safety and tolerability profile, well controllable and maintains immunocompetence against pathogens.

Major depressive disorder is a common mental health disease with a chronic and treatment-resistant course in about one-third of patients. Invasive vagus nerve stimulation (VNS) as a long-term adjunctive treatment option has increasingly been used in the last years. VNS was CE-certified in the European Union for use in chronic and treatment-resistant depression in 2001. Method In this narrative literature review we provide an overview on VNS as a treatment option in patients with depression. We particularly focus on aspects with high clinical relevance. Results Indication to conduct VNS is determined after comprehensive evaluation of the patients' symptoms and psychiatric history. After education of patients and caregivers and obtaining informed consent, a pacemaker-like pulse generator is implanted in the left chest in a short surgical procedure. In the first weeks after implantation, the stimulation is turned on stepwise in an outpatient setting. The left vagal nerve is stimulated for 30 sec. every 5 minutes. Hoarseness during stimulation is the most frequent side-effect. There is a delay in the onset of antidepressant action of about 6-12 months. In a large registry, the cumulative response rate after 5 years was significantly higher (67.6%) in patients treated with VNS plus treatment-as-usual (TAU) than TAU alone (40.9%). Long-term benefits of VNS on quality of life, cognition, morbidity and mortality have been described previously. Conclusion VNS is a long-term safe treatment option in severely affected patients with depression with positive impact on depression severity, quality of life and cognitive function. Increase of monoaminergic transmission and anti-inflammatory effects of VNS are possible mechanisms of action.