Fortschritte Der Neurologie Psychiatrie最新文献

{"title":"[Rare hereditary and acquired diseases with parkinson's syndrome].","authors":"Florian Schöberl, Franziska Hopfner, Thomas Klopstock","doi":"10.1055/a-2844-5563","DOIUrl":"10.1055/a-2844-5563","url":null,"abstract":"<p><strong>Background: </strong>Despite established clinical diagnostic criteria for Parkinson's disease and the neurodegeneration-related atypical parkinsonian syndromes (progressive supranuclear palsy/PSP, corticobasal degeneration syndrome/CBD, multiple system atrophy with parkinsonian or cerebellar predominance/MSA-P/C, and dementia with Lewy bodies/DLB), the differential diagnosis from rare hereditary and acquired disorders presenting with parkinsonism can be challenging.</p><p><strong>Methods: </strong>Based on a PubMed search, relevant original studies and review articles were analyzed to identify rare hereditary and acquired disorders associated with parkinsonism. Secondary parkinsonian syndromes resulting from medication or toxin exposure were excluded but are summarized in an overview.</p><p><strong>Results: </strong>Without claiming completeness, the major hereditary and acquired disorders associated with parkinsonism were summarized in tabular form. Selected entities were described in more detail in short profiles focusing on those with therapeutic modifiability, characteristic pattern-like constellations of findings, or notable pathophysiological mechanisms. Paradigmatic cerebral MRI patterns are illustrated.</p><p><strong>Conclusions: </strong>A broad spectrum of rare acquired and genetic entities can manifest with clinically relevant parkinsonian syndromes. Frequently, parkinsonism occurs in combination with other neurological features of variable severity, including extrapyramidal-hyperkinetic symptoms (dystonia/chorea), cerebellar signs (ataxia), pontomesencephalic involvement (oculomotor disturbances, bulbar dysarthria/dysphagia), motor neuron signs (spasticity and/or amyotrophic paresis), cognitive or neuropsychiatric symptoms, and epilepsy.For several disease groups - such as neurodegeneration with brain iron accumulation (NBIA), Wilson's disease, and primary familial brain calcification (PFBC) - distinctive MRI patterns are diagnostically informative.A relevant subset of disorders exhibits at least a partial and sometimes transient presynaptic dopaminergic deficit responsive to dopaminergic medication (e.g., certain NBIA forms, spinocerebellar ataxias/SCA, cerebrotendinous xanthomatosis/CTX).Neuropathologically, some of these disorders are associated with secondary synucleinopathies (e.g., MPAN), tauopathies (e.g., IgLON5 syndrome) or TDP-43 (e.g., Perry syndrome/DCTN1).</p>","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":" ","pages":"351-370"},"PeriodicalIF":0.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148375117","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[4-Repeat Tauopathies: Progressive Supranuclear Palsy and Corticobasal Degeneration].","authors":"Carla Teresa Palleis, Alexander Bernhardt, Günter Höglinger","doi":"10.1055/a-2895-4480","DOIUrl":"10.1055/a-2895-4480","url":null,"abstract":"<p><strong>Abstract: </strong>Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are primary tauopathies that are neuropathologically characterized by deposits of 4-repeat tau proteins. Both diseases lead to a progressive impairment of motor skills, balance, speech, cognition, and everyday functioning. While PSP is often associated with axially emphasized symptoms and characteristic midbrain atrophy, CBD usually presents with asymmetric frontoparietal atrophy with cortical and subcortical deficits. Due to overlapping clinical phenotypes, it is often difficult to distinguish the two diseases from other neurodegenerative diseases in everyday clinical practice. A distinction must be made between neuropathologically defined CBD and the clinical phenotype of corticobasal syndrome (CBS), which can also be caused by other pathologies, such as Alzheimer's disease. The diagnosis of PSP or CBD is made according to current clinical criteria, based on the evaluation of multiple functional domains and the severity of presenting symptoms. Disease-modifying therapies are not yet available. Treatment currently follows symptom-oriented approaches with the aim of maintaining the quality of life and independence of patients for as long as possible. This article provides an up-to-date overview of the diagnosis, clinical presentation, management strategies, and treatment options for individuals affected by PSP and CBD.</p>","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":"94 9","pages":"389-399"},"PeriodicalIF":0.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13569406/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148944810","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Multiple system atrophy].","authors":"Ida Wilkens, Sabrina Katzdobler, Alexander Bernhardt, Günter Höglinger, Franziska Hopfner","doi":"10.1055/a-2877-5054","DOIUrl":"10.1055/a-2877-5054","url":null,"abstract":"<p><strong>Abstract: </strong>Multiple system atrophy is a rare, rapidly progressive neurodegenerative disorder within the spectrum of atypical parkinsonian syndromes. It is clinically defined by the combination of parkinsonism, cerebellar ataxia, and autonomic failure. Neuropathological findings reveal misfolded α-synuclein accumulation in oligodendrocytes, leading to multisystem neurodegeneration. Diagnosis is based on the Movement Disorder Society criteria and characteristic MRI findings but can only be definitively confirmed post mortem. Currently, no disease-modifying therapies are available; management is symptomatic and requires a multidisciplinary approach. Ongoing research aims to establish sensitive biomarkers, identify prodromal disease stages, and develop targeted interventions addressing key pathophysiological mechanisms such as α-synuclein aggregation and neuroinflammation.</p>","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":" ","pages":"377-388"},"PeriodicalIF":0.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148683673","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Suicidal Ideation and Behavior: Clinical Assessment, Prevention, and Therapeutic Approaches].","authors":"Xenia Kersting, Ute Lewitzka","doi":"10.1055/a-2699-3482","DOIUrl":"https://doi.org/10.1055/a-2699-3482","url":null,"abstract":"<p><strong>Abstract: </strong>Suicidality represents a common and potentially life-threatening phenomenon that can occur across a wide range of psychiatric, somatic, and psychosocial contexts. In this article, we provide a clinically oriented classification of suicidality and present epidemiological data as well as relevant risk factors. Suicidality is conceptualized as a dynamic, multifactorial process that should not be understood as a linear stage model, but rather as the result of an interaction between individual vulnerabilities, situational stressors, and protective factors.We place a particular focus on the exploration and assessment of suicide risk, including the limitations of predictive approaches and the role of standardized assessment instruments in routine clinical practice. The importance of the therapeutic relationship and of alliance-forming capacity as central protective factors is highlighted. In addition, we present evidence-based concepts of suicide prevention at the primary, secondary, and tertiary levels.</p><p><strong>Abstract: </strong>Finally, we give a short review of current therapeutic approaches to the treatment of suicidality. The article emphasizes the need for continuous, context-sensitive risk assessment, structured follow-up care, and interdisciplinary prevention strategies to reduce suicidal crises and behaviors.</p>","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":"94 9","pages":"401-414"},"PeriodicalIF":0.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148944758","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Parkinson-Syndrome im Wandel – von der klinischen Phänotypisierung zur Präzisions(differenzial)diagnostik und biologischen Krankheitsdefinition als Grundlagen für zielgerichtete pathophysiologisch orientierte Therapieansätze.","authors":"Florian Schöberl, Marianne Dieterich","doi":"10.1055/a-2896-0762","DOIUrl":"https://doi.org/10.1055/a-2896-0762","url":null,"abstract":"","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":"94 9","pages":"338-339"},"PeriodicalIF":0.5,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148944803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Predicting and Preventing Alzheimer's Disease].","authors":"Maria-Christina Weber, Jana Goedecke, Gereon R Fink, Oezguer A Onur","doi":"10.1055/a-2906-0084","DOIUrl":"https://doi.org/10.1055/a-2906-0084","url":null,"abstract":"<p><strong>Abstract: </strong>Alzheimer's disease is a long-term biological process whose neuropathological changes begin decades before clinical symptoms appear. Advances in biomarker diagnostics enable the early identification of pathological changes and a more precise classification of different disease stages. Since therapeutic options have shown only limited effects so far, modifiable risk factors and preventive lifestyle interventions are increasingly coming into focus.</p>","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":" ","pages":""},"PeriodicalIF":0.5,"publicationDate":"2026-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148577323","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Neurofilament light chain as a tool for assessing disease activity and treatment response in Multiple Sclerosis].","authors":"Hernan Inojosa, Katja Akgün, Lars Masanneck, Sven Günther Meuth, Jens Kuhle, Stefan Bittner, Michael Khalil, Marc Pawlitzki, Tjalf Ziemssen","doi":"10.1055/a-2873-1243","DOIUrl":"https://doi.org/10.1055/a-2873-1243","url":null,"abstract":"<p><strong>Abstract: </strong>Serum neurofilament light chain (sNfL), a neuronal structural protein, is released upon neuroaxonal damage and correlates with clinical and imaging activity, brain atrophy, and disability progression, though it is not disease-specific. This review addresses measurement methods (SiMoA, automated platforms), pre-analytics/matrix effects, and central confounders (esp. age, BMI/volume of distribution, renal function, intercurrent events), emphasizing standardization via age- and BMI-corrected reference data using Z-scores or percentiles.Clinically, sNfL supports baseline risk stratification and treatment planning, serial treatment monitoring as an early warning signal for subclinical disease activity, the differentiation of relapse versus pseudo-relapse, the assessment of progressive courses, and decision-making in special situations (pregnancy, older age, de-escalation). Serial measurements are superior to single measurements for longitudinal assessment. Priorities include assay harmonization and prospective studies on NfL-guided, multimodal decision strategies.</p>","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":" ","pages":""},"PeriodicalIF":0.5,"publicationDate":"2026-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148434920","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Welchen Nutzen bietet das Lesen deutschsprachiger Journale für Neurologie und Psychiatrie?","authors":"Heinz Reichmann","doi":"10.1055/a-2882-6906","DOIUrl":"https://doi.org/10.1055/a-2882-6906","url":null,"abstract":"","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":"94 7-08","pages":"264-265"},"PeriodicalIF":0.5,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148420003","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Acceptance by and evaluation of unidirectional hearing systems by patients in outpatient and (partial) inpatient geriatric psychiatric care].","authors":"Barbara Leicher, Verena Caroline Buschert, Monia Belkahia, Sarah Winter, Norbert Scherbaum, Jens Benninghoff","doi":"10.1055/a-2314-3049","DOIUrl":"10.1055/a-2314-3049","url":null,"abstract":"<p><strong>Abstract: </strong>AIM OF STUDY: The aim of the present study was to investigate the use and subjective benefit of specific temporary hearing-improvement measures (unidirectional hearing aids) in hearing-impaired, geriatric psychiatric patients. Simultaneously, employees evaluated the handling and acceptance of the hearing-improving measures.</p><p><strong>Abstract: </strong>MATERIAL AND METHOD: Between October 2022 and July 2023, subjective hearing ability and use of conventional hearing aids were recorded in outpatients and in those in partial inpatient care (n=151) based on a self-assessment questionnaire. After using unidirectional hearing aids in diagnostics and treatment for four to six weeks, the hearing ability of 21 patients who had not used the hearing-improving measures was surveyed again and the experiences of active users (n=34) and employees (n=24) with the hearing-improving measures were analyzed via questionnaires.</p><p><strong>Abstract: </strong>RESULTS: Of the 151 included patients (79.2 years, 62.1% female), 147 patients and 24 employees (79.2% female) took part in the study. Subjective hearing impairments were reported by 50 patients (34.0%). The hearing of 93 patients (63.2%) had already been assessed once. Treatment with conventional hearing aids was recommended for 34 of those surveyed (23.1%). Likewise, 34 patients (23.1%) took advantage of the offer of hearing-improving measures. All 34 users and all participating employees rated the hearing-improving measures used as mostly positive.</p><p><strong>Abstract: </strong>CONCLUSION: Hearing impairment in geriatric psychiatric patients is common and often not adequately treated with conventional hearing aids, yet hearing-improving measures are only used to a limited extent. Mostly positive results among the users of hearing-improving measures favor their implementation in patients in routine outpatient and (partial) inpatient geriatric psychiatric care.</p>","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":" ","pages":"283-289"},"PeriodicalIF":0.5,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141310482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Experiences of (Potentially) Traumatizing Deployments and Posttraumatic Stress in Police Officers: An Exploratory Survey of Early Career Police Officers.","authors":"Lena Posch, Anna-Lena Zube","doi":"10.1055/a-2351-2533","DOIUrl":"10.1055/a-2351-2533","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Abstract: &lt;/strong&gt;BACKGROUND: As part of their career, police officers are faced with traumatic events on a regular basis and are at a higher risk of developing post-traumatic-stress disorder (PTSD) compared to the general population. The aim of this study was to examine if and how many early career police officers had already experienced potentially traumatizing situations and how many met either subsyndromal or complete PTSD criteria. A further subject of interest was if the officers knew the concept of psychosocial emergency care for first responders (PSNV-E) and if such support was made use of.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Abstract: &lt;/strong&gt;METHOD: Early career police officers (&lt;i&gt;n&lt;/i&gt;=221) were assessed via an online survey about their posttraumatic stress symptoms.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Abstract: &lt;/strong&gt;RESULTS: More than half of the participants (&lt;i&gt;n&lt;/i&gt;=121) reported having experienced at least one traumatic deployment. PTSD prevalence in this group was at 1.7%, an additional 14.9% showed partial PTSD. One-fifth did not know about the PSNV-E concept.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Abstract: &lt;/strong&gt;CONCLUSION: Police officers are confronted with a range of extremely stressful events early in their career leading to first symptoms of PTSD in some of them. Early prevention strategies as well as the identification of those affected for secondary prevention is of very high relevance for long-term mental health.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Zusammenfassung: &lt;/strong&gt;HINTERGRUND: Polizeibeamt:innen werden in ihrer beruflichen Karriere oft mit traumatischen Einsätzen konfrontiert und weisen im Vergleich zur Allgemeinbevölkerung ein erhöhtes Risiko eine posttraumatische Belastungsstörung (PTBS) zu entwickeln auf. Ziel der vorliegenden Studie war, zu untersuchen, ob und wie häufig Berufsanfänger:innen der Polizei bereits (potenziell) traumatische Einsätze erlebt haben und wie häufig eine PTBS oder eine subsyndromale Ausprägung der PTBS vorliegt. Ferner war von Interesse, ob ihnen das Konzeptzur psychosozialen Notfallversorgung für Einsatzkräfte (PSNV-E) bekannt ist und ob diese Angebote in Anspruch genommen werden.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Zusammenfassung: &lt;/strong&gt;METHODE: &lt;i&gt;N&lt;/i&gt;=221 Berufsanfänger:innen der Polizei wurden im Rahmen einer Online-Befragung zum Erleben potenziell traumatischer Einsätze befragt. Zudem wurde mit der PCL-5 das Ausmaß posttraumatischer Belastungssymptome erhoben.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Zusammenfassung: &lt;/strong&gt;ERGEBNISSE: Über die Hälfte der Befragten (&lt;i&gt;n&lt;/i&gt;=121) hatte mindestens einen (potenziell) traumatischen Einsatz erlebt. Die PTBS-Prävalenz lag in dieser Gruppe bei 1,7%, weitere 14,9% wiesen eine partielle PTBS auf. Einem Fünftel der Befragten war das PSNV-E Konzept nicht bekannt.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Zusammenfassung: &lt;/strong&gt;SCHLUSSFOLGERUNG: Bereits in den ersten Berufsjahren sind Polizeibeamt:innen mit extrem belastenden Einsätzen konfrontiert, woraufhin ein Teil von ihnen erste Anzeichen einer PTBS aufweist. Frühe Präventionsmaßnahmen sowie Identifikation von Betro","PeriodicalId":12353,"journal":{"name":"Fortschritte Der Neurologie Psychiatrie","volume":" ","pages":"274-282"},"PeriodicalIF":0.5,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141619794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}