{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T13:39:20Z","timestamp":1789220360789,"version":"build-2803163510"},"reference-count":35,"publisher":"Georg Thieme Verlag KG","issue":"09","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Fortschr Neurol Psychiatr"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n                  <jats:p>Trotz klinischer diagnostischer Kriterien f\u00fcr die Parkinson-Krankheit sowie\n                        die neurodegenerativ bedingten atypischen Parkinson-Syndrome (PSP, CBD,\n                        MSA-P\/C sowie LBD) kann die Differentialdiagnose zu seltenen heredit\u00e4ren und\n                        erworbenen Erkrankungen mit Parkinson-Syndrom schwierig sein.<\/jats:p>\n                  <jats:p>Es wurden seltene heredit\u00e4re und erworbene Erkrankungen mit Parkinson-Syndrom\n                        ausgew\u00e4hlt. Sekund\u00e4re Parkinson-Syndrome als Folge von Medikation oder\n                        Toxin-Exposition wurden ausgeklammert und nur im systematischen \u00dcberblick\n                        mit dargestellt.<\/jats:p>\n                  <jats:p>Ohne Anspruch auf Vollst\u00e4ndigkeit wurden die wesentlichen heredit\u00e4ren und\n                        erworbenen Erkrankungen mit Parkinson-Syndrom tabellarisch zusammengefasst.\n                        Einzelne ausgew\u00e4hlte Entit\u00e4ten wurden in Form kurzer Steckbriefe\n                        detaillierter beschrieben. Hierf\u00fcr ausgew\u00e4hlt wurden Entit\u00e4ten mit\n                        therapeutischer Beeinflussbarkeit, besonderen Muster-artigen\n                        Befundkonstellationen und interessanten pathophysiologischen Zusammenh\u00e4ngen.\n                        Zudem wurden paradigmatische zerebrale MRT-Muster einzelner Entit\u00e4ten\n                        dargestellt.<\/jats:p>\n                  <jats:p>Es existiert eine Vielzahl seltener erworbener und genetischer Entit\u00e4ten mit\n                        klinisch relevanten Parkinson-Syndromen. H\u00e4ufig tritt das Parkinson-Syndrom\n                        dabei mit zus\u00e4tzlichen anderen klinischen Affektionen\n                        (extrapyramidal-hyperkinetisch: Dystonie\/Chorea; zerebell\u00e4r: Ataxie;\n                        pontomesencephal: Okulomotorikst\u00f6rungen, bulb\u00e4re Dysarthrie\/Dysphagie;\n                        Motoneurone: Spastik und\/oder myatrophe Paresen; Demenz\/neuropsychiatrische\n                        Symptomatik; Epilepsie) in variabler Kombination und Schweregradauspr\u00e4gung\n                        auf. F\u00fcr einige Erkrankungsgruppen (z.B. Neurodegeneration mit\n                        Eisenablagerung\/NBIA, M. Wilson, Prim\u00e4re Famili\u00e4re Hirnkalzifikation\/PFBC)\n                        ist das bildgebende MRT-Muster diagnostisch wegweisend. Eine relevante\n                        Anzahl von Erkrankungen weist ein therapeutisch zumindest partiell und\n                        zeitlich vor\u00fcbergehend mittels dopaminerger Medikation beeinflussbares\n                        pr\u00e4synaptisches dopaminerges Defizit (z.B. einige NBIA-Formen, SCA-Formen,\n                        CTX) auf. Pathophysiologisch treten bei einigen Erkrankungen sekund\u00e4r\n                        pathologische Proteinaggregate (z.B. MPAN: Synukleinopathie; IgLON5-Syndrom:\n                        Tauopathie; Perry-Syndrom\/DCTN1: TDP-43 Aggregate) auf.<\/jats:p>","DOI":"10.1055\/a-2844-5563","type":"journal-article","created":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T23:10:23Z","timestamp":1783033823000},"page":"351-370","source":"Crossref","is-referenced-by-count":1,"title":["Seltene heredit\u00e4re und erworbene Erkrankungen mit\n                    Parkinson-Syndrom"],"prefix":"10.1055","volume":"94","author":[{"given":"Florian","family":"Sch\u00f6berl","sequence":"additional","affiliation":[{"name":"Neurologie, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Klinik und\n                    Poliklinik f\u00fcr Neurologie mit Friedrich-Baur-Institut, Munich,\n                    Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franziska","family":"Hopfner","sequence":"additional","affiliation":[{"name":"Neurologie, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Klinik und\n  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