Die Erblichen Myoklonisch-Epileptisch-Dementiellen Kernsyndrome: Progressive Myoklonusepilepsien-Dyssynergia Cerebellaris Myoclonica-Myoklonische Vari
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1. Begriffsbestimmung, Klassifikation und Pathophysiologie der Myoklonien.- 2. Erbliche und exogene dementielle und/oder epileptische und/oder myoklonische Syndrome.- 2.1. Myoklonische Syndrome.- 2.2. Myoklonisch-epileptische Syndrome.- 2.3. Myoklonisch-epileptisch-dementielle Syndrome.- 3. Geschichte.- 3.1. Progressive Myoklonusepilepsien (PME) mit intracerebraler Speicherung von Mucopolysacchariden.- 3.2. Progressive Myoklonusepilepsien (PME) mit degenerativen Veränderungen cerebellarer und/oder extrapyramidaler Neuronensysteme.- 3.3. Dyssynergia cerebellaris myoclonica (DCM) mit degenerativen Veränderungen cerebellarer Neuronensysteme.- 3.4. Myoklonische Varianten der drei nachinfantilen Formen der amaurotischen Idiotie (MVI).- 4. Fragestellung, Material und Untersuchungsgang.- 5. Progressive Myoklonusepilepsien (PME) und Dyssynergia cerebellaris myoclonica (DCM).- 5.1. Pathomorphologische, genetische und klinische Datenauswertung sowie Datenvergleich der histologisch gesicherten Fälle.- 5.2. Genetische und klinische Datenauswertung sowie Datenvergleich der klinisch wahrscheinlichen und histologisch gesicherten Fälle.- 6. Die myoklonischen Varianten der drei nachinfantilen Formen der amaurotischen Idiotie (MVI).- 6.1. Pathomorphologie und Biochemie.- 6.2. Genetik.- 6.3. Klinik.- 6.4. Besprechung der Ergebnisse.- 6.5. Fragliche Sonderformen der amaurotischen Idiotie mit und ohne Myoklonien.- 7. Psychopathologie.- 7.1. Progressive Myoklonusepilepsien (PME) und Dyssynergia cerebellaris myoclonica (DCM).- 7.2. Myoklonische Varianten der drei nachinfantilen Formen der amaurotischen Idiotie (MVI).- 8. Differentialdiagnose.- 8.1. Die erblichen myoklonisch-epileptisch-dementiellen Kernsyndrome und die exogenen dementiellen und/oder epileptischen und/oder myoklonischenSyndrome.- 8.2. Die verschiedenen Krankheitsformen der erblichen myoklonisch-epi-leptisch-dementiellen Kernsyndrome.- 8.3. Die erblichen myoklonisch-epileptisch-dementiellen Kernsyndrome und die sonstigen erblichen dementiellen und/oder epileptischen und/oder myoklonischen Syndrome.- 9. Eugenik.- 10. Zusammenfassende Schlußbetrachtungen und Anregungen für künftige Untersuchungen.- 11. Kasuistischer Anhang.- 11.1. Histologisch gesicherte Fälle der progressiven Myoklonusepilepsien (PME).- 11.2. Klinisch wahrscheinliche Fälle der progressiven Myoklonusepilepsien (PME) und Dyssynergia cerebellaris myoclonica (DCM).- 11.3. Histologisch gesicherte Fälle der myoklonischen Varianten der drei nachinfantilen Formen der amaurotischen Idiotie (MVI).- 12. Zusammenfassung.- 13. Summary.- 14. Literatur.- 15. Sachverzeichnis.
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