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DOI: 10.1055/s-2005-866949
Die Silberkornerkrankung als Ursache einer rasch progredienten Demenz mit triphasischen EEG-Veränderungen: Eine Differenzialdiagnose zur Creutzfeldt-Jakob-Erkrankung
Argyrophilic Grain Disease with Progressive Dementia and Triphasic Waves in the EEG: A Differential Diagnosis to the Creutzfeldt-Jakob DiseasePublication History
Publication Date:
05 September 2005 (online)
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Zusammenfassung
Wir berichten über eine 77-jährige Patientin, die klinisch typische Symptome einer Creutzfeldt-Jakob-Erkrankung mit einer rasch fortschreitenden Demenz, Myoklonien und im EEG triphasischen Wellen, aufwies. Erst post mortem konnte histologisch und immunchemisch eine Prionenerkrankung ausgeschlossen und die Diagnose einer Silberkornkrankheit gesichert werden. Die Symptomatik dieser Erkrankung erscheint weiterhin unspezifisch und erlaubt bisher im Vergleich zu anderen Demenzen keine eindeutige klinische Abgrenzung. Dennoch wird sie klinisch oft unterschätzt und stellt bei demenziellen Prozessen eine wichtige Differenzialdiagnose dar.
Abstract
We report a case of a 77 year old female patient, who presented with clinical symptoms of Creutzfeldt-Jakob disease such as progressive dementia, myocloni and triphasic waves in the EEG. An autopsy was performed, but a prion disease couldn't be verified histologically or immunochemically. Instead the diagnosis of argyrophilic grain disease was made. The clinical signs of this disease still remain unspecific and don't allow a clear distinction to other forms of dementia. Nevertheless argyrophilic grain disease is often underestimated in its clinical impact and is an important differential diagnosis in demential processes.
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Christian Roth
Prof. Dr. A. Ferbert
Neurologische Klinik · Klinikum Kassel
Mönchebergstraße 41 - 43
34125 Kassel
Email: roth99@web.de