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Molekulare Neuropathologie der Nicht-Alzheimer-Demenzen

Molecular neuropathology of Non-Alzheimer dementia

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Zusammenfassung

Mit steigender Lebenserwartung der Bevölkerung wird der Anteil an den Gesundheitskosten zunehmen, den die neurodegenerativen und demenziellen Erkrankungen verursachen. Neuere Entwicklungen, wie etwa die Entdeckung der Rolle von TDP-43 haben es möglich gemacht, die frontotemporalen Demenzen unter einem neuen Blickwinkel zu sehen und einen Zusammenhang mit der amyotrophen Lateralsklerose herzustellen. Es ist unsere Aufgabe als Pathologen und Neuropathologen, zu einer Verbesserung der häufig schwierigen klinischen Diagnostik durch eine standardisierte postmortale Diagnostik beizutragen. Diese diagnostische Verantwortung wird in dem Maße zunehmen, in dem spezifische Therapeutika oder auch Kontraindikationen wie etwa die Verwendung von Neuroleptika bei Patienten mit Lewy-Körperchen-Demenz bekannt werden. Für diagnostisch schwierige oder aufwendige Fälle steht das Referenzzentrum für neurodegenerative Krankheiten der Deutschen Gesellschaft für Neuropathologie und Neuroanatomie und die deutsche Hirnbank (Brain-Net) am Institut für Neuropathologie der Ludwig-Maximilians-Universität München zur Verfügung.

Abstract

The increasing life expectancy will cause an increasing share for neurodegenerative and dementing illnesses in the total cost for health care. New developments such as the discovery of TDP-43 as disease protein have opened new viewpoints on frontotemporal dementias, as well as its relation to amyotrophic lateral sclerosis. As pathologists and neuropathologists we are committed to contributing to the progress of clinical diagnosis, which often proves difficult, by standardized post-mortem diagnosis. The diagnostic responsibility will increase with the development of new specific therapeutics and knowledge of contraindications such as the use of neuroleptics in patients suffering from Lewy body dementia. The Reference Center for Neurodegenerative Diseases of the German Society of Neuropathology and Neuroanatomy and the German Brain Bank (Brain-Net) at the Institute for Neuropathology, Ludwig-Maximilians-University Munich, are available for diagnosis in difficult or complex cases.

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Abbreviations

AD:

Alzheimer-Krankheit (Disease)

AGD:

Argyrophilic grain disease (Erkrankung mit argyrophilen Körnchen)

AGs:

Argyrophilic grains

CBD:

Kortikobasale Degeneration

DLB:

Demenz mit Lewy-Körperchen

FTD:

Frontotemporale Demenz

FTLD:

Frontotemporale Lobärdegeneration

FTLD-MAPT:

Familiäre frontotemporale Lobärdegeneration mit Mutationen im MAPT-Gen

FTLD-U:

Frontotemporale Lobärdegeneration mit Ubiquitin-positiven Einschlüssen

FTLD-PGRN:

Familiäre frontotemporale Lobärdegeneration mit Mutationen im Progranulin-Gen

LBs:

Lewy-Körperchen (Bodies)

MAPT:

Mikrotubuli-assoziiertes Protein Tau

NFTs:

Neurofibrilläre Tangles

NIFID:

Neuronale Intermediärfilament-Einschlusskörperchen-Krankheit

PBs:

Pick-Körperchen (Bodies)

PD:

Parkinson-Krankheit (Disease)

PDD:

Parkinson-Krankheit mit Demenz

PGRN:

Progranulin

PSP:

Progressive supranuclear palsy (progressive supranukleäre Blicklähmung)

TDP-43:

TAR-DNA-bindendes Protein 43

VCP:

Valosin-containing protein

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  1. Zentrum für Neuropathologie und Prionforschung, Ludwig Maximilians Universität München, Feodor-Lynen-Str. 23, 81377, München, Deutschland

    M. Neumann &  H.A. Kretzschmar

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  1. M. Neumann
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  2. H.A. Kretzschmar
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Correspondence to H.A. Kretzschmar.

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Neumann, M., Kretzschmar, H. Molekulare Neuropathologie der Nicht-Alzheimer-Demenzen. Pathologe 29, 434–441 (2008). https://doi.org/10.1007/s00292-008-1063-6

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