Pick und die fokalen Hirnatrophien

Abstract

In 1892 Arnold Pick presented the case of an elderly demented patient with severe aphasia and global brain atrophy most accentuated at the left temporal lobe. Picks main interest was the relationship between focally accentuated brain degeneration and focally accentuated neuropsychological deficits, and this was what he wanted to demonstrate. He made no effort to describe a new form of dementia. The term "Pick's disease" which was introduced 30 years later implies the existence of a nosological entity with characteristic clinical features, localisation and histology. The neuropathological causes of focally accentuated brain atrophies are varied. Neocortical pyramidal cell loss with or without spongiform changes, cortical and subcortical gliosis have commonly been described. Achromatic neuronal ballooning ("Pick's cells") and intraneuronal argentophilic inclusion bodies ("Pick's bodies"), Alzheimer type plaques and tangles and other features were found in a smaller number of cases. Several authors confirmed an association between the localisation of brain atrophy and its clinical manifestations, but no convincing relationship has been demonstrated between the clinical symptoms and the underlying histology. In several studies frontal lobe degeneration was found in 10% to 20% of the demented patients, whereas aphasia, apraxia and other pronounced deficits in the context of focally accentuated brain atrophy were described less frequently. An early clinical distinction between atypical early stages of Alzheimer's disease and other forms of slowly progressive dementing disorders is virtually impossible in these cases. Prospective studies documenting the clinical and anatomical findings are needed to examine the reliability of these surmised brain-behaviour relationships in degenerative brain diseases more reliably. A descriptive approach offers a better basis for data collection than the premature diagnosis of a poorly defined disease like "Pick's".

Overlap between neurodegenerative disorders

“…PD is defined by the presence of PB and Pick cells but many cases are at variance with these classic features 140 . In fact Pick made no effort to describe a new form of dementia and the concept of PD was established later, therefore implying the existence of a nosological entity with characteristic clinical features and histology 141 . Moreover, a number of studies have suggested that there is no convincing evidence of linkage between clinical symptoms and histology in PD 141 .…”

“…In fact Pick made no effort to describe a new form of dementia and the concept of PD was established later, therefore implying the existence of a nosological entity with characteristic clinical features and histology 141 . Moreover, a number of studies have suggested that there is no convincing evidence of linkage between clinical symptoms and histology in PD 141 . In more recent times, PD has became incorporated within the concept of FTD 55 but this seems to have created a group of similar diseases with extensive overlap between its constituent members.…”

Can neurodegenerative disease be defined by four ‘primary determinants’: anatomy, cells, molecules, and morphology?

“…Pick's disease [139], for example, is defined pathologically by the presence of tau-immunoreactive Pick bodies (PB) and abnormally enlarged neurons ('Pick cells'), but many cases of clinically typical PiD are at variance with these classic neuropathological features [98], e.g., some clinically typical PiD cases may lack PB [83]. Moreover, there is no convincing evidence linking the clinical symptoms of PiD with its histology, a challenge to the original clinico-pathological concept [60]. Subsequently, PiD became subsumed within the concept of 'frontotemporal dementia' (FTD) [87], but this classification also resulted in a heterogeneous group of disorders with considerable overlap between its constituent members [19].…”

Klinische Aspekte der frontotemporalen Demenz

“…Der Krankheitsverlauf ist rasch progredient, die Alltagskompetenz ist bald im Verlauf eingeschränkt[19,21,36,38,39].Weil es sich bei den drei Syndromen der frontotemporalen Demenz, der primär progressiven Aphasie und der semantischen Demenz in den meisten Fällen um topographisch unterschiedliche Ausdruckformen derselben neurodegenerativen Prozesse im Stirnhirn und im Schläfenlappen handelt, ist es verständlich, dass es zwischen ihnen groûe Überlappungen und längsschnittliche Übergänge gibt. Eine klare Einordnung des Krankheitsbildes ist in vielen Fällen nur nach einer mehrjährigen Verlaufsbeobachtung möglich[16,19,39]. Zu diesem Zeitpunkt war Herr F. kaum zu testen.…”