Förstl H, Baldwin B
Psychiatrische Klinik, Zentralinstitut für Seelische Gesundheit, Mannheim.
Fortschr Neurol Psychiatr. 1994 Sep;62(9):345-55. doi: 10.1055/s-2007-999066.
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".
1892年,阿诺德·皮克报告了一例老年痴呆患者,该患者患有严重失语症,全脑萎缩,以左侧颞叶最为明显。皮克主要关注的是局灶性脑变性与局灶性神经心理缺陷之间的关系,这也是他想要证明的。他并未试图描述一种新的痴呆形式。30年后引入的“皮克病”一词意味着存在一种具有特征性临床特征、定位和组织学的病种实体。局灶性脑萎缩的神经病理学原因多种多样。新皮质锥体细胞丢失伴或不伴有海绵状改变、皮质和皮质下胶质增生是常见的描述。在少数病例中发现了无色神经元气球样变(“皮克细胞”)和神经元内嗜银包涵体(“皮克小体”)、阿尔茨海默型斑块和缠结以及其他特征。几位作者证实了脑萎缩的定位与其临床表现之间的关联,但尚未证明临床症状与潜在组织学之间存在令人信服的关系。在几项研究中,10%至20%的痴呆患者存在额叶变性,而在局灶性脑萎缩背景下失语、失用症和其他明显缺陷的描述则较少。在这些病例中,几乎不可能在阿尔茨海默病非典型早期阶段与其他形式的缓慢进展性痴呆疾病之间进行早期临床区分。需要进行前瞻性研究来记录临床和解剖学发现,以便更可靠地检验这些推测的脑-行为关系在退行性脑疾病中的可靠性。与过早诊断像“皮克病”这样定义不明确的疾病相比,描述性方法为数据收集提供了更好的基础。