Brun A, Passant U
Department of Pathology and Psychogeriatrics, Lund University Hospital, Sweden.
Acta Neurol Scand Suppl. 1996;168:28-30.
Frontal lobe degenerative dementias, the second largest degenerative dementia group after Alzheimer's disease, is dominated by frontal lobe degeneration of non-Alzheimer type. It is classified in a group also containing Pick's disease, progressive aphasia and dementia in motor neuron disease. Frontal lobe degeneration of non-Alzheimer type is clinically marked by frontal lobe symptoms and frontotemporal reduction of blood flow. From a histopathological point of view it is characterized by gliosis, microvacuolation, neuronal atrophy-loss and 40-50% loss of synapses in three superficial cortical laminae of the frontal convexity and anterior temporal cortex, while the deeper laminae are little or not changed. The structural changes of Alzheimer's disease including amyloid, Levy body dementia and Pick's disease are entirely lacking. A strong heredity points to a genetic cause as yet undefined.
额叶变性性痴呆是仅次于阿尔茨海默病的第二大变性性痴呆组，以非阿尔茨海默型额叶变性为主。它被归类于一个还包括皮克病、进行性失语和运动神经元病性痴呆的组。非阿尔茨海默型额叶变性的临床特征是额叶症状和额颞叶血流减少。从组织病理学角度来看，其特征是胶质增生、微空泡形成、神经元萎缩-丧失以及额叶凸面和颞叶前部皮质三个浅表皮质层中40%-50%的突触丧失，而较深的层几乎没有变化或没有变化。完全不存在阿尔茨海默病的结构变化，包括淀粉样蛋白、路易体痴呆和皮克病。强烈的遗传倾向表明存在尚未明确的遗传病因。
