Fronto-Temporal Dementia: a clinical and genetic-epidemiological study

At the turn of the nineteenth century into the 20th century many leading neurologists were active to devise new pathological or clinical classifications of the large group of dementing illnesses in later life, the 'dementia senilis' . Until then that eponym included every psychiatrie, behavioral and cognitive disturbance, occurring after middle age and leading to complete deterioration of the mental functions. In 1892, Arnold Piek (1851-1924), professor in neurology and psychiatry at the German University of Prague, reported a patient with a two-year history of progressive 'feeble-mindedness' , behavioral disturbances and eventually aphasia. Focal temporal atrophy of the brain was found at autopsy. Piek subsequently described a few more cases with frontal and temporal atrophy and considered this focal pathology as a localized type of 'seniIe dementia' and not a distinct disease-entity. However he suggested a possible clinical-pathological relation without being specific. Alois Alzheimer (1911) described the microscopical findings to become associated with 'Piek's disease": neuronalloss, spongiosis and gliosis in the frontal and temporal cortex, argentophilic granules in the neuronal cytoplasm pushing the nucleus towards the cell body (Pick bodies), and swollen neurons (Piek cells) in the absence of neurofibrillary tangles and plaques. Van Mansvelt (1953), in a review, classified Pick's disease according to the localization of atrophy into three types: frontal, temporal and mixed' Piek bodies were reported in only one third of the cases. For a diagnosis of Piek' s disease at that time, Piek bodies were not essential. Constantinidis (1974) classified frontotemporal atrophy into three types based on the presence of Piek bodies and Piek cells: (1) cases with: Piek bodies and swollen neurons, (2) cases with only swollen neurons, and cases without Pick bodies and Piek cells.' Fronto-temporal dementia in the absence of Piek bodies became also described by Brun as 'frontal lobe degeneration of non-Alzheimer type'.

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