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Living with Dementia

Pick’s disease and the frontal dementias

  • - Dementia: general aspects
  • Jun 20, 2011
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Tags: | arnold pick | carl schneider | circumscribed cerebral atrophy | frontal dementia |

Dementias believed to be related to frontal lobe pathology have once again become of interest, and authors often invoke the name of Arnold Pick (Niery et al., 1988). However, when the great Prague neuropsychiatrist described the syndrome named after him, all he wanted was to draw attention to a form of localized (as opposed to diffuse) atrophy of the temporal lobe (Pick, 1892). This alteration was to give rise to a dysfunction of language and praxis, and be susceptible to diagnosis during life. Pick believed that lobar atrophies constituted a stage in the evolution of the senile dementias.

The story starts, as it should, before Pick. Louis Pierre Gratiolet (1854)  was responsible for renaming the cerebral lobes after their overlying skull: thus ‘anterior’ became ‘frontal’ lobe. He made no assumption as to the function of the ‘anterior extremity of the cerebral hemisphere’.  ‘Phrenologists’, however, did and related reflective and perceptive functions (qualitatively defined)  to the forehead (Anonymous, 1832) (for the science of phrenology see Combe, 1873;  Lanteri-Laura, 1970). ‘Modular’ assumptions (i.e. a one-to-one correlation between mental function and brain site)  involving the frontal lobes started only during the 1860s, following reports on dysfunction of language in lesions of the frontal lobes (Broca, 1861; Henderson, 1986). These claims ran parallel to those of Jackson’s that the cerebral cortex was the general seat of personality and mind (Jackson, 1894). Meynert (1885)  believed that ‘the frontal lobes reach a high state of development in man’ but still defined mental disorders as diseases of the ‘fore-brain’  (by which he meant ‘prosencephalon’ or human brain as a whole).

In his first report (the case of focal senile atrophy and aphasia in a man of 71) Pick (1892) did not inculpate the frontal lobes nor did he in his second case (Pick, 1901) (a woman of 59 with generalized cortical atrophy, particularly of the left hemisphere).

The association with the frontal lobes appears only in his fourth case (Pick, 1906) (a 60-year-old man with ‘bilateral frontal atrophy’).  Which of these cases should, therefore, be considered as the first with Pick’s disease? At the time, in fact, no one thought that Pick had described a new disease; Barrett (1913) considered the two first cases of Pick’s as atypical forms of AD, and Ziehen (1911) did not see anything special in them.

During   the   same   period,  Liepmann,  Stransky,  and Spielmeyer had described similar cases with aphasia and circumscribed cerebral atrophy (Mansvelt, 1954); so much so, that Urechia and Mihalescu felt tempted to name the syndrome ‘Spielmeyer’s disease’ (Caron, 1934). This did not catch on, however, and in two classical papers on what he called ‘Pick’s disease’, Carl Schneider (1927, 1929)  constructed the new view of the condition by suggesting that it evolved in three stages - the first with a disturbance of judgement and behaviour, the second with localized symptoms (e.g. speech), and the third with generalized dementia. He recognized rapid and slow forms, the former with an akinetic and aphasic subtypes and a malignant course, and the latter with a predominance of plaques (probably indistinguishable from AD).

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