History of Pick's disease - 03/12/18
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Résumé |
Pick's disease (PiD) is considered as a rare disease and even if it has been the subject of many controversies, its role appears pivotal in the recognition of neurodegenerative diseases of non-Alzheimer type. After a short summary of Pick's biography [1] the different periods of individualization of the disease will be briefly reviewed as many books [2, 3, 4], theses [5, 6] and hundreds of articles have been published on this pathology.
Arnold Pick (1851–1924) was born of German Jewish parents in Gross-Meseritsch in Moravia, at the time controlled by the Austro-Hungarian Empire. After studying medicine, he became successively assistant to T.H. Meynert, Head of the Chair of Psychiatry in Vienna, when C. Wernicke was present, then to C.F.O. Westphal in Berlin. His training included Neurology, Psychiatry and Neuropathology. It has to be noted that he was considered as a Neuropathologist among the Founders of Neurology [7]. He came to Prague where he practiced in different psychiatric Departments and was nominated Professor of Psychiatry in 1886. Arnold Pick exchanged his ideas with many international Colleagues. He was well appreciated although he taught in German, even when many students were more fluent in Czech. He retired in 1921 and died of sepsis in 1924.
Arnold Pick published about 350 papers in German or English as well as a textbook on Neuropathology, mostly on apraxia, apragmatism and language disturbances. Between 1892 and 1906, he published five inaugural publications covering localized brain atrophy, the first one entitled “On the relationships between senile atrophy and aphasia” [8]. The analysis of these cases has shown that most of them were probably not what nowadays is called PiD; at the time [6] A. Pick did not realize that he was describing a new disease, but he wanted to emphasize the role of a localized atrophy, correlated with an amnestic aphasia. He suggested a degenerative process although, in this period, most dementias were supposed to result from vascular diseases or syphilis. Studying two such cases, A. Alzheimer, using the Bielchowski method, showed the presence of intracellular balls of different size and enlarged cells with an eccentric nucleus, that is the Pick's bodies and the ballooned cells [9] (Fig. 1). Subsequently, many cases of frontal and or temporal atrophy were studied but rejected by many authors because of the non-specific character of the lesions, the absence of senile plaques and Alzheimer neurofibrillary tangles being the unique argument [6]. Later on, two papers of importance were published. A. Gans (1923) used the term “Pick's atrophy” which became a source of confusion since it did not indicate the localization [10]. K. Onari and H. Spatz (1926) then identified the “Picksche Krankheit” (Pick's disease) in which the fronto-temporal atrophy was the source of cell loss of degenerative origin [11]. The clinical symptoms were then described by C. Schneider (1927–1929) [12]. The non-specificity was claimed by L. Marchand who described the “Abiotrophic dementias due to Encephalosis” [13].
New studies were performed in France (1962) [2] and in Switzerland (1975) [3]. They described a presenile frontal dementia with modifications of behavior and character as well as amnesic aphasia. Atrophy was present in the frontal lobe and in the temporal lobe, the posterior part of the superior gyrus being preserved (knife blade atrophy). Basal ganglia may have been involved as well as the white matter [14]. The main histological lesions were upper cortical neuronal loss, gliosis, microvacuolisation, ballooned neurons and argyrophilic inclusions and involvement of the hippocampus [15]. Tissot et al. (1975) described three forms:
– temporal atrophy;
– frontal atrophy with only ballooned cells;
– cellular lesions without significant atrophy.
The diagnosis was confirmed by silver staining techniques and electron microscopy studies showing the presence of straight filaments (100 to 120Å in diameter) [16, 17] and mixed with a variable number of twisted filaments.
The development of immunochemistry has facilitated the visualization of inclusions using antibodies, firstly against neurofilaments then against the normal and phosphorylated tau protein (family of microtubule-associated proteins). Thus the criteria of the disease seemed well established. However, in 1987, A. Brun described a frontal lobe degeneration (FLD) of non-Alzheimer type, different from PiD [18]. D. Neary reported a dementia of the frontal lobe (1988) [19], M.M. Mesulam a primary progressive aphasia (1992) [20], J.S. Snowsden a semantic dementia (1989) [21] and M.A. Neumann a Progressive Subcortical Gliosis (1967) [22]. Gradually, the concept of fronto-temporal dementia (FTD) was proposed, including a behavioral variant of FTD, progressive non-fluent aphasia, semantic aphasia and PiD also denominated Pick Complex [4]. Furthermore, biochemical studies of Pick brain homogenates showed that they were composed of a major doublet (tau60, tau64) and a minor tau 64 [23]. They were exclusively composed of 3R-tau, the microtubule binding repeat encoded by exon 10 being absent. As the same tau isoforms were present in Progressive Supranuclear Paralysis and Cortico-Basal Degeneration, a group of 3R-tau diseases was defined including PiD as part of the protein-based classification of neurodegenerative diseases. Based on this result, an exhaustive study using many new antibodies was recently published (2016) [24]. Four sequential patterns of pathological tau deposition were identified and tau-positivity of neuropil threads, ramified astrocytic inclusions and oligodendroglial coiled inclusions were demonstrated. The authors proposed a limbic/paralimbic cortices origin of PiD.
Some correlations between clinical and pathological subtypes may be observed, but the clinical presentation does not always predict the underlying pathology. Previous typical forms of PiD have been reported but they do not include tau detection: forms associated with ALS, few familial forms. This led to an interest in an ultrasensitive and selective detection of 3-repeat tau seeding activity in PiD brain and cerebral spinal fluid with RT-Quick r (2017) [25]. Perhaps this biomarker will help determine the unknown mechanism of disease development.
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Vol 174 - N° 10
P. 740-741 - décembre 2018 Retour au numéro
Article précédent
- From frontal neurobehavioral disorders to executive processes
- Olivier Godefroy, Groupe de Réflexion sur l’Évaluation des Fonctions Exécutives (GREFEX) study group
- History of frontal lobe seizures
- Michel Baulac
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