Rinsho Shinkeigaku (Clinical Neurology)


Pick's disease: clinicopathological features for antemortem diagnosis

Osamu Yokota, M.D.1)2)4) and Kuniaki Tsuchiya, M.D.1)3)

1)Department of Neuropathology, Tokyo Institute of Psychiatry
2)Department of Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
3)Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital
4)Clinical Neuroscience Research Group, Greater Manchester Neurosciences Centre, Hope Hospital, University of Manchester

Frontotemporal lobar degeneration (FTLD) with ubiquitin/TDP-43-positive inclusions (FTLD-TDP) and Pick's disease are two major pathological substrates in sporadic FTLD patients. Although identifying these underlying pathologies during the life of the patient is crucial for specific pathology-based treatment in the future, adequate clinical data to infer pathologies are not available. Several recent studies demonstrated that Pick's disease cases tend to present clinically with frontotemporal dementia (FTD) or progressive non-fluent aphasia as the first syndrome, while sporadic FTLD-TDP cases frequently show semantic dementia. Some asymmetric motor disturbances (e.g., pyramidal signs, parkinsonism, and contracture) are frequent in sporadic FTLD-TDP during the course, but rare in Pick's disease. On the other hand, several previous studies have demonstrated that the most frequent first syndrome of FTLD-TDP with progranulin gene (PGRN) mutations is FTD and that neuronal loss in the frontal cortex is more severe than that in the temporal cortex. Therefore, it is plausible that the clinicopathological features of sporadic FTLD-TDP are different from those of Pick's disease and FTLD-TDP with PGRN mutations. Given that in vivo Aβ imaging will soon be put to practical use, clinical data useful for clinical differentiation of pathological subtypes of FTLD besides AD with atypical cerebral atrophy will be essential in the future.
Full Text of this Article in Japanese PDF (2578K)

(CLINICA NEUROL, 49: 235|248, 2009)
key words: Pick's disease, tau, TDP-43, clinical diagnosis

(Received: 6-Feb-09)