Please use this identifier to cite or link to this item: http://hdl.handle.net/2445/126019
Title: Globular glial tauopathies (GGT): consensus recommendations
Author: Ahmed, Zeshan
Bigio, Eileen H.
Budka, Herbert
Dickson, Dennis W.
Ferrer, Isidro (Ferrer Abizanda)
Ghetti, Bernardino
Giaccone, Giorgio
Hatanpaa, Kimmo J.
Holton, Janice L.
Josephs, Keith A.
Powers, James
Spina, Salvatore
Takahashi, Hitoshi
White III, Charles L.
Revesz, Tamas
Kovacs, Gabor G.
Keywords: Malalties neurodegeneratives
Atròfia muscular
Paràlisi
Neurodegenerative diseases
Muscular atrophy
Paralysis
Issue Date: 1-Oct-2013
Publisher: Springer Verlag
Abstract: Recent studies have highlighted a group of 4-repeat (4R) tauopathies that are characterised neuropathologically by widespread, globular glial inclusions (GGIs). Tau immunohistochemistry reveals 4R immunoreactive globular oligodendroglial and astrocytic inclusions and the latter are predominantly negative for Gallyas silver staining. These cases are associated with a range of clinical presentations, which correlate with the severity and distribution of underlying tau pathology and neurodegeneration. Their heterogeneous clinicopathological features combined with their rarity and under-recognition have led to cases characterised by GGIs being described in the literature using various and redundant terminologies. In this report, a group of neuropathologists form a consensus on the terminology and classification of cases with GGIs. After studying microscopic images from previously reported cases with suspected GGIs (n = 22), this panel of neuropathologists with extensive experience in the diagnosis of neurodegenerative diseases and a documented record of previous experience with at least one case with GGIs, agreed that (1) GGIs were present in all the cases reviewed; (2) the morphology of globular astrocytic inclusions was different to tufted astrocytes and finally that (3) the cases represented a number of different neuropathological subtypes. They also agreed that the different morphological subtypes are likely to be part of a spectrum of a distinct disease entity, for which they recommend that the overarching term globular glial tauopathy (GGT) should be used. Type I cases typically present with frontotemporal dementia, which correlates with the fronto-temporal distribution of pathology. Type II cases are characterised by pyramidal features reflecting motor cortex involvement and corticospinal tract degeneration. Type III cases can present with a combination of frontotemporal dementia and motor neuron disease with fronto-temporal cortex, motor cortex and corticospinal tract being severely affected. Extrapyramidal features can be present in Type II and III cases and significant degeneration of the white matter is a feature of all GGT subtypes. Improved detection and classification will be necessary for the establishment of neuropathological and clinical diagnostic research criteria in the future.
Note: Versió postprint del document publicat a: https://doi.org/10.1007/s00401-013-1171-0
It is part of: Acta Neuropathologica, 2013, vol. 126, num. 4, p. 537-544
URI: http://hdl.handle.net/2445/126019
Related resource: https://doi.org/10.1007/s00401-013-1171-0
ISSN: 0001-6322
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Patologia i Terapèutica Experimental)

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