Primary progressive aphasia as the initial manifestation of corticobasal degeneration and unusual tauopathies
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Primary progressive aphasia as the initial manifestation of corticobasal degeneration and unusual tauopathies. / Ferrer, I; Hernández, I; Boada, M; Llorente, A; Rey, M J; Cardozo, A; Ezquerra, M; Puig, B; Puig Martorell, Berta.
In: ACTA NEUROPATHOL, Vol. 106, No. 5, 01.11.2003, p. 419-35.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Primary progressive aphasia as the initial manifestation of corticobasal degeneration and unusual tauopathies
AU - Ferrer, I
AU - Hernández, I
AU - Boada, M
AU - Llorente, A
AU - Rey, M J
AU - Cardozo, A
AU - Ezquerra, M
AU - Puig, B
AU - Puig Martorell, Berta
PY - 2003/11/1
Y1 - 2003/11/1
N2 - The clinical, neuroradiological, neuropathological and biochemical findings in four patients with primary progressive aphasia and tauopathy are described. The aphasic syndrome preceded by several years the appearance of other symptoms in every case. Asymmetrical apraxia with alien hand phenomenon occurred in one case. Frontotemporal symptoms occurred in three cases, but progressed to dramatic cognitive devastation in only one of these. Generalized dementia consistent with probable Alzheimer's disease (AD) developed with time in another. Cerebral computer tomography scans, magnetic resonance imaging and SPECT studies revealed marked asymmetries in one case, and showed nonspecific cerebral atrophy in the remaining ones. The neuropathological examination revealed typical corticobasal degeneration (CBD) in one case; CBD and AD in another; and atypical CBD, argyrophilic grain disease (AGD) and alpha-synucleinopathy consistent with Parkinson's disease in a third. Unique neuropathological findings were found in the remaining case. This was characterized by severe cerebral atrophy, marked neuronal loss in the cerebral cortex and abnormal tau deposition in neurons of the cerebral cortex, diencephalon and brain stem. Ballooned neurons, Pick bodies, generalized cortical neurofibrillary tangles and astrocytic plaques were absent. However, massive globular inclusions, containing phospho-tau, occurred in glial cells, mainly oligodendrocytes, in the white matter. Biochemical studies of frontal homogenates revealed four bands of 73/74, 68, 64 and 60 kDa of phosphorylated tau (using antibodies recognizing phospho-tau Thr181, Ser262 and Ser422) in the patient with AD and CBD, suggesting a predominant AD pattern in this case. Two bands of 68 and 64 kDa of phospho-tau were recovered in the sarkosyl-insoluble fraction in the other three cases. This pattern is similar to that found in CBD, progressive supranuclear palsy and AGD. Taken together, the present series further supports pure and combined CBD as causes of primary progressive aphasia, and they extend the hypothesis that primary progressive aphasia may be the initial symptom of distinct tauopathies.
AB - The clinical, neuroradiological, neuropathological and biochemical findings in four patients with primary progressive aphasia and tauopathy are described. The aphasic syndrome preceded by several years the appearance of other symptoms in every case. Asymmetrical apraxia with alien hand phenomenon occurred in one case. Frontotemporal symptoms occurred in three cases, but progressed to dramatic cognitive devastation in only one of these. Generalized dementia consistent with probable Alzheimer's disease (AD) developed with time in another. Cerebral computer tomography scans, magnetic resonance imaging and SPECT studies revealed marked asymmetries in one case, and showed nonspecific cerebral atrophy in the remaining ones. The neuropathological examination revealed typical corticobasal degeneration (CBD) in one case; CBD and AD in another; and atypical CBD, argyrophilic grain disease (AGD) and alpha-synucleinopathy consistent with Parkinson's disease in a third. Unique neuropathological findings were found in the remaining case. This was characterized by severe cerebral atrophy, marked neuronal loss in the cerebral cortex and abnormal tau deposition in neurons of the cerebral cortex, diencephalon and brain stem. Ballooned neurons, Pick bodies, generalized cortical neurofibrillary tangles and astrocytic plaques were absent. However, massive globular inclusions, containing phospho-tau, occurred in glial cells, mainly oligodendrocytes, in the white matter. Biochemical studies of frontal homogenates revealed four bands of 73/74, 68, 64 and 60 kDa of phosphorylated tau (using antibodies recognizing phospho-tau Thr181, Ser262 and Ser422) in the patient with AD and CBD, suggesting a predominant AD pattern in this case. Two bands of 68 and 64 kDa of phospho-tau were recovered in the sarkosyl-insoluble fraction in the other three cases. This pattern is similar to that found in CBD, progressive supranuclear palsy and AGD. Taken together, the present series further supports pure and combined CBD as causes of primary progressive aphasia, and they extend the hypothesis that primary progressive aphasia may be the initial symptom of distinct tauopathies.
KW - Aged
KW - Aged, 80 and over
KW - Amyloid beta-Peptides
KW - Aphasia, Primary Progressive
KW - Astrocytes
KW - Blotting, Western
KW - Brain
KW - Female
KW - Glial Fibrillary Acidic Protein
KW - Humans
KW - Immunohistochemistry
KW - Magnetic Resonance Imaging
KW - Male
KW - Nerve Tissue Proteins
KW - Neurodegenerative Diseases
KW - Neurofibrillary Tangles
KW - Neurons
KW - Staining and Labeling
KW - Synucleins
KW - Tauopathies
KW - Tomography, Emission-Computed, Single-Photon
KW - alpha-Crystallin B Chain
KW - tau Proteins
U2 - 10.1007/s00401-003-0756-4
DO - 10.1007/s00401-003-0756-4
M3 - SCORING: Journal article
C2 - 12955398
VL - 106
SP - 419
EP - 435
JO - ACTA NEUROPATHOL
JF - ACTA NEUROPATHOL
SN - 0001-6322
IS - 5
ER -