Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations

Wakabayashi, K.; Oyanagi, Kiyomitsu; Makifuchi, Takao; Ikuta, Fusahiro; Homma, A.; Homma, Y.; Horikawa, Yo; Tokiguchi, S.

doi:10.1007/bf00293314

Cited by 150 publications

(64 citation statements)

References 32 publications

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“…b Those in the pyramidal neurons in the motor cortex were composed of STs, measuring 13-20 nm in diameter, and c those in the neurons of the nucleus centralis superior were composed of STs, 13-16 nm in diameter (STs straight tubules). Uranyl-lead, × 47,000 observed in CBD [11,26,37,43], were not seen in the present patient. From the view point of complications of PDC, granular hazy astrocytic inclusions, which have been observed in every patient with PDC [35], were not evident in the present patient.…”

Section: Discussioncontrasting

confidence: 41%

Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration

Oyanagi¹,

Chen

Craig

et al. 2000

Acta Neuropathol

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A 58-year-old Chamorro female patient, who died in 1993, was examined clinicopathologically. At the age of 51, she suffered from hemiparkinsonism, then bradykinesia, rigidity without tremor, and dementia. Extrapyramidal symptoms developed, and at the age of 57, vertical gaze palsy was noted. The clinical diagnosis was parkinsonism-dementia complex (PDC) with vertical gaze palsy. The brain showed atrophy in the frontal and temporal lobes, and the atrophy was accentuated in the dentate gyrus, Ammon's horn and parahippocampal gyrus. The basal ganglia, thalamus and midbrain were moderately atrophic. The substantia nigra and locus ceruleus were completely depigmented. Numerous neurofibrillary tangles (NFTs) were seen in the subiculum and amygdaloid nucleus. Many NFTs were evident in the parahippocampal gyrus, lateral occipitotemporal gyrus, insula, Sommer sector, basal nucleus of Meynert, lateral nucleus of the thalamus, subthalamic nucleus and brain stem, and several were observed in the globus pallidus and hypothalamus. The Sommer sector, substantia nigra, locus ceruleus and basal nucleus of Meynert showed severe loss of neurons, and a moderate loss of neurons was exhibited by the globus pallidus. These findings were apparently consistent with those associated with PDC. However, in this patient, severe neuronal loss was seen in the subthalamic nucleus and lateral nucleus of the thalamus, and grumose degeneration, which has not previously been reported in PDC, was seen in the dentate nucleus. In addition, many tufted astrocytes, which have been reported to occur in progressive supranuclear palsy (PSP) and postencephalitic parkinsonism, but scarcely observed in PDC, were present. Furthermore, astrocytic plaques, which have been considered as a specific finding of corticobasal degeneration (CBD), were observed in the cerebral cortex. On the other hand, granular hazy astrocytic inclusions, previously reported to occur in PDC, were not seen. Chromatolytic neurons were not observed. The question thus arises as to whether it is appropriate to consider this patient as having suffered from a combination of PDC, PSP and CBD. From the view points of absence of granular hazy astrocytic inclusions and chromatolytic neurons, and of tufted astrocytes in the neostriatum, it is conceivable that this patient is a case of a new disease entity.

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Section: Discussioncontrasting

confidence: 41%

Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration

Oyanagi¹,

Chen

Craig

et al. 2000

Acta Neuropathol

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“…Recently, it was reported that complicated cases have exhibited overlapping findings clinically and pathologically [3,12,18,19]. Furthermore, clinical diagnosis and pathological diagnosis are inconsistent with each other in some cases [6,10,11,24,25,27].A case whose clinical diagnosis does not correspond with pathological diagnosis is referred to as atypical CBD or PSP in this report. Pathological diagnosis of CBD and PSP is based on microscopic findings, but not gross findings according to the neuropathologic criteria [4,7,17].…”

Section: Introductionmentioning

confidence: 58%

“…Several atypical cases showed that microscopic findings of CBD or PSP extended beyond the specific areas for these diseases [3,6,10,18,19,24,25,27]. Degeneration with tau positive intranuclear inclusion in the nucleus of the aqua duct, locus ceruleus, pontine nucleus and dentate nucleus as well as the cerebrum were reported in the atypical CBD cases [18,19,25,27]. In contrast, the precentral cortical degeneration was reported in PSP cases [8,9,23].…”

Section: Discussionmentioning

confidence: 80%

“…The diversity of pathological findings of CBD and PSP has already been pointed out [14,15,22]. Several atypical cases showed that microscopic findings of CBD or PSP extended beyond the specific areas for these diseases [3,6,10,18,19,24,25,27]. Degeneration with tau positive intranuclear inclusion in the nucleus of the aqua duct, locus ceruleus, pontine nucleus and dentate nucleus as well as the cerebrum were reported in the atypical CBD cases [18,19,25,27].…”

Section: Discussionmentioning

confidence: 90%

“…Some cases shared the symptoms of CBD and PSP, and clinical diagnoses were different pathological diagnoses in other cases. We have referred to these cases as atypical PSP and CBD, including our cases, and discussed some issues to clarify confused features of these cases (Table 1) [3,6,10,18,19,24,25,27]. We have reviewed 7 atypical PSP cases which were diagnosed as CBD clinically [3,10,18,24].…”

Section: Discussionmentioning

confidence: 92%

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Discrepancy between clinical and pathological diagnoses of CBD and PSP

et al. 2005

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Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are often clinically confused with each other. Moreover, the discrepancy between clinical and pathological diagnoses of CBD and PSP are still controversial. We report here two atypical cases of PSP and CBD. A 73-year old woman was admitted with right hand rigidity, limb kinetic apraxia and cortical sensory loss. Brain atrophy, hypoperfusion and hypometabolism predominantly in the left frontoparietal lobes indicated CBD clinically. Pathological studies revealed neuronal loss and spongy change without ballooned neurons (BN) in the cerebral cortex. Modified Gallyas-Braak (G-B) staining revealed neurofibrillary tangles (NFTs) and tufted astrocytes, indicating pathological diagnosis of PSP. A 75-year-old man admitted with vertical gaze palsy, neck dystonia, parkinsonism and dementia. Atrophy of the frontal lobes and tegmentum of the midbrain and symmetrical frontal hypoperfusion in SPECT indicated PSP. However, neuronal loss and BN in the frontal lobes and clusters of astrocytic plaques indicated CBD pathologically. The G-B staining was useful for differentiating between CBD and PSP, but our atypical cases bring up a new issue about differential diagnosis of CBD and PSP.

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Missense point mutations of tau to segregate with FTDP-17 exhibit site-specific effects on microtubule structure in COS cells: A novel action of R406W mutation

2000

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Missense and splicing point mutations have been found in the tau gene in families with frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17). Of these mutations, we examined four exonic missense point mutations (G272V, P301L, V337M and R406W) in 3-repeat or 4-repeat tau isoform on the transfection experiment. The effects of two mutations (G272V or P301L) on microtubules were subtle whereas those of two other mutations (V337M or R406W) were dramatically significant when these two mutations were constructed into 3-repeat tau but not into 4-repeat tau. The R406W mutation induced an alternation of microtubules to form dotted or fragmented forms retaining colocalization of tau with tubulin whereas the V337M mutation predominantly disrupted microtubule networks and diminished colocalization of tau and tubulin. The effect of the mutations on microtubules were thus site-dependent and isoform-dependent. Tau with R406W mutation was found to be colocalized with tubulin without filamentous structures on confocal views, suggesting that the carboxyl region of tau played a different role from tubulin-binding domain on microtubule assemble. Another abnormal property was identified in tau with R406W mutation that failed to suffer phosphorylation. Thus, diverse effects of tau mutations on microtubules may explain the various clinicopathologies of FTDP-17 and related tauopathies.

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Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations

Cited by 150 publications

References 32 publications

Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration

Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration

Discrepancy between clinical and pathological diagnoses of CBD and PSP

Missense point mutations of tau to segregate with FTDP-17 exhibit site-specific effects on microtubule structure in COS cells: A novel action of R406W mutation

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