Clinical and pathological study of two patients with progressive supranuclear palsy and Alzheimer's changes. Antigenic determinants that distinguish cortical and subcortical neurofibrillary tangles

Cruz-Sánchez, F. F.; Rossi, Marco; Cardozo, Adriana; Deacon, Patricia; Tolosa, Eduardo

doi:10.1016/0304-3940(92)90643-l

Cited by 20 publications

(9 citation statements)

References 17 publications

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“…The patients' average latency of the R2 response on the investigated side was longer than 40 ms, which is the upper limit of normal latencies 25. This suggests that the blink reflex pathways in the patients are impaired, and is consistent with a study describing neuronal loss and gliosis of brainstem nuclei including oculomotor nuclei 32. Another study11 reported normal R2 latencies, and an anatomical study described the cranial and spinal nerve roots as being “almost constantly spared” from severe neural loss 6.…”

Section: Discussionsupporting

confidence: 89%

Impairment of eyeblink classical conditioning in progressive supranuclear palsy

et al. 2001

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In a previous study we showed that learning in eyeblink classical conditioning (EBCC) is normal in Parkinson's disease (PD) and that the serial reaction time task (SRTT) is only marginally impaired. Since pathological lesions are more widespread in the atypical parkinsonian disorder of progressive supranuclear palsy (PSP) than in PD, we hypothesized that PSP patients may show more profound deficits in the EBCC and SRTT learning tasks. We therefore investigated EBCC with a delay and two trace paradigms, an SRTT and the California Verbal Learning Test (CVLT) in eight patients with PSP and an age-matched control group. In all EBCC paradigms, we found a significant difference between groups with no significant learning in PSP patients. In the SRTT, implicit learning may have been impaired, but verbal and manual sequence recall were only marginally impaired. Verbal memory was significantly worse in PSP patients than in the control group. Our study shows a dissociated pattern of learning abilities in PSP, where the EBCC as a measure of implicit learning is impaired, the explicit sequence detection in the SRTT is relatively preserved, and the verbal memory impaired. We hypothesize that the PSP patients' deficits in EBCC learning may be due to lesions of deep cerebellar nuclei. There may be a clinical role for EBCC in distinguishing PD and PSP patients.

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Section: Discussionsupporting

confidence: 89%

Impairment of eyeblink classical conditioning in progressive supranuclear palsy

et al. 2001

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“…NFTs and glialfibrillary tangles are present in both PSP and familial MSTD although the tau pathology in familial MSTD is more widespread. In familial MSTD, neuronal and glial tau deposits are ubiquitin-immunoreactive, although some tangles have been reported to be ubiquitin-negative in PSP (44,45). Furthermore, no tufted astrocytes (34) are present in familial MSTD.…”

Section: Fig 1 Temporal Cortex From a Familial Mstd Patient Showingmentioning

confidence: 95%

Familial multiple system tauopathy with presenile dementia: A disease with abundant neuronal and glial tau filaments

Spillantini¹,

Goedert

Crowther

et al. 1997

Proc. Natl. Acad. Sci. U.S.A.

324

214

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Neurofibrillary lesions made of hyperphosphorylated microtubule-associated protein tau constitute not only one of the defining neuropathological features of Alzheimer disease but also are present in a number of other neurodegenerative diseases with dementia. Here we describe a novel autosomal dominant disease named familial ''multiple system tauopathy with presenile dementia,'' which is characterized by abundant fibrillary deposits of tau protein in both neurons and glial cells. There are no detectable deposits of ␤-amyloid. The tau deposits are in the form of twisted filaments that differ in diameter and periodicity from the paired helical filaments of Alzheimer disease. They are stained by both phosphorylation-independent and -dependent anti-tau antibodies. Moreover, tau immunoreactivity coexists with heparan sulfate in affected nerve and glial cells. Tau protein extracted from filaments of familial multiple system tauopathy with presenile dementia shows a minor 72-kDa band and two major bands of 64 and 68 kDa that contain mainly hyperphosphorylated four-repeat tau isoforms of 383 and 412 amino acids.

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“…While PSP sometimes coexists with AGD, 36 PSP associated with AD has rarely been reported. [14][15][16][17][18] Individuals with typical clinical features of PSP and overlapping severe symptoms of dementia have been reported to show combined PSP and AD pathology. 14,15 Gearing et al 16 reported comorbidity of AD in five of 13 patients who had pathologically been diagnosed as having PSP.…”

Section: Discussionmentioning

confidence: 99%

“…Progressive supranuclear palsy and AD both belong to the same category of tauopathy, and may therefore be present in the same patient. However, there have been only a few reports of patients having both diseases 14–18 . Here, we report the details of two Japanese patients who were autopsy‐confirmed to be combined PSP with AD.…”

Section: Introductionmentioning

confidence: 96%

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Progressive supranuclear palsy combined with Alzheimer's disease: A clinicopathological study of two autopsy cases

Sakamoto¹,

Tsuchiya²,

Yoshida³

et al. 2009

Neuropathology

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We present here the clinicopathological characteristics of two autopsy-confirmed cases comorbid of progressive supranuclear palsy (PSP) and Alzheimer's disease (AD). Histopathologically, the amount and distribution of neurofibrillary tangles (NFTs) in the basal ganglia and brainstem fulfilled the pathological criteria of PSP proposed by the National Institute of Neurological Disorders and Stroke--The Society for PSP (NINDS-SPSP). The Braak stages of senile plaques and NFTs were stage C and stage V in Case 1, and stage C and stage IV in Case 2. These neuropathological findings confirmed that the two patients had combined PSP with AD. Our patients presented clinically with executive dysfunction prior to memory disturbance as an early symptom. Not only neurological symptoms such as gait disturbance, supranuclear ophthalmoplegia and pseudobulbar palsy, but emotional and personality changes and delirium were prominent. Therefore, symptoms of subcortical dementia of PSP were more predominant than AD-related symptoms in the present two patients. Comorbid PSP and AD further complicates the clinical picture and makes clinical diagnosis even more difficult.

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Clinical and pathological study of two patients with progressive supranuclear palsy and Alzheimer's changes. Antigenic determinants that distinguish cortical and subcortical neurofibrillary tangles

Cited by 20 publications

References 17 publications

Impairment of eyeblink classical conditioning in progressive supranuclear palsy

Impairment of eyeblink classical conditioning in progressive supranuclear palsy

Familial multiple system tauopathy with presenile dementia: A disease with abundant neuronal and glial tau filaments

Progressive supranuclear palsy combined with Alzheimer's disease: A clinicopathological study of two autopsy cases

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