Spongiform Encephalopathy of Long Duration: A Family Study

Rice, George P.; Paty, Donald W.; Ball, Melvin; Tatham, R.

doi:10.1017/s0317167100023155

Cited by 10 publications

(4 citation statements)

References 12 publications

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“…Some cases of spongiform encephalopathy with PPA may have had Pick variant pathology, similar to one of our cases of FLD published previously, as a case of familial spongiform encephalopathy (Rice et al, 1980). However, prion proteins were never found and a recent review of the pathology revealed Pick complex.…”

supporting

confidence: 85%

Pick complex and Pick's disease: the nosology of frontal lobe dementia, primary progressive aphasia, and corticobasal ganglionic degeneration

Kertesz

1996

Euro J of Neurology

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supporting

confidence: 85%

Pick complex and Pick's disease: the nosology of frontal lobe dementia, primary progressive aphasia, and corticobasal ganglionic degeneration

Kertesz

1996

Euro J of Neurology

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“…We also included four patients, who were autopsied before 1990 and did not have the clinical diagnosis of FTD-bv, but had enough clinical information to fulfil the criteria. Two of these patients were members of a family published as 'long duration spongiform encephalopathy' (Rice et al, 1980) and subsequently as a family with probable genetic linkage to chromosome 17, but with tau negative, ubiquitin positive inclusions (Kertesz et al, 2000b). One patient was diagnosed as 'dementia with frontal features' and one from the University of Western Ontario dementia study diagnosed as atypical dementia, but both had documented onset with behavioural symptoms, followed by aphasia.…”

Section: Methods Patientsmentioning

confidence: 99%

The evolution and pathology of frontotemporal dementia

Kertesz

McMonagle²,

Blair³

et al. 2005

Brain

583

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This is a clinicopathologic study of a prospective, clinic-based cohort of patients with frontotemporal dementia (FTD)/Pick complex, who were followed to autopsy. A total of 60 patients with the clinical syndromes of the behavioural variant of FTD (FTD-bv) (n = 32), primary progressive aphasia (PPA) (n = 22), corticobasal degeneration syndrome (CBDS) (n = 4) and progressive supranuclear palsy (PSP) (n = 2) at onset, referred to a cognitive neurology clinic who had subsequent post-mortem examination were included. The most common histological variety was motor neurone disease type inclusion (MNDI) (n = 18), followed by corticobasal degeneration (CBD) (n = 12), then Pick's disease (n = 6), dementia lacking distinctive histology (DLDH) (n = 6) and PSP (n = 3). Others fulfilled the histological criteria for Alzheimer's disease combined with glial pathology (n = 6), Alzheimer's disease only (n = 4), Lewy body variant (n = 2), prion disease (n = 1), vascular dementia (n = 1) and undetermined (n = 1). The most common first syndrome among the MNDI and DLDH (tau negative) pathologies was FTD-bv, but subsequently progressive aphasia (PA), occasionally CBDS and semantic dementia also developed. Tau positive histologies of CBD, PSP and Pick bodies were most frequently associated with PPA onset or CBDS/PSP, but behavioural symptoms were also common. Age of onset was earlier in tau negative cases, but the duration of illness and gender distribution were about the same in all histological variants. Although the tau negative and positive histologies are predicted to some extent by the clinical onset, the extent of the overlap and the convergence of the syndromes in the course of the disease argue in favour of maintaining the clinical and pathological varieties under a single umbrella.

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“…Postmortem examination was reported by Rice et al, 11 but the material has since been lost. Postmortem examination was reported by Rice et al, 11 but the material has since been lost.…”

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confidence: 96%

Familial frontotemporal dementia with ubiquitin-positive, tau-negative inclusions

et al. 2000

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This family with autosomal dominant frontotemporal dementia (FTD) shows no tau expression in neurons. The ubiquitin-positive, tau-negative inclusions have been described before in FTD with and without motor neuron disease, but not in a familial form. The clinical and some pathologic features are similar to those of several of the families included in descriptions of FTD with parkinsonism linked to chromosome 17, but the linkage to tau has been excluded. The defect in this family, however, could be functionally related to tau mutations.

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Spongiform Encephalopathy of Long Duration: A Family Study

Cited by 10 publications

References 12 publications

Pick complex and Pick's disease: the nosology of frontal lobe dementia, primary progressive aphasia, and corticobasal ganglionic degeneration

Pick complex and Pick's disease: the nosology of frontal lobe dementia, primary progressive aphasia, and corticobasal ganglionic degeneration

The evolution and pathology of frontotemporal dementia

Familial frontotemporal dementia with ubiquitin-positive, tau-negative inclusions

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