Positron emission tomography in pallido-ponto-nigral degeneration (PPND) family (frontotemporal dementia with parkinsonism linked to chromosome 17 and point mutation in tau gene)

P. K. Pal, Zbigniew K Wszolek, A. Kishore, R. De La Fuente-Fernandez, V. Sossi, R. J. Uitti, T. Dobko, A. J. Stoessl

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Pallido-ponto-nigral degeneration (PPND) is a rapidly progressive disorder characterized by frontotemporal dementia with parkinsonism unresponsive to levodopa therapy. In this study, we have further characterized the regional abnormalities of cerebral function using PET with 6-[18F]fluoro-L-dopa (FD), [11C] raclopride (RAC), and 2-deoxy-2-fluoro-[18F]-D-glucose (FDG). FD and RAC scans were performed in 3 patients-2 new patients and a previously reported asymptomatic at-risk individual who became symptomatic 2years after the first FD scan. Cerebral glucose metabolism was studied by FDG in 2 other patients. In keeping with previous reports, there was a severe reduction of FD uptake, which affected both caudate and putamen to a similar degree in all 3 patients. RAC scans showed normal to elevated striatal D2-receptor binding in all patients. Cerebral glucose metabolism was globally reduced (>2 SD below control mean) in one patient, with maximal involvement of frontal regions, and to a lesser degree in the other patient. Our study showed severe presynaptic dopaminergic dysfunction with intact striatal D2 receptors in PPND patients, implying that the dopa unresponsiveness is probably a result of pathology downstream to the striatum. The pattern of presynaptic dysfunction contrasts with that seen in idiopathic parkinsonism, where the putamen is affected more than the caudate nucleus. The pattern of glucose hypometabolism correlates well with the presence of frontotemporal dementia.

Original languageEnglish (US)
Pages (from-to)81-88
Number of pages8
JournalParkinsonism and Related Disorders
Volume7
Issue number2
DOIs
StatePublished - 2001

Fingerprint

Frontotemporal Dementia
Chromosomes, Human, Pair 17
Substantia Nigra
Point Mutation
Positron-Emission Tomography
Levodopa
Raclopride
Genes
Corpus Striatum
Putamen
Glucose
Dihydroxyphenylalanine
Caudate Nucleus
Fluorodeoxyglucose F18
Parkinsonian Disorders
Pathology

Keywords

  • Dopamine D2 receptors
  • Fluorodeoxyglucose
  • Fluorodopa
  • Frontotemporal dementia
  • Pallido-ponto-nigral degeneration
  • Parkinsonism
  • Positron emission tomography

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Neurology

Cite this

Positron emission tomography in pallido-ponto-nigral degeneration (PPND) family (frontotemporal dementia with parkinsonism linked to chromosome 17 and point mutation in tau gene). / Pal, P. K.; Wszolek, Zbigniew K; Kishore, A.; De La Fuente-Fernandez, R.; Sossi, V.; Uitti, R. J.; Dobko, T.; Stoessl, A. J.

In: Parkinsonism and Related Disorders, Vol. 7, No. 2, 2001, p. 81-88.

Research output: Contribution to journalArticle

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