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Published in: Journal of Neurology 9/2020

01-09-2020 | Apraxia | Original Communication

Ioflupane 123I (DAT scan) SPECT identifies dopamine receptor dysfunction early in the disease course in progressive apraxia of speech

Authors: Zeynep Idil Seckin, Jennifer L. Whitwell, Rene L. Utianski, Hugo Botha, Farwa Ali, Joseph R. Duffy, Heather M. Clark, Mary M. Machulda, Lennon G. Jordan, Hoon-Ki Min, Val J. Lowe, Keith A. Josephs

Published in: Journal of Neurology | Issue 9/2020

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Abstract

Objective

To describe 123I-FP-CIT (DAT scan) SPECT findings in progressive apraxia of speech (PAOS) patients and to compare those findings with progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS).

Background

PAOS is a neurodegenerative syndrome in which patients present with apraxia of speech, a motor speech disorder affecting programming and planning of speech. Patients with PAOS predictably develop Parkinsonism. DAT scan is a neuroimaging tool that assesses the integrity of presynaptic dopamine transporters in striatum and is usually abnormal in PSP and CBS.

Methods

As part of an NIH-funded grant, we performed a DAT scan on 17 PAOS patients early in the disease course. DaTQUANT software was used to quantify uptake in the left and right caudate and anterior/posterior putamen, with striatum to background ratios (SBRs). The PAOS cohort was compared to 15 PSP and 8 CBS patients.

Results

Five PAOS patients (29%) showed abnormalities in at least one striatal region on DAT scan. When the five PAOS patients with abnormal DAT were compared to the PSP and CBS patients, the only difference observed was lower uptake in the posterior putamen in PSP (p = 0.03). There were no differences is putamen/caudate ratio or in symmetry of uptake, across all groups. There was also no difference in MDS-UPDRS-III scores between PAOS patients with and without abnormal DAT scans (p = 0.56).

Conclusions

Abnormal DAT scan is observed early in the disease course in approximately 30% of PAOS patients, with striatal abnormalities similar to those in PSP and CBS.
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Metadata
Title
Ioflupane 123I (DAT scan) SPECT identifies dopamine receptor dysfunction early in the disease course in progressive apraxia of speech
Authors
Zeynep Idil Seckin
Jennifer L. Whitwell
Rene L. Utianski
Hugo Botha
Farwa Ali
Joseph R. Duffy
Heather M. Clark
Mary M. Machulda
Lennon G. Jordan
Hoon-Ki Min
Val J. Lowe
Keith A. Josephs
Publication date
01-09-2020
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 9/2020
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-020-09883-4

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