Abstract
Objective To evaluate a potential multisystem involvement of neurodegeneration in Asidan, in addition to cerebellar ataxia and signs of motor neuron disease. Methods We compared the new Asidan patients and those identified in previous studies with Parkinson's disease (PD, n = 21), and progressive supranuclear palsy (PSP, n = 13) patients using 123I-2β-Carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl) nortropane (123I-FP-CIT) dopamine transporter single photon emission computed tomography (DAT-SPECT) and 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy (Asidan, DAT: n = 10; MIBG: n = 15). Results Both the PD and PSP groups served as positive controls for DAT decline. The PD and PSP groups served as a positive and negative control, respectively, of MIBG decline in the early phase H/M ratio. Of the Asidan patients, 60.0% showed DAT decline without evident parkinsonian features and 6.7% showed impaired MIBG in only the delayed phase H/M ratio. Combined with a normal range of the early phase H/M ratio, this phenotype was newly named Declined DAT Without Evident Parkinsonism (DWEP). Interpretation The results of present study including DWEP suggest a wider spectrum of neurodegeneration for extrapyramidal and autonomic systems in Asidan patients than expected, involving cerebellar, motor system and cognitive functioning.
| Original language | English |
|---|---|
| Pages (from-to) | 216-222 |
| Number of pages | 7 |
| Journal | Journal of the Neurological Sciences |
| Volume | 373 |
| DOIs | |
| Publication status | Published - Feb 15 2017 |
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Keywords
- Asidan
- DAT
- MIBG
- PD
- PSP
- SCA36
ASJC Scopus subject areas
- Neurology
- Clinical Neurology
Cite this
Potential multisystem degeneration in Asidan patients. / Ohta, Yasuyuki; Yamashita, Toru; Hishikawa, Nozomi; Sato, Kota; Matsuzono, Kosuke; Tsunoda, Keiichiro; Hatanaka, Noriko; Takemoto, Mami; Takemi, Toshihiko; Takamatsu, Kazuhiro; Abe, Koji.
In: Journal of the Neurological Sciences, Vol. 373, 15.02.2017, p. 216-222.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Potential multisystem degeneration in Asidan patients
AU - Ohta, Yasuyuki
AU - Yamashita, Toru
AU - Hishikawa, Nozomi
AU - Sato, Kota
AU - Matsuzono, Kosuke
AU - Tsunoda, Keiichiro
AU - Hatanaka, Noriko
AU - Takemoto, Mami
AU - Takemi, Toshihiko
AU - Takamatsu, Kazuhiro
AU - Abe, Koji
PY - 2017/2/15
Y1 - 2017/2/15
N2 - Objective To evaluate a potential multisystem involvement of neurodegeneration in Asidan, in addition to cerebellar ataxia and signs of motor neuron disease. Methods We compared the new Asidan patients and those identified in previous studies with Parkinson's disease (PD, n = 21), and progressive supranuclear palsy (PSP, n = 13) patients using 123I-2β-Carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl) nortropane (123I-FP-CIT) dopamine transporter single photon emission computed tomography (DAT-SPECT) and 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy (Asidan, DAT: n = 10; MIBG: n = 15). Results Both the PD and PSP groups served as positive controls for DAT decline. The PD and PSP groups served as a positive and negative control, respectively, of MIBG decline in the early phase H/M ratio. Of the Asidan patients, 60.0% showed DAT decline without evident parkinsonian features and 6.7% showed impaired MIBG in only the delayed phase H/M ratio. Combined with a normal range of the early phase H/M ratio, this phenotype was newly named Declined DAT Without Evident Parkinsonism (DWEP). Interpretation The results of present study including DWEP suggest a wider spectrum of neurodegeneration for extrapyramidal and autonomic systems in Asidan patients than expected, involving cerebellar, motor system and cognitive functioning.
AB - Objective To evaluate a potential multisystem involvement of neurodegeneration in Asidan, in addition to cerebellar ataxia and signs of motor neuron disease. Methods We compared the new Asidan patients and those identified in previous studies with Parkinson's disease (PD, n = 21), and progressive supranuclear palsy (PSP, n = 13) patients using 123I-2β-Carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl) nortropane (123I-FP-CIT) dopamine transporter single photon emission computed tomography (DAT-SPECT) and 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy (Asidan, DAT: n = 10; MIBG: n = 15). Results Both the PD and PSP groups served as positive controls for DAT decline. The PD and PSP groups served as a positive and negative control, respectively, of MIBG decline in the early phase H/M ratio. Of the Asidan patients, 60.0% showed DAT decline without evident parkinsonian features and 6.7% showed impaired MIBG in only the delayed phase H/M ratio. Combined with a normal range of the early phase H/M ratio, this phenotype was newly named Declined DAT Without Evident Parkinsonism (DWEP). Interpretation The results of present study including DWEP suggest a wider spectrum of neurodegeneration for extrapyramidal and autonomic systems in Asidan patients than expected, involving cerebellar, motor system and cognitive functioning.
KW - Asidan
KW - DAT
KW - MIBG
KW - PD
KW - PSP
KW - SCA36
UR - http://www.scopus.com/inward/record.url?scp=85008324810&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85008324810&partnerID=8YFLogxK
U2 - 10.1016/j.jns.2017.01.003
DO - 10.1016/j.jns.2017.01.003
M3 - Article
C2 - 28131191
AN - SCOPUS:85008324810
VL - 373
SP - 216
EP - 222
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
SN - 0022-510X
ER -