Potential multisystem degeneration in Asidan patients.

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.

• Authors: Yasuyuki Ohta, Toru Yamashita, Nozomi Hishikawa, Kota Sato, Kosuke Matsuzono, Keiichiro Tsunoda, Noriko Hatanaka, Mami Takemoto, Toshihiko Takemi, Kazuhiro Takamatsu, Koji Abe
• Journal: Journal of the neurological sciences (J Neurol Sci) Vol. 373 Pg. 216-222 (Feb 15 2017) ISSN: 1878-5883 [Electronic] Netherlands
• PMID: 28131191 (Publication Type: Comparative Study, Journal Article)
• Copyright: Copyright © 2017 Elsevier B.V. All rights reserved.

Chemical References

• Radiopharmaceuticals
• Tropanes
• 2-carbomethoxy-8-(3-fluoropropyl)-3-(4-iodophenyl)tropane
• 3-Iodobenzylguanidine

Topics

• 3-Iodobenzylguanidine
• Aged
• Brain (diagnostic imaging)
• Family
• Female
• Humans
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Myocardial Perfusion Imaging
• Parkinson Disease (diagnostic imaging, physiopathology)
• Pedigree
• Radiopharmaceuticals
• Spinocerebellar Degenerations (diagnostic imaging, physiopathology)
• Supranuclear Palsy, Progressive (diagnostic imaging, physiopathology)
• Tomography, Emission-Computed, Single-Photon
• Tropanes