title: 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 using123I-2β-Carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl) nortropane (123I-FP-CIT) dopamine transporter single photon emission computed tomography (DAT-SPECT) and123I-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).
  
  
  CONCLUSIONS
  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.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Ohta
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Toru
authors.initials: T
authors.lastname: Yamashita
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Nozomi
authors.initials: N
authors.lastname: Hishikawa
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Kota
authors.initials: K
authors.lastname: Sato
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Kosuke
authors.initials: K
authors.lastname: Matsuzono
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Keiichiro
authors.initials: K
authors.lastname: Tsunoda
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Hatanaka
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
authors.forename: Mami
authors.initials: M
authors.lastname: Takemoto
authors.affiliation: Department of Neurology, St. Luke's international Hospital, 9-1 Akashi-cho, Chuo-ku, Tokyo 104-8560, Japan.
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Takemi
authors.affiliation: Department of Neurology, Ota Memorial Hospital, 3-6-28 Okinogami-cho, Fukuyama 720-0825, Japan.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Takamatsu
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan. Electronic address: yasuyuki@okayama-u.ac.jp.
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D019797:3-Iodobenzylguanidine; D000368:Aged; D001921:Brain / Q000000981:diagnostic imaging; D005190:Family; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D055414:Myocardial Perfusion Imaging; D010300:Parkinson Disease / Q000000981:diagnostic imaging / Q000503:physiopathology; D010375:Pedigree; D019275:Radiopharmaceuticals; D013132:Spinocerebellar Degenerations / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D013494:Supranuclear Palsy, Progressive / Q000000981:diagnostic imaging / Q000503:physiopathology; D015899:Tomography, Emission-Computed, Single-Photon; D014326:Tropanes
chemical_list: D019275:Radiopharmaceuticals; D014326:Tropanes; C087552:2-carbomethoxy-8-(3-fluoropropyl)-3-(4-iodophenyl)tropane; D019797:3-Iodobenzylguanidine
keywords: Asidan; DAT; MIBG; PD; PSP; SCA36