title: Depression and clinical progression in spinocerebellar ataxias.
abstract: BACKGROUND
  Depression is a common comorbidity in spinocerebellar ataxias (SCAs) but its association with ataxia progression is not well understood.
  
  
  OBJECTIVE
  To study the prevalence and influence of depressive symptoms in SCAs.
  
  
  METHODS
  We studied 300 participants with SCA 1, 2, 3 and 6 from the Clinical Research Consortium for Spinocerebellar Ataxias (CRC-SCA) and repeatedly measured depressive symptoms by the 9-item Patient Health Questionnaire (PHQ-9) along with other clinical features including ataxia, functional status, and quality of life every 6 months for 2 years. We employed regression models to study the effects of depressive symptoms on clinical progression indexed by Scale for Assessment and Rating of Ataxia (SARA), Unified Huntington's Disease Rating Scale Part IV (UHDRS-IV) and EQ5D after adjusting for age, sex and pathological CAG repeats.
  
  
  RESULTS
  Comorbid depression is common in SCAs (26%). Although the baseline prevalence of depression was similar among different SCA types, suicidal ideation was more frequently reported in SCA3 (65%). Depressive symptoms were associated with SARA scores but did not significantly progress over time within 2 years or deteriorate by increased numbers of pathological CAG repeats. The effects of depression on ataxia progression varied across different SCA types. Nevertheless, depression had consistently negative and significant impact on functional status and quality of life in all SCAs, even after accounting for ataxia progression.
  
  
  CONCLUSIONS
  Depressive symptoms are not simply the consequence of motor disability in SCAs. Comorbid depression per se contributes to different health outcomes and deserves more attention when caring patients with SCAs.
authors.affiliation: Department of Neurology, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien, Taiwan.
authors.forename: Raymond Y
authors.initials: RY
authors.lastname: Lo
authors.affiliation: Department of Neurology, University of Utah, Salt Lake City, UT, USA.
authors.forename: Karla P
authors.initials: KP
authors.lastname: Figueroa
authors.affiliation: Department of Neurology, University of Utah, Salt Lake City, UT, USA.
authors.forename: Stefan M
authors.initials: SM
authors.lastname: Pulst
authors.affiliation: Department of Neurology, University of California, Los Angeles, CA, USA.
authors.forename: Susan
authors.initials: S
authors.lastname: Perlman
authors.affiliation: Department of Neurology, Emory University, Atlanta, GA, USA.
authors.forename: George
authors.initials: G
authors.lastname: Wilmot
authors.affiliation: Department of Neurology, University of Chicago, Chicago, IL, USA.
authors.forename: Christopher
authors.initials: C
authors.lastname: Gomez
authors.affiliation: Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Jeremy
authors.initials: J
authors.lastname: Schmahmann
authors.affiliation: Department of Neurology, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Henry
authors.initials: H
authors.lastname: Paulson
authors.affiliation: Department of Neurology, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Vikram G
authors.initials: VG
authors.lastname: Shakkottai
authors.affiliation: Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Sarah
authors.initials: S
authors.lastname: Ying
authors.affiliation: Department of Neurology, University of South Florida, Tampa, FL, USA.
authors.forename: Theresa
authors.initials: T
authors.lastname: Zesiewicz
authors.affiliation: Department of Neurology, University of Minnesota, Minneapolis, MN, USA.
authors.forename: Khalaf
authors.initials: K
authors.lastname: Bushara
authors.affiliation: Department of Neurology, University of California, San Francisco, CA, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Geschwind
authors.affiliation: Department of Neurology and McKnight Brain Institute, University of Florida, Gainesville, FL, USA.
authors.forename: Guangbin
authors.initials: G
authors.lastname: Xia
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
authors.forename: Jui-Tsen
authors.initials: JT
authors.lastname: Yu
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
authors.forename: Lue-En
authors.initials: LE
authors.lastname: Lee
authors.affiliation: Department of Neurology and McKnight Brain Institute, University of Florida, Gainesville, FL, USA.
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Ashizawa
authors.affiliation: Department of Neurology and McKnight Brain Institute, University of Florida, Gainesville, FL, USA.
authors.forename: S H
authors.initials: SH
authors.lastname: Subramony
authors.affiliation: Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA. Electronic address: sk3295@columbia.edu.
authors.forename: Sheng-Han
authors.initials: SH
authors.lastname: Kuo
mesh_terms: D000328:Adult; D000368:Aged; D015897:Comorbidity; D003863:Depression / Q000453:epidemiology* / Q000523:psychology; D018450:Disease Progression; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000453:epidemiology / Q000523:psychology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012720:Severity of Illness Index; D020754:Spinocerebellar Ataxias / Q000453:epidemiology* / Q000523:psychology; D059020:Suicidal Ideation
keywords: Cerebellum; Depression; Neurodegeneration; Spinocerebellar ataxias; Suicide