title: Unawareness of motor phenoconversion in Huntington disease.
abstract: OBJECTIVE
  To determine whether Huntington disease (HD) mutation carriers have motor symptoms (complaints) when definite motor onset (motor phenoconversion) is diagnosed and document differences between the groups with and without unawareness of motor signs.
  
  
  METHODS
  We analyzed data from 550 HD mutation carriers participating in the multicenter PREDICT-HD Study followed through the HD prodrome. Data analysis included demographics, the Unified Huntington's Disease Rating Scale (UHDRS) and the Participant HD History of symptoms, self-report of progression, and cognitive, behavioral, and imaging measures. Unawareness was identified when no motor symptoms were self-reported but when definite motor HD was diagnosed.
  
  
  RESULTS
  Of 38 (6.91%) with onset of motor HD, almost half (18/38 = 47.36%) had no motor symptoms despite signs of disease on the UHDRS motor rating and consistent with unawareness. A group with motor symptoms and signs was similar on a range of measures to the unaware group. Those with unawareness of HD signs reported less depression. Patients with symptoms had more striatal atrophy on imaging measures.
  
  
  CONCLUSIONS
  Only half of the patients with newly diagnosed motor HD had motor symptoms. Unaware patients were less likely to be depressed. Self-report of symptoms may be inaccurate in HD at the earliest stage.
authors: From the Neurology Department (E.A.M., D.G.G., C.T.L., K.R., J.G.), Westmead Hospital, Sydney; Sydney Medical School (E.A.M., C.T.L.), University of Sydney, Australia; and Department of Psychiatry (E.A.E., J.A.M., J.D.L, J.S.P.), University of Iowa, Iowa City.
authors: Elizabeth A
authors: EA
authors: McCusker
authors: David G
authors: DG
authors: Gunn
authors: Eric A
authors: EA
authors: Epping
authors: Clement T
authors: CT
authors: Loy
authors: Kylie
authors: K
authors: Radford
authors: Jane
authors: J
authors: Griffith
authors: James A
authors: JA
authors: Mills
authors: Jeffrey D
authors: JD
authors: Long
authors: Jane S
authors: JS
authors: Paulsen
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D001364:Awareness; D016009:Chi-Square Distribution; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000150:complications* / Q000235:genetics / Q000473:pathology / Q000523:psychology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D010641:Phenotype; D011597:Psychomotor Performance; D057566:Self Report; D012720:Severity of Illness Index; D013223:Statistics as Topic
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