title: Psychiatric symptoms do not correlate with cognitive decline, motor symptoms, or CAG repeat length in Huntington's disease.
abstract: OBJECTIVE
  To investigate the hypothesis that psychiatric disturbances in Huntington's disease are related to degree of cognitive or motor compromise and to determine correlations between CAG repeat length within the gene for Huntington's disease and disease severity.
  
  
  METHODS
  Consecutive series of patients with Huntington's disease.
  
  
  METHODS
  Neurological specialty hospital.
  
  
  METHODS
  Seventeen men and 12 women from 24 families.
  
  
  METHODS
  The Hamilton Psychiatric and Anxiety Rating Scales and Brief Psychiatric Rating Scale were used to assess psychiatric disturbances; Folstein's Quantified Neurological Examination to evaluate motor status; and the Mini-Mental State Examination, Raven Progressive Matrices), Phonemic Verbal Fluency Test, Short Tale Test, Visual Search Test, and Benton's Visual Orientation Line Test to evaluate cognitive function. The length of the CAG repeat sequence in the Huntington's gene was determined by quantitative polymerase chain reaction.
  
  
  RESULTS
  Cognitive test scores correlated significantly with each other; of these, results of the Visual Search and Short Tale tests correlated significantly with the Folstein's Quantified Neurological Examination score (P = .05 and P = .03, respectively). Results of the Folstein's Quantified Neurological Examination also correlated with the illness duration and the length of the CAG repeat. Although psychiatric scores correlated significantly among themselves (P < .01), neither cognitive compromise, motor deterioration, nor CAG length were related to the extent of psychiatric compromise. Patients who were depressed when they were examined tended to have a history of psychiatric disorders.
  
  
  CONCLUSIONS
  The lack of correlation between disease severity and psychiatric disturbances indicates that psychiatric disorders progress nonlinearly, possibly because of differential degeneration of the striatal-cortical circuits; the possibility that psychiatric disorders are prevalent in certain families with a member who has Huntington's disease is being further investigated. The lack of correlation between CAG length and cognitive and psychiatric variables needs further investigation.
authors.affiliation: Istituto Nazionale Neurologico C. Besta, Milan, Italy.
authors.forename: B
authors.initials: B
authors.lastname: Zappacosta
authors.forename: D
authors.initials: D
authors.lastname: Monza
authors.forename: C
authors.initials: C
authors.lastname: Meoni
authors.forename: L
authors.initials: L
authors.lastname: Austoni
authors.forename: P
authors.initials: P
authors.lastname: Soliveri
authors.forename: C
authors.initials: C
authors.lastname: Gellera
authors.forename: R
authors.initials: R
authors.lastname: Alberti
authors.forename: M
authors.initials: M
authors.lastname: Mantero
authors.forename: G
authors.initials: G
authors.lastname: Penati
authors.forename: T
authors.initials: T
authors.lastname: Caraceni
authors.forename: F
authors.initials: F
authors.lastname: Girotti
mesh_terms: D000328:Adult; D001364:Awareness; D003072:Cognition Disorders / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D003717:Denial, Psychological; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D007406:Interview, Psychological; D008297:Male; D008875:Middle Aged; D019965:Neurocognitive Disorders / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D016133:Polymerase Chain Reaction; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D012803:Sick Role; D018911:Trinucleotide Repeats / Q000235:genetics*