title: Dopamine dysregulation syndrome in Parkinson's disease: from clinical and neuropsychological characterisation to management and long-term outcome.
abstract: OBJECTIVE
  Dopamine dysregulation syndrome (DDS) refers to a compulsive pattern of dopaminergic drug misuse complicating Parkinson's disease (PD). To date, few data are available on DDS risk factors, cognitive profile and long-term outcome.
  
  
  METHODS
  In this retrospective case-control study, consecutive PD outpatients fulfilling criteria for DDS were assessed over a 6-year period (2005-2011). They were compared with 70 PD cases matched for age at onset, gender and disease duration, and with 1281 subjects with motor fluctuations and dyskinesias. DDS patients and matched controls underwent extensive neuropsychological assessment. Strategies for DDS patients management and the outcome at the last follow-up visit were recorded.
  
  
  RESULTS
  Thirty-five patients with DDS were identified, reporting history of depression, family history of PD and drug abuse, greater difference between 'Off' versus 'On' motor symptoms compared to age-matched controls. They had younger age at onset (but not any gender difference) compared to general PD population. Cognitive profile of DDS did not show major abnormalities, including executive functions. DDS patients have been followed up for 3.2±2.1 years and remission was recorded in 40% of cases. Negative DDS outcome was significantly associated with poor caregiver supervision. Sustained remission occurred more commonly on clozapine and on duodenal levodopa infusion and subthalamic nucleus deep brain stimulation (STN-DBS) than on apomorphine pump treatment.
  
  
  CONCLUSIONS
  Clinicians should be aware of risk factors predisposing to DDS. Duodenal levodopa infusion and, less consistently, STN-DBS were more commonly associated with DDS remission. Effective caregiving plays a key role in long-term behavioural outcome.
authors.affiliation: Parkinson Institute, Istituti Clinici di Perfezionamento, , Milan, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Cilia
authors.forename: Chiara
authors.initials: C
authors.lastname: Siri
authors.forename: Margherita
authors.initials: M
authors.lastname: Canesi
authors.forename: Anna Lena
authors.initials: AL
authors.lastname: Zecchinelli
authors.forename: Danilo
authors.initials: D
authors.lastname: De Gaspari
authors.forename: Francesca
authors.initials: F
authors.lastname: Natuzzi
authors.forename: Silvana
authors.initials: S
authors.lastname: Tesei
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Meucci
authors.forename: Claudio Bruno
authors.initials: CB
authors.lastname: Mariani
authors.forename: Giorgio
authors.initials: G
authors.lastname: Sacilotto
authors.forename: Michela
authors.initials: M
authors.lastname: Zini
authors.forename: Claudio
authors.initials: C
authors.lastname: Ruffmann
authors.forename: Gianni
authors.initials: G
authors.lastname: Pezzoli
mesh_terms: D016022:Case-Control Studies; D015259:Dopamine Agents / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D010300:Parkinson Disease / Q000150:complications / Q000188:drug therapy / Q000523:psychology*; D063487:Prescription Drug Misuse / Q000517:prevention & control / Q000523:psychology* / Q000706:statistics & numerical data; D011581:Psychological Tests; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D006113:United Kingdom / Q000453:epidemiology
chemical_list: D015259:Dopamine Agents
keywords: BEHAVIOURAL DISORDER; CLINICAL NEUROLOGY; MOVEMENT DISORDERS; NEUROPSYCHIATRY; PARKINSON'S DISEASE