Frequency of new-onset pathologic compulsive gambling or hypersexuality after drug treatment of idiopathic Parkinson disease.

OBJECTIVE: To determine the frequency of new-onset compulsive gambling or hypersexuality among regional patients with Parkinson disease (PD), ascertaining the relationship of these behaviors to PD drug use. PATIENTS AND METHODS: We retrospectively reviewed the medical records of patients from 7 rural southeastern Minnesota counties who had at least 1 neurology appointment for PD between July 1, 2004, and June 30, 2006. The main outcome measure was compulsive gambling or hypersexuality developing after parkinsonism onset, including the temporal relationship to PD drug use. RESULTS: Of 267 patients with PD who met the study inclusion criteria, new-onset gambling or hypersexuality was documented in 7 (2.6%). All were among the 66 patients (10.6%) taking a dopamine agonist. Moreover, all 7 (18.4%) were among 38 patients taking therapeutic doses (defined as >/=2 mg of pramipexole or 6 mg of ropinirole daily). Behaviors were clearly pathologic and disabling in 5: 7.6% of all patients taking an agonist and 13.2% of those taking therapeutic doses. Of the 5 patients, 2 had extensive treatment for what was considered a primary psychiatric problem before the agonist connection was recognized. CONCLUSION: Among the study patients with PD, new-onset compulsive gambling or hypersexuality was documented in 7 (18.4%) of 38 patients taking therapeutic doses of dopamine agonists but was not found among untreated patients, those taking subtherapeutic agonist doses, or those taking carbidopa/levodopa alone. Behaviors abated with discontinuation of agonist therapy or dose reduction. Because this is a retrospective study, cases may have been missed, and hence this study may reflect an underestimation of the true frequency. Physicians who care for patients taking these drugs should recognize the drug's potential to induce pathologic syndromes that sometimes masquerade as primary psychiatric disease.

Inappropriate use of symptom-triggered therapy for alcohol withdrawal in the general hospital.

OBJECTIVE: To determine if hospitalized medical and surgical patients were placed inappropriately on symptom-triggered therapy (STT) for alcohol withdrawal syndrome (AWS) and if certain conditions were more likely to be associated with inappropriate STT use or adverse events. PATIENTS AND METHODS: We randomly selected 124 (25%) of the 495 Mayo Clinic inpatients who received STT according to the Revised Clinical Institute for Withdrawal Assessment for Alcohol (CIWA-Ar) protocol in 2003 and assessed them for STT appropriateness, defined as having both intact verbal communication and recent alcohol use. Adverse events, including delirium tremens, seizures, or death, were correlated with CIWA-Ar appropriateness. RESULTS: Of the 124 randomly selected patients, only 60 (48%) met both inclusion criteria. Of the remaining 64 patients, 9 (14%) were drinkers but could not communicate, and 35 (55%) could communicate but had not been drinking. Twenty (31%) met neither criterion. Univariate analysis identified a significant association between inappropriate initiation and chronic heart failure, postoperative status (POS), liver disease (LD), nonmetastatic cancer, and chemical dependency consultation. On multivariate analysis, only LD (P equals .02) and POS (P equals .01) retained significance, with LD more and POS less likely to predict appropriateness. Seven of 11 patients who experienced adverse events had received STT according to the CIWA-Ar protocol (P equals .05). Univariate analysis identified a significant association between adverse events and a history of alcohol dependence or AWS. Multivariate analysis showed significance only for a history of alcohol dependence (P equals .049). CONCLUSION: Fewer than half of the randomly selected patients met both of the inclusion criteria for the CIWA-Ar instrument, leading us to conclude that more stringent evaluation is needed. Particularly postoperatively, alternative explanations for putative AWS should be sought. Health care professionals should more aggressively seek information on recent alcohol use from medical records, family members, and patients themselves.

Candidate gene mutation analysis in idiopathic acquired sideroblastic anemia (refractory anemia with ringed sideroblasts).

BACKGROUND: For most cases of idiopathic acquired sideroblastic anemia (IASA), the molecular pathogenesis is unknown, despite the consistent morphological signature of abundant pathological ringed sideroblasts with their characteristic iron-engorged mitochondria. Moderately elevated free erythrocyte protoporphyrin (FEP) levels have been described in IASA, suggesting that the activity of ferrochelatase, the enzyme that catalyzes the final step in heme biosynthesis (incorporation of ferrous iron into protoporphyrin), might be diminished in erythroid progenitor cells from IASA patients. METHODS: We confirmed FEP elevation in IASA, then pursued a candidate gene approach that included screening the gene encoding ferrochelatase, FECH, for promoter and coding region mutations and mRNA expression changes in bone marrow from 37 patients with IASA. RESULTS: The analytical techniques employed detected mutations in a test cohort of previously undiagnosed patients with biochemical evidence for erythropoietic protoporphyria, a condition resulting from germline mutations in FECH, but somatic missense mutations of FECH and its promoter were not observed in IASA patients. FECH was modestly overexpressed in progenitor cells from patients with IASA, compared with MDS patients without sideroblasts and healthy controls. In addition, we analyzed ABCB7 and PUS1, genes implicated in congenital sideroblastic anemia syndromes, but again found no coding mutations in acquired cases. CONCLUSION: We conclude that acquired mutations in the factors currently known to cause inherited sideroblastic anemias are uncommon in IASA.