The Outcome of Dopamine Dysregulation Syndrome in Parkinson's Disease: A Retrospective Postmortem Study

Abstract

Introduction We have performed a retrospective analysis of the frequency and relation to treatment of dopamine dysregulation syndrome (DDS) using the Queen Square Brain Bank (QSBB) database. Methods A search of the QSBB database for consecutive cases donated between 2005 and 2016 with a pathological diagnosis of Parkinson's disease was performed. Results DDS was present in 8.8% of cases, was more prevalent in males, and was associated with younger age of onset, longer disease duration, and more dopa‐induced dyskinesias. Treatment approaches for DDS included: reduction of levodopa, reduction/cessation of dopamine agonist (DA), and initiation of infusion therapies. DDS had completely resolved in just over half the patients. DA peak l‐dopa equivalent daily dose (LEDD) was higher in patients who failed to achieve remission. Conclusion This is the first study to provide data on the course of DDS until death. Treatment strategies consisted mainly of reduction of dopaminergic treatment, and, despite the majority of patients showing some improvement, half remained symptomatic. Successful treatment was associated with a lower l‐dopa dosage at death.

Implications of dopaminergic medication withdrawal in Parkinson’s disease

“…In the case of a DDS, complete discontinuation of levodopa will generally not be possible, but attempts should still be made to reduce the levodopa medication as much as possible. Tapering should be monitored closely, best as in-patient, and also adapted to the patients’ perception of his condition, to maintain his or her cooperation (Barbosa et al 2018 ).…”

Impulse control disorders and related behaviors in Parkinson's disease: risk factors, clinical and genetic aspects, and management

“…Hypersexuality is characterized by recurrently or persistently present sexual or erotic thoughts or fantasies and desire for sexual activity [8,10]. Repetitive behaviors such as, punding, hobbyism, walkabouts and hoarding and, as in DDS, the compulsive use of DRT medication, have also been described as related behaviors [11][12][13][14][15][16]. Punding results in repetitive, purposeless behaviors focused on specific activities or motor tasks such as unnecessary repetitive home repair or assembling and disassembling objects repetitively.…”

Too much of a good thing: hedonistic homoeostatic dysregulation and other behavioural consequences of excessive dopamine replacement therapy in Parkinson’s disease

“…The difficulty we described in attempting to reduce levodopa and dopamine replacement therapy in this population has been shared by others, with drug hoarding and obtaining additional supplies from different providers or now online particularly problematic. Many patients are resistant to therapeutic interventions with only 40%–53% of patients achieving remission in longitudinal studies 8 22…”