Objective: to determine whether Dextrometorphan/quinidine (DMQ) is effective in the treatment of PBA, AG or bulbar symptoms in corticobasal syndrome (CBS), multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and Huntington disease (HD).

Background: Pseudobulbar affect (PBA) may occur in different neurological conditions, it is characterized by sudden, unprovoked and involuntary episodes of laughing and/or crying.  Agitation (AG) is another condition affecting persons with dementia. PBA and AG constitute a highly distressing condition to patients, their families and caregivers. FDA approved DMQ for the treatment of PBA, in patients with underlying amyotrophic lateral sclerosis  or multiple sclerosis. Preliminary studies suggested the potential efficacy of DMQ to treat PBA and AG as well as impaired speech, swallowing, and saliva control in other neurodegenerative disorders.

Methods: We conducted an open-label study, approved by institutional Committee.  After signed an informed consent, 6 adult patients, with a normal electrocardiogram and stable concurrent medications, were included. CBS criteria were fulfilled in 3 patients, MSA in 1, PSP in 1 and HD in 1. A baseline visit included a neurological examination, Pittsburgh Agitation Scale (PAS), Pathological Laughing and Crying Scale (PLACS), when appropriate. Follow-up visits occurred at 1st and 6 weeks, including CGI scale. DMQ was administered once a day for one week and then titrated over a 2-week period to a dose of twice daily. 

Paired T test was done between baseline and follow-up variables. P value ≤0.05 was considered significant

Results:  Six patients compose the sample, median age  66 years old . Median baseline score of PAS was  3 ( 0-10) and 0 ( 0-8) at the follow up (p:0.009). Mean baseline PLACS score  8 (0-17), follow up 0 (0-8), p: 0.009. CGI showed an improvement of 2 (1-3), severity 6 (4-7), a significant therapy effect was observed in 66.66% (4/6 cases) of patients. Family, caregivers reported subjective improvement of symptoms in all patients.No side effects, nor pharmacological interactions were observed.

Conclusions: In this series DMQ was safe and effective  in atypical parkinsonisms and HD. More extensive and randomized studies are warranties.

References: Hammond FM, Alexander DN, Cutler AJ, D’Amico S, Doody RS, Sauve W, Zorowitz RD, Davis CS, Shin P, Ledon F, Yonan C, Formella AE, Siffert J. PRISM II: an open-label study to assess effectiveness of dextromethorphan/quinidine for pseudobulbar affect in patients with dementia, stroke or traumatic brain injury. BMC Neurol. 2016 Jun 9;16:89. doi: 10.1186/s12883-016-0609-0. Erratum in: BMC Neurol. 2016;16(1):160.

Pattee GL, Wymer JP, Lomen-Hoerth C, Appel SH, Formella AE, Pope LE. An open-label multicenter study to assess the safety of dextromethorphan/quinidine in patients with pseudobulbar affect associated with a range of underlying neurological conditions. Curr Med Res Opin. 2014 Nov;30(11):2255-65. doi: 10.1185/03007995.2014.940040.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/dextrometorphanquinidine-in-atypical-parkinsonisms-and-huntington-disease/