Rebecca Gilbert

Parkinsonism and Motor Neuron Diseases: Twenty-Seven Patients with Diverse Overlap Syndromes

It has long been recognized that signs of motor neuron disease (MND) may accompany clinical evidence of parkinsonism in different neurodegenerative conditions. By using the Columbia University Division of Movement Disorders database, we reviewed data from 5,500 cases of parkinsonism and recorded the presence of upper motor neuron (UMN) dysfunction, lower motor neuron (LMN) dysfunction, or both. Among the 27 patients so identified, we counted those with autonomic dysfunction, cerebellar dysfunction, or dementia. Among the 27 cases, seven had UMN signs and LMN signs as well as parkinsonism and were diagnosed with amyotrophic lateral sclerosis (ALS)‐parkinsonism (Brait‐Fahn disease). Three of the seven had dementia that was not deemed to be frontotemporal dementia (FTD). Six other patients had no LMN signs but had UMN signs and parkinsonism and were classified as having primary lateral sclerosis (PLS)‐parkinsonism. Four patients had both UMN and LMN signs with parkinsonism as well as the characteristic dementia of FTD; they were diagnosed with FTD‐parkinsonism‐ALS. Seven patients had MND, parkinsonism, and autonomic or cerebellar dysfunction, a combination compatible with multiple system atrophy (MSA). Three patients had syndromes compatible with hereditary spastic paraplegia (HSP). In sum, we found that MND occurs in association with diverse parkinsonian syndromes; some are heritable, others sporadic and causes are uncertain. Having MND may be a risk factor forparkinsonism. A prospective study may elucidate this possibility.

Tic Modulation Using Sensory Tricks

Background A sensory trick, or geste antagoniste, is defined as a physical gesture (such as a touch on a particular body part) that mitigates the production of an involuntary movement. This phenomenon is most commonly described as a feature of dystonia. Here we present a case of successful modulation of tics using sensory tricks. Case Report A case report and video are presented. The case and video demonstrate a 19-year-old male who successfully controlled his tics with various sensory tricks. Discussion It is underappreciated by movement disorder physicians that sensory tricks can play a role in tics. Introducing this concept to patients could potentially help in tic control. In addition, understanding the pathophysiological underpinnings of sensory tricks could help in the understanding of the pathophysiology of tics.

Outcomes of intradetrusor onabotulinum toxin A injection in patients with Parkinson's disease

To assess the safety and efficacy of intradetrusor onabotulinum toxin A injections for the treatment of overactive bladder (OAB) in patients with Parkinsons disease (PD).

A Neural Mechanism for the Opportunity Cost of Time

Recent interest has focused on a class of decision problems in which subjects encounter options serially and must decide when to leave an option in search of a better one, rather than directly comparing simultaneously presented options. Although such problems have a rich history in animal foraging and economics, relatively little is known about their neural substrates. Suggestively, however, a separate literature has argued that the key decision variable in these tasks – the opportunity cost of time, given by the average reward rate – may also govern behavioral vigor and may be reported by tonic dopamine (DA). In this study, we test whether this putative dopaminergic opportunity cost signal plays an analogous role in serial decisions by examining the behavior of patients with Parkinson’s disease (PD), on and off their DA replacement medication, in a patch-foraging task. In these tasks, subjects’ decisions about when to leave a depleting resource implicitly reflect their beliefs about the opportunity cost of time spent harvesting that resource. Consistent with the opportunity cost hypothesis, umedicated patients harvested longer than matched controls, and medication remediated this deficit. These effects were not explained by motor perseveration. Our results suggest a functional role for DA, and an associated cognitive deficit in PD, in a type of decision process that may be distinct from (but related to) the neuromodulator’s well studied roles in behavioral invigoration and learning from rewards. Significance Statement This study addresses two important questions whose answers are, unexpectedly, linked. First, what is the scope of cognitive functions of the neuromodulator dopamine, whose contributions – for instance, as assessed by both the motoric and more subtle cognitive deficits of patients with PD, which depletes dopamine – range from movement to reward and decision-making? Second, what are the neural mechanisms supporting an important but understudied class of problems, in which, rather than choose among a set of alternatives (like apples and oranges), one makes serial decisions about whether to stick with an option (like a job, or a mate) or seek another? We demonstrate a novel cognitive deficit in PD that integrates this function into the web of DA’s contributions.

Mirabegron in patients with Parkinson disease and overactive bladder symptoms: A retrospective cohort

INTRODUCTION This study aimed to assess the outcomes of mirabegron for the treatment of overactive bladder (OAB) symptoms in patients with Parkinson disease (PD). METHODS A retrospective study was conducted including patients with PD who received mirabegron 50 mg once daily for OAB symptoms between 2012 and 2017. The primary endpoint was clinical success defined as any improvement in overactive bladder symptoms self-assessed by the patients 6 weeks after mirabegron initiation. Secondary endpoints included number of pads per day, number of nocturia episodes and adverse events. RESULTS Fifty patients (mean 74 years old) were included. Before being treated with mirabegron, 56% had failed prior anticholinergic therapy. After 6 weeks of mirabegron 50 mg, five patients (11.4%) had a complete resolution of their OAB symptoms; 25 patients (50%) reported improvement, 23 (46%) reported no change and 2(4%) reported worsening of their OAB symptoms. The number of pads per day decreased from 1.5 to 0.9 (p = 0.01) and so did the number of nocturia episodes (from 3 to 2.6/night; p = 0.02). Only 2 adverse events were reported during mirabegron treatment (4%): one dizziness and one diaphoresis, that disappeared after mirabegron discontinuation. After a median follow-up of 19 months, 23 patients (46%) persisted on mirabegron. Persistence rates were 51.5%, 44.6% and 36.4% at 1, 2 and 3 years respectively. CONCLUSION Mirabegron has an excellent safety profile and appears to be an effective treatment for overactive bladder symptoms in patients with PD. Further prospective randomized trials are needed to properly assess mirabegron in PD patients.