Deep Brain Stimulation in "On"-State Parkinson Hyperpyrexia P arkinsonism hyperpyrexia syndrome (PHS) is a life-threatening condition seen in Parkinson disease (PD) characterized by high fever, muscle rigidity, elevated creatine phosphokinase (CPK), autonomic instability, and altered consciousness.2][3] Withdrawal from dopamine replacement therapy (DRT), infection, injury, surgery, dehydration, and metabolic abnormalities can provoke PHS. 4 Treatment includes increasing the frequency of DRT and management of systemic complications.Mortality is 4% if treated and 16% if untreated. 5,6Among survivors, 30% have worsening of symptoms of parkinsonism and never return to their baseline.CASE REPORT A 52-year-old woman with a 17-year history of PD was referred for deep brain stimulation (DBS) surgery.She had bilateral tremor, rigidity, gait impairment, postural instability, motor fluctuations with dyskinesias, and wearing off symptoms of limb dystonia and freezing.Medications included carbidopa/ levodopa (CD/LD) and amantadine.The Unified Parkinson's Disease Rating Scale-motor subscore (UPDRS-III) in a challenged "on" state was 27 (video 1 on the Neurology ® Web site at www.neurology.org).Off medication (Ͼ12 hours) she had such severe tremor and rigidity in the waiting area that a physician passing by sent her to Figure Time course of parkinsonism hyperpyrexia syndrome and complications
Aims This study evaluated the association between provider types for patients with newly diagnosed Huntington's disease (HD) and healthcare resource utilization (HCRU), costs, and treatment patterns.
I thank Marques et al. for the interest in our study,1 the clarification, and the thoughtful comments. Their study reported that patients with Parkinson disease (PD) with postoperative emergence of restless legs syndrome (RLS) had higher preoperative and postoperative dopamine agonist (DA) doses, and had a lower percentage of DA reduction after deep brain stimulation (DBS),2 not "more significant reduction" as misinterpreted from our article.1 This underscores the controversy surrounding this issue since there are reports of RLS emergence after DBS in patients with PD with greater reduction of dopaminergic therapy,3 less reductions,2 and no correlation with degree of medication reduction.1 We thank Marques et al. for bringing attention to the complexity of this topic, and the call for further research in this area that might shed light on the effects of DBS on RLS symptoms and the mechanisms of RLS in general.
Chorea is the primary manifestation of Huntington's disease. Different clinicians pursue varied approaches to chorea management, and real-world evidence describing them is needed. The objective of this study was to assess the presence and severity of chorea, chorea pharmacotherapy, and treatment practice, and patterns in a large natural-history cohort with Huntington's disease. The Enroll-HD research platform Periodic Dataset 5.0 was used to select subjects. Outcomes included demographics, disease-related baseline characteristics (Primary Analysis Set), and treatment patterns (Treatment Analysis Set). A total of 2590 manifest participants comprised the Primary Analysis Set with 1040 in the Treatment Analysis Set; 96.8% of participants had chorea. Mean Unified Huntington's Disease Rating Scale scores for Total Maximal Chorea, Total Motor Score, and Total Functional Capacity were 9.6, 39.5, and 7.8, respectively. During the observation period from June 2012 to October 2020, 906 (36.1%) participants received treatment for chorea. Among these, the most common first-line therapies were monotherapy VMAT2 inhibitors (49.9%) and antipsychotics (27.7%), while 7.8% of participants discontinued first-line therapy. Of those receiving VMAT2 inhibitors or antipsychotics as first line, 92% and 84%, respectively, remained on VMAT2 inhibitors or antipsychotics alone or in combination for the duration of the study. The most common second-line treatment was combination therapy. Only 36.1% of participants with chorea were taking a medication indicated for chorea, and, while 49.9% of treated participants received VMAT2 inhibitors first-line, approximately half were prescribed off-label alternatives. It is unclear why patients with indications for treatment were untreated or why off-label alternatives were prescribed. Future research should elaborate on these observations.
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