Patient values and preferences regarding prognostic counseling in isolated REM sleep behavior disorder.

Study Objectives: Isolated REM sleep behavior disorder (iRBD) carries a high lifetime risk for phenoconversion to a defined neurodegenerative disease (NDD) including Parkinson disease, dementia with Lewy bodies, and multiple system atrophy. We aimed to examine iRBD patient values and preferences regarding prognostic counseling.

Methods: One hundred thirteen iRBD patient participants enrolled in the Mayo Clinic iRBD Patient Registry were sent an email survey concerning their values and preferences concerning NDD prognostic counseling and their experiences following diagnosis with iRBD.

Specialist approaches to prognostic counseling in isolated REM sleep behavior disorder.

Objectives/background: Most middle-aged and older adult patients with isolated (idiopathic) REM sleep behavior disorder (RBD) eventually develop parkinsonism, dementia with Lewy bodies, or multiple system atrophy. We aimed to describe the current sleep medicine specialist approach toward RBD prognostic counseling, and to determine physician beliefs and characteristics that impact provision of counseling.

Patients/methods: We surveyed 70 sleep medicine physicians with RBD expertise for demographic information, counseling practices, and their beliefs and understandings concerning the association between RBD and synucleinopathies, among other questions.

Physician and patient determinants of prognostic counseling in idiopathic REM sleep-behavior disorder.

Objectives/background: Prognostic counseling about the risk for developing overt neurodegenerative disorders for patients with idiopathic REM sleep-behavior disorder (iRBD) and isolated REM sleep without atonia (iRSWA) is difficult, given lack of disease-modifying interventions and uncertainty in accurate prognostication for individuals. We aimed to analyze patient and physician characteristics associated with documented prognostic discussions for patients with iRBD and iRSWA.

Patients/methods: We retrospectively reviewed the medical records for 138 (112 iRBD and 26 iRSWA) patients seen at the Mayo Clinic between 2012 and 2015.

Perioperative risks of narcolepsy in patients undergoing general anesthesia: A case-control study.

Study Objective: To compare the perioperative outcomes between patients with narcolepsy and matched controls undergoing anesthetic management.

Design: Retrospective 2:1 matched study design.

Setting: Large tertiary medical center.

Neuroimaging-evident lesional pathology associated with REM sleep behavior disorder.

Background/rationale: Rapid eye movement (REM) sleep behavior disorder (RBD) is a potentially injurious parasomnia characterized by dream enactment behavior and polysomnographic REM sleep without atonia (RSWA). Recently, RBD not only has been shown to be strongly associated with synucleinopathy neurodegeneration but has also been rarely reported to be associated with structural lesions involving the brainstem or limbic system. The aim of this study was to describe the clinical, neuroimaging, and outcome characteristics in a case series of patients with lesional RBD.

DPPX potassium channel antibody: frequency, clinical accompaniments, and outcomes in 20 patients.

Objective: To describe the detection frequency and clinical associations of immunoglobulin G (IgG) targeting dipeptidyl-peptidase-like protein-6 (DPPX), a regulatory subunit of neuronal Kv4.2 potassium channels.

Methods: Specimens from 20 patients evaluated on a service basis by tissue-based immunofluorescence yielded a synaptic immunostaining pattern consistent with DPPX-IgG (serum, 20; CSF, all 7 available).

Validation of the Mayo Sleep Questionnaire to screen for REM sleep behavior disorder in a community-based sample.

Objective: To validate a questionnaire focused on REM sleep behavior disorder (RBD) in a community-based sample.

Background: RBD is a parasomnia manifested by recurrent dream enactment behavior during REM sleep. While confirmation of RBD requires the presence of REM sleep without atonia on polysomnography (PSG), a screening measure for RBD validated in older adults would be desirable for clinical and research purposes.

Treatment outcomes in REM sleep behavior disorder.

Objective: REM sleep behavior disorder (RBD) is usually characterized by potentially injurious dream enactment behaviors (DEB). RBD treatment aims to reduce DEBs and prevent injury, but outcomes require further elucidation. We surveyed RBD patients to describe longitudinal treatment outcomes with melatonin and clonazepam.

Characteristics of REM sleep behavior disorder in childhood.

Study Objective: To describe our experience regarding the clinical and polysomnographic features of REM sleep behavior disorder (RBD) in childhood.

Methods: This was a retrospective chart review of children and adolescents with RBD and REM sleep without atonia. Demographics, and clinical and polysomnographic information were tabulated.

Nocturnal moaning and groaning-catathrenia or nocturnal vocalizations.

Purpose: Descriptions of nocturnal vocalizations, including catathrenia, are few. We undertook a study at our center on patients diagnosed with catathrenia, to evaluate the characteristic features of these events and their response to continuous positive airway pressure (CPAP) treatment.

Methods: Retrospective study of patients with a diagnosis of catathrenia who had an overnight polysomnogram (PSG) and available synchronized audio video recordings (to confirm the presence of moaning and groaning), at our center between January 2007 and May 2010.

Validation of the Mayo Sleep Questionnaire to screen for REM sleep behavior disorder in an aging and dementia cohort.

Objective: To validate a questionnaire focused on rapid eye movement sleep (REM) sleep behavior disorder (RBD) among participants in an aging and dementia cohort.

Background: RBD is a parasomnia that can develop in otherwise neurologically-normal adults as well as in those with a neurodegenerative disease. Confirmation of RBD requires polysomnography (PSG).

Impulse control disorders with the use of dopaminergic agents in restless legs syndrome: a case-control study.

Study Objectives: To determine the frequency of impulse control disorders (ICDs) with the use of dopaminergic agents in restless legs syndrome (RLS).

Design: Prospective case-control study using a screening questionnaire for ICDs, followed by phone interview to confirm diagnoses for those meeting preset scoring thresholds on the questionnaire.

Setting: Academic, comprehensive sleep medicine center.

Effects of continuous positive airway pressure on stridor in multiple system atrophy-sleep laryngoscopy.

The presence of stridor in patients with multiple system atrophy (MSA) is associated with poor prognosis, mainly due to a high risk of complete airway obstruction at night. Continuous positive airway pressure (CPAP) therapy has been proposed as a treatment of MSA-associated stridor, but, until now, there has been no visual documentation of the effect of CPAP on laryngeal patency during nonpharmacologically induced spontaneous sleep of a patient with MSA. We present a video-laryngoscopic documentation of a 57-year-old woman with MSA who was evaluated for nocturnal stridor.

Idiopathic rapid-eye-movement sleep disorder: associations with antidepressants, psychiatric diagnoses, and other factors, in relation to age of onset.

Background: A retrospective, case-control chart review was performed to examine the relationship between the age of onset of idiopathic RBD and secondary associations.

Methods: Forty-eight idiopathic RBD patients were divided into early-onset and late-onset groups, compared to each other, and to their respective non-RBD controls.

Results: There were more females in the early-onset group as compared to their older counterparts (45% vs.

Ornithine transcarbamylase deficiency presenting as encephalopathy during adulthood following bariatric surgery.

Background: Neurological complications following bariatric surgery are rare. Whereas nutritional deficiencies are the most common cause of neurological symptoms, the unmasking of previously subclinical metabolic disorders can also lead to significant morbidity.

Objective: To characterize the clinical presentation, serum biochemical fluctuations, and functional enzymatic analysis of a case of functional ornithine transcarbamylase deficiency unmasked by a dietary change following bariatric surgery.

Coma, hypertension, and brainstem imaging abnormalities in a paraplegic man with necrotizing fasciitis.

In this 2-part article, the reader has the opportunity to identify the differential diagnosis for the patient described in the title, on the basis of the history, examination results, laboratory evaluations, and neurophysiologic and imaging findings. The differential diagnosis and a discussion of the case are presented on pages 136-138.