Heidi Schwarz

A subtype of sporadic prion disease mimicking fatal familial insomnia

Objective: To establish a variant of sporadic prion disease as the sporadic form of fatal familial insomnia (FFI). Background: FFI is a recently described prion disease characterized clinically by severe sleep impairment, dysautonomia, and motor signs, and pathologically by atrophy of thalamic nuclei, especially the medial dorsal and anterior ventral, and of the inferior olive. FFI is linked to the D178N mutation coupled with the methionine codon at position 129 in the prion protein gene (PRNP). It is also identified by the properties of the abnormal prion protein (PrPSc), which has the relative molecular mass of 19 kDa, corresponding to the so-called type 2, and a marked underrepresentation of the unglycosylated form relative to the diglycosylated and monoglycosylated forms. Methods: Clinical, pathologic, PrPSc, and PRNP data from 5 subjects with a sporadic prion disease phenotypically similar to FFI were collected and analyzed. Results: All 5 subjects had a disease clinically similar and histopathologically virtually identical to FFI. PrPSc type 2 was present in all subjects in amount and distribution similar to those of FFI. However, the PrPSc did not show the striking underrepresentation of the unglycosylated isoform of the protein that is characteristic of FFI. Moreover, none of the subjects had the D178N PRNP mutation but all were homozygous for methionine at codon 129. Conclusion: This condition is likely to represent the sporadic form of FFI and the term “sporadic fatal insomnia” is proposed.

Burnout, career satisfaction, and well-being among US neurologists in 2016

Objective: To study prevalence of and factors that contribute to burnout, career satisfaction, and well-being in US neurologists. Methods: A total of 4,127 US American Academy of Neurology member neurologists who had finished training were surveyed using validated measures of burnout, career satisfaction, and well-being from January 19 to March 21, 2016. Results: Response rate was 40.5% (1,671 of 4,127). Average age of participants was 51 years, with 65.3% male and nearly equal representation across US geographic regions. Approximately 60% of respondents had at least one symptom of burnout. Hours worked/week, nights on call/week, number of outpatients seen/week, and amount of clerical work were associated with greater burnout risk. Effective support staff, job autonomy, meaningful work, age, and subspecializing in epilepsy were associated with lower risk. Academic practice (AP) neurologists had a lower burnout rate and higher rates of career satisfaction and quality of life than clinical practice (CP) neurologists. Some factors contributing to burnout were shared between AP and CP, but some risks were unique to practice setting. Factors independently associated with profession satisfaction included meaningfulness of work, job autonomy, effectiveness of support staff, age, practicing sleep medicine (inverse relationship), and percent time in clinical practice (inverse relationship). Burnout was strongly associated with decreased career satisfaction. Conclusions: Burnout is common in all neurology practice settings and subspecialties. The largest driver of career satisfaction is the meaning neurologists find in their work. The results from this survey will inform approaches needed to reduce burnout and promote career satisfaction and well-being in US neurologists.

National Randomized Controlled Trial of Virtual House Calls for People with Parkinson's Disease: Interest and Barriers

BACKGROUND Delivering specialty care remotely directly into peoples homes can enhance access for and improve the healthcare of individuals with chronic conditions. However, evidence supporting this approach is limited. MATERIALS AND METHODS Connect.Parkinson is a randomized comparative effectiveness study that compares usual care of individuals with Parkinsons disease in the community with usual care augmented by virtual house calls with a Parkinsons disease specialist from 1 of 18 centers nationally. Individuals in the intervention arm receive four virtual visits from a Parkinsons disease specialist over 1 year via secure, Web-based videoconferencing directly into their homes. All study activities, including recruitment, enrollment, and assessments, are conducted remotely. Here we report on interest, feasibility, and barriers to enrollment in this ongoing study. RESULTS During recruitment, 11,734 individuals visited the studys Web site, and 927 unique individuals submitted electronic interest forms. Two hundred ten individuals from 18 states enrolled in the study from March 2014 to June 2015, and 195 were randomized. Most participants were white (96%) and college educated (73%). Of the randomized participants, 73% had seen a Parkinsons disease specialist within the previous year. CONCLUSIONS Among individuals with Parkinsons disease, national interest in receiving remote specialty care directly into the home is high. Remote enrollment in this care model is feasible but is likely affected by differential access to the Internet.

Feasibility of Virtual Research Visits in Fox Trial Finder

Abstract Background: Fox Trial Finder is an online registry for individuals with and without Parkinson disease (PD) interested in participating in PD research. However, distance or disability could prevent such individuals from participating in traditional, clinic-based research at major centers. Objective: Use videoconferencing to connect participants to specialists to: (1) demonstrate feasibility of virtual research visits within this population (2) collect phenotypic data of the participants, (3) validate self-reported diagnosis, and (4) gauge interest in virtual research visits. Methods: We solicited volunteers throughout the United States through Fox Trial Finder. Interested individuals with PD provided consent, were given web cameras if needed, completed baseline surveys, and downloaded videoconferencing software remotely. Participants had a test connection and assessment appointment which included the Montreal Cognitive Assessment (MoCA), then a virtual research visit with a neurologist who reviewed their history and assessed their PD using a modified Movement Disorders Society Unified Parkinson’s Disease Rating Scale. Neurologists assessed PD diagnosis and symptomatology. Physicians and participants were surveyed about their experience. Results: Of 204 individuals who consented, 166 (81% ) individuals from 39 states completed all visits. The mean age was 62 and mean disease duration was 8.0 years. Mean MoCA score was 26.5, and mean modified MDS-UPDRS motor score was 22.8 (out of a possible 124). Neurologists judged PD as the most likely diagnosis in 97% of cases. Overall satisfaction with the visits was 79% (satisfied or very satisfied) among neurologists and 93% among participants. Conclusions: Through virtual research visits, neurologists engaged, characterized, and validated self-reported diagnosis in individuals with PD over a broad geography. This model may facilitate future research participation.

Virtual visits for Parkinson disease: A multicenter noncontrolled cohort

Objective: Previous small-scale studies have demonstrated the feasibility of providing remote specialty care via virtual visits. We assessed the feasibility and benefits of a one-time consultation between a remote Parkinson Disease (PD) specialist and an individual with PD at home on a larger scale. Methods: We conducted a multicenter noncontrolled cohort of virtual visits administered over videoconferencing between remote PD specialists and individuals with PD in their home. Specialists performed a patient history and a PD-specific physical examination and provided recommendations to patients and their local physicians. The primary outcome measures were feasibility, as measured by the proportion of visits completed as scheduled, and the 6-month change in quality of life, as measured by the Parkinsons Disease Questionnaire 39. Additional outcomes included satisfaction with visits and interest in future virtual visits. Results: A total of 277 participants from 5 states enrolled, 258 participants completed virtual visits with 14 different physicians, and 91% of visits were completed as scheduled. No improvement in quality of life was observed at 6 months (0.4-point improvement; 95% confidence interval −1.5 to 0.6; p = 0.39). Overall satisfaction with virtual visits was high among physicians (94% satisfied or very satisfied) and patients (94% satisfied or very satisfied), and 74% of participants were interested in receiving future care via virtual visits. Conclusions: Providing specialty care remotely into the homes of individuals with PD is feasible, but a one-time visit did not improve quality of life. Satisfaction with the visits was high among physicians and patients, who were interested in receiving such care in the future. Classification of evidence: This study provides Class IV evidence that for patients with PD, remote specialty care is feasible but does not improve quality of life. Clinicaltrials.gov identifier: NCT02144220.

Author response: Burnout, career satisfaction, and well-being among US neurologists in 2016

We thank Bagot et al. for their comments. Burnout, career satisfaction, and well-being in neurologists who practice telemedicine is important to study as this practice model becomes more common.

Author response: Burnout, career satisfaction, and well-being among US neurology residents and fellows in 2016

We agree that factors outside the workplace play a potentially important role in emotional exhaustion and burnout among neurology trainees. We concentrated on workplace drivers of burnout, except for one general question about work–life balance, in order to keep the survey length manageable.1 Burnout is associated with poor quality of life and educational debt.2 Work–home interference can be a cause as well as a result of stress during residency.3 A more in-depth study of work–life balance and stressors outside the workplace among neurology trainees could provide additional insights.