A Comparison of Patients' and Neurologists' Assessments of their Teleneurology Encounter: A Cross-Sectional Analysis.

Background and Objectives: To better understand patients' and neurologists' assessments of their experiences regarding effectiveness of teleneurology encounters. Methods: Following an audio-video telehealth visit, neurologists asked patients to participate in a survey-based research study about the encounter, and then, the neurologists also recorded their own evaluations. Data were analyzed using standard quantitative and qualitative techniques for dichotomous and ordered-category survey responses in this cross-sectional analysis. Results: The study included unique encounters between 187 patients and 11 general neurologists. The mean patient age was 49 ± 17.5 years. Two thirds of the patients (66.8%, 125/187) were female. One third (33.2%; 62) were patients new to the NYU Langone Health neurology practices. The most common patient chief complaints were headache (69/187, 36.9%), focal and generalized numbness or tingling (21, 11.2%), memory difficulty (15, 8%), spine-related symptoms (12, 6.4%), and vertigo (11, 5.9%). Most patients (94.7%, 177/187) reported that the teleneurology encounter satisfied their needs. Patients and their neurologists agreed that the experience was effective in 91% (162/178) of encounters, regardless of whether the visit was for a new or established patient visit. Discussion: More than 90% of new and established patients and their neurologists agreed that teleneurology encounters were effective despite some limitations of the examination, the occasional need for patient assistance, and technical difficulties. Our results provide further evidence to justify and to expand the clinical use of teleneurology.

Neurologists' Evaluations of Experience and Effectiveness of Teleneurology Encounters.

Background and Objectives: To better understand neurologists' assessments of the experiences and effectiveness of teleneurology encounters. Methods: After completing an audio-video telehealth visit with verbally consenting patients, neurologists recorded their evaluations of the encounter. Data were analyzed using standard quantitative and qualitative techniques. Results: The study included unique encounters between 187 patients and 11 neurologists. The mean patient age was 49 ± 17.5 years. Two thirds of patients (66.8%, 125/187) were female. One third of patients (33.2%; 62) were new patients. The most common patient complaints were headache (69/187, 36.9%), focal and generalized numbness or tingling (21, 11.2%), memory difficulty (15, 8%), spine-related symptoms (12, 6.4%), and vertigo (11, 5.9%). Neurologists reported that they completed a virtual examination that provided enough information for medical decision-making in 94.9% of encounters (169/178, 9 missing responses). Fourteen of 25 examination elements important for medical decision-making could be performed sufficiently during virtual encounters. Examination assistance was needed for 16.4% (30/183) of patients, who were, on average, 17.3 years older than those who did not require assistance (62.9 years vs. 45.6 years, p = 0.0002). In 19.1% (34/178) of encounters, neurologists learned clinically relevant information from seeing patients in their homes. Neurologists' assessments of the effectiveness of encounters were not related to the presence (97.2%, 35/36 effective) or absence (95%, 134/141 effective) of technical difficulties (p = 0.5729) in 177 encounters (10 missing responses). Discussion: Neurologists reported that nearly 95% of teleneurology encounters were effective despite limitations of the virtual examination, occasional need for patient assistance, and technical difficulties.

Immune checkpoint inhibition in patients with inactive pre-existing neuromuscular autoimmune diseases.

OBJECTIVES: To evaluate the safety of immune checkpoint inhibitor use in patients with pre-existing neurological autoimmune diseases. METHODS: In this retrospective case-series, we examined exacerbations of underlying disease and the occurrence of immune-related adverse events in 5 patients who had been diagnosed with a neurological autoimmune disease prior to receiving immune checkpoint inhibitor therapy for advanced malignancy. RESULTS: Two patients had a prior diagnosis of myasthenia gravis, two had Guillain-Barré syndrome, and one had chronic idiopathic demyelinating polyneuropathy. Only one patient experienced a flare of neurological autoimmune disease. Four of the five patients experienced immune-related adverse events unrelated to their neurological disease. CONCLUSIONS: In this case-series, exacerbations of neurological autoimmune disease were less common and less severe than expected. Further research is needed to determine which individuals are at greatest risk of neurological autoimmune disease complication while receiving immune checkpoint inhibitor therapy.

Celiac disease and risk of myasthenia gravis - nationwide population-based study.

BACKGROUND: Case reports suggest there may be an association between celiac disease (CD) and myasthenia gravis (MG). METHODS: We identified 29,086 individuals with CD in Sweden from 1969 to 2008. We compared these individuals with 144,480 matched controls. Hazard ratios (HRs) for future MG (identified through ICD codes) were estimated using Cox regression. RESULTS: During 326,376 person-years of follow-up in CD patients, there were 7 MG cases (21/million person-years) compared to 22 MG cases in controls during 1,642,273 years of follow-up (14/million person-years) corresponding to a HR of 1.48 (95% CI = 0.64-3.41). HRs did not differ when stratifying for age, sex or calendar period. HRs were highest in the first year after follow-up, though insignificant. Individuals with CD were at no increased risk of MG more than 5 years after CD diagnosis (HR = 0.70; 95% CI = 0.16-3.09). CONCLUSION: This study found no increased risk of MG in patients with CD.

Rapid Number Naming and Quantitative Eye Movements May Reflect Contact Sport Exposure in a Collegiate Ice Hockey Cohort.

BACKGROUND: The King-Devick (K-D) test of rapid number naming is a reliable visual performance measure that is a sensitive sideline indicator of concussion when time scores worsen (lengthen) from preseason baseline. Within cohorts of youth athletes <18 years old, baseline K-D times become faster with increasing age. We determined the relation of rapid number-naming time scores on the K-D test to electronic measurements of saccade performance during preseason baseline assessments in a collegiate ice hockey team cohort. Within this group of young adult athletes, we also sought to examine the potential role for player age in determining baseline scores. METHODS: Athletes from a collegiate ice hockey team received preseason baseline testing as part of an ongoing study of rapid rink-side performance measures for concussion. These included the K-D test (spiral-bound cards and tablet computer versions). Participants also performed a laboratory-based version of the K-D test with simultaneous infrared-based video-oculographic recordings using an EyeLink 1000+. This allowed measurement of the temporal and spatial characteristics of eye movements, including saccadic velocity, duration, and intersaccadic interval (ISI). RESULTS: Among 13 male athletes, aged 18-23 years (mean 20.5 ± 1.6 years), prolongation of the ISI (a combined measure of saccade latency and fixation duration) was the measure most associated with slower baseline time scores for the EyeLink-paired K-D (mean 38.2 ± 6.2 seconds, r = 0.88 [95% CI 0.63-0.96], P = 0.0001), the K-D spiral-bound cards (36.6 ± 5.9 seconds, r = 0.60 [95% CI 0.08-0.87], P = 0.03), and K-D computerized tablet version (39.1 ± 5.4 seconds, r = 0.79 [95% CI 0.42-0.93], P = 0.001). In this cohort, older age was a predictor of longer (worse) K-D baseline time performance (age vs EyeLink-paired K-D: r = 0.70 [95% CI 0.24-0.90], P = 0.008; age vs K-D spiral-bound cards: r = 0.57 [95% CI 0.03-0.85], P = 0.04; age vs K-D tablet version: r = 0.59 [95% CI 0.06-0.86], P = 0.03) as well as prolonged ISI (r = 0.62 [95% CI 0.11-0.87], P = 0.02). Slower baseline K-D times were not associated with greater numbers of reported prior concussions. CONCLUSIONS: Rapid number-naming performance using the K-D at preseason baseline in this small cohort of collegiate ice hockey players is best correlated with ISI among eye movement-recording measures. Baseline K-D scores notably worsened with increasing age, but not with numbers of prior concussions in this small cohort. While these findings require further investigation by larger studies of contact and noncontact sports athletes, they suggest that duration of contact sports exposure may influence preseason test performance.

Risk of Neuropathy Among 28,232 Patients With Biopsy-Verified Celiac Disease.

IMPORTANCE: Earlier research on celiac disease (CD) and neuropathy has been hampered by the use of inpatient data, low study power, and lack of neuropathic characteristics. OBJECTIVE: To examine the relative risk and absolute risk of developing neuropathy in a nationwide population-based sample of patients with biopsy-verified CD. DESIGN, SETTING, AND PARTICIPANTS: Between October 27, 2006, and February 12, 2008, we collected data on small-intestinal biopsies performed at Sweden's 28 pathology departments between June 16, 1969, and February 4, 2008. We compared the risk of neuropathy in 28,232 patients with CD (villous atrophy, Marsh 3) with that of 139,473 age- and sex-matched controls. Cox proportional hazards regression estimated hazard ratios (HRs) and 95% CIs for neuropathy defined according to relevant International Classification of Diseases codes in the Swedish National Patient Register (consisting of both inpatient and outpatient data). MAIN OUTCOMES AND MEASURES: Neuropathy in patients with biopsy-verified CD. RESULTS: Celiac disease was associated with a 2.5-fold increased risk of later neuropathy (95% CI, 2.1-3.0; P < .001). We also found an increased risk (with results reported as HRs [95% CIs]) of chronic inflammatory demyelinating neuropathy (2.8; 1.6-5.1; P = .001), autonomic neuropathy (4.2; 1.4-12.3; P = .009), and mononeuritis multiplex (7.6; 1.8-32.4; P = .006), but no association between CD and acute inflammatory demyelinating polyneuropathy (0.8; 0.3-2.1; P = .68). CONCLUSIONS AND RELEVANCE: We found an increased risk of neuropathy in patients with CD. This statistically significant association in a population-based sample suggests that CD screening should be completed in patients with neuropathy.

Bortezomib-associated demyelinating neuropathy--clinical and pathologic features.

OBJECTIVES: Bortezomib is a proteasome inhibitor that is frequently used for multiple myeloma and lymphoma. A sensory predominant axonal neuropathy is associated with bortezomib treatment but a demyelinating neuropathy is also described primarily based on electrodiagnostic findings. We report a series of patients treated with bortezomib who developed peripheral neuropathy and were found to have demyelinating features on electrodiagnostic testing. METHODS: Four patients who developed a bortezomib-induced peripheral neuropathy underwent electrophysiological testing, and 1 patient had a nerve biopsy. RESULTS: The four patients with bortezomib-induced peripheral neuropathy had demyelinating features on their electrophysiological testing. The nerve biopsy performed in 1 patient demonstrated a demyelinating component in a background of axonal degeneration. CONCLUSIONS: Although most toxic neuropathies are symmetrical axonal neuropathies, bortezomib is part of a small list of agents that may cause a demyelinating polyneuropathy and axonal degeneration. These findings have been confirmed by nerve biopsy.

Essential tremor is associated with dementia: prospective population-based study in New York.

BACKGROUND: Mild cognitive deficits, mainly in frontal-executive function and memory, have been reported in patients with essential tremor (ET). Furthermore, an association between ET and dementia has been reported in a single population-based study in Spain. This has not been confirmed elsewhere. OBJECTIVE: To determine whether baseline ET is associated with prevalent and incident dementia in an ethnically diverse, community-based sample of elders. METHODS: Community-dwelling elders in northern Manhattan were enrolled in a prospective cohort study. Baseline ET diagnoses were assigned from handwriting samples. Dementia was diagnosed at baseline and follow-up using DSM-III-R criteria. RESULTS: In cross-sectional analyses, 31/124 (25.0%) ET cases had prevalent dementia vs 198/2,161 (9.2%) controls (odds ratio [OR](unadjusted) = 3.31, 95% confidence interval [CI] = 2.15-5.09, p < 0.001; OR(adjusted) = 1.84, 95% CI = 1.13-2.98, p = 0.01). In prospective analyses, 17/93 (18.3%) ET cases vs 171/1,963 (8.7%) controls developed incident dementia (hazard ratio [HR](unadjusted) = 2.78, 95% CI = 1.69-4.57, p < 0.001; HR(adjusted) = 1.64, 95% CI = 0.99-2.72, p = 0.055). CONCLUSIONS: In a second population-based study of elders, essential tremor (ET) was associated with both increased odds of prevalent dementia and increased risk of incident dementia. Presence of dementia, therefore, appeared to be greater than that expected for age (i.e., a disease-associated feature). Rather than attributing cognitive complaints in patients with ET to old age, assessment and possible treatment of dementia should be routinely incorporated into the treatment plan.

Prevalence of essential tremor in a multiethnic, community-based study in northern Manhattan, New York, N.Y.

BACKGROUND: Our aims were to: (1) estimate the prevalence of essential tremor (ET) in a community-based study in northern Manhattan, New York, N.Y., USA; (2) compare prevalence across ethnic groups, and (3) provide prevalence estimates for the oldest old. METHODS: This study did not rely on a screening questionnaire. Rather, as part of an in-person neurological evaluation, each participant produced several handwriting samples, from which ET diagnoses were assigned. RESULTS: There were 1,965 participants (76.7 +/- 6.9 years, range = 66-102 years); 108 had ET [5.5%, 95% confidence interval (CI) = 4.5-6.5%]. Odds of ET were robustly associated with Hispanic ethnicity versus white ethnicity [odds ratio (OR) = 2.19, 95% CI = 1.03-4.64, p = 0.04] and age (OR = 1.14, 95% CI = 1.03-1.26, p = 0.01), i.e. with every 1 year advance in age, the odds of ET increased by 14%. Prevalence reached 21.7% among the oldest old (age > or = 95 years). CONCLUSIONS: This study reports a significant ethnic difference in the prevalence of ET. The prevalence of ET was high overall (5.5%) and rose markedly with age so that in the oldest old, more than 1 in 5 individuals had this disease.