Self-scheduling in a Large Multispecialty and Multisite Clinic: A Retrospective, Longitudinal Examination of Multiple Self-Scheduled Visit Types.

Background: Self-scheduling of medical visits is becoming available at many medical institutions. We aimed to examine the self-scheduled visit counts and rate of growth of self-scheduled visits in a multispecialty practice. Methods: For 85 weeks extending from January 1, 2022 through August 24, 2023, we examined self-scheduled visit counts for over 1500 self-scheduled visit types. We compared completed self-scheduled visit counts to all scheduled completed visit counts for the same visit types. We collected counts of the most frequently self-scheduled visit types for each week and examined the change over time. We also determined the proportion that each visit type was self-scheduled. Results: There were 20,769 699 completed visits during the course of the study that met the criteria for inclusion. Self-scheduled visits accounted for 4.0% of all completed visits (838 592/20,769 699). Over the 85-week span, self-scheduled visits rose from 3.0% to 5.3% of the total. There were 1887 unique visit types that were associated with completed visits. There were just 6 appointment visit types of the total 1887 self-scheduled visit types that accounted for 50.7% of the total 838 592 self-scheduled visits. Those 6 visit types were a lab blood test visit (19.5%, 163 K visits), two Family Medicine office visit types (13.0%, 109 K visits), a screening mammogram visit type (6.6%, 55 K visits), a scheduled express care visit type (6%, 50 K visits) and a COVID immunization visit type (5.7%, 48 K visits). Twenty-one visit types that were self-scheduled accounted for 75% of the total self-scheduled visits. Four seasonal visits, accounting for 10.6% of the total self-scheduled visits, were responsible for almost all the non-linear change in self-scheduling. Conclusion: Self-scheduling accounted for a small but growing percent of all outpatient scheduled visits in a multispecialty, multisite practice. A wide range of visit types can be successfully self-scheduled.

Self-scheduling Medical Visits in a Multispecialty, Multisite Medical Practice: Complexity, Challenges, and Successes.

Background: Self-scheduling of medical visits is becoming more common but the complexity of applying multiple requirements for self-scheduling has hampered implementation. Mayo Clinic implemented self-scheduling in 2019 and has been increasing its portfolio of self-schedulable visits since then. Our aim was to show measures quantifying the complexity associated with medical visit scheduling and to describe how opportunities and challenges of scheduling complexity apply in self-scheduling. Methods: We examined scheduled visits from January 1, 2022, through August 24, 2023. For seven visit categories, we counted all unique visit types that were scheduled, for both staff-scheduled and self-scheduled. We examined counts of self-scheduled visit types to identify those with highest uptake during the study period. Results: There were 9555 unique visit types associated with 20.8 M (million) completed visits. Self-scheduled visit types accounted for 4.0% (838,592/20,769,699) of the completed total visits. Of seven visit categories, self-scheduled established patient visits, testing visits, and procedure visits accounted for 93.5% (784,375/838,592) of all self-scheduled visits. Established patient visits in primary care (10 visit types) accounted for 273,007 (32.6%) of all self-scheduled visits. Testing visits (blood and urine testing, 2 visit types) accounted for 183,870 (21.9%) of all self-scheduled visits. Procedure visits for screening mammograms, bone mineral density, and immunizations (8 visit types) accounted for 147,358 (17.6%) of all self-scheduled visits. Conclusion: Large numbers of unique visit types comprise a major challenge for self-scheduling. Some visit types are more suitable for self-scheduling. Guideline-based procedure visits such as screening mammograms, bone mineral density exams, and immunizations are examples of visits that have high volumes and can be standardized for self-scheduling. Established patient visits and laboratory testing visits also can be standardized for self-scheduling. Despite the successes, there remain thousands of specific visit types that may need some staff-scheduler intervention to properly schedule.

Online Self-Triage of Ear or Hearing Concerns in a Patient Portal: Comparison of Subsequent Diagnoses and Hospitalizations to National Emergency Department and National Ambulatory Ear or Hearing Visits.

Background: Although online self-triage is easily accessible, little is known about the patients who use self-triage or their subsequent diagnoses. We compared ear/hearing self-triage subsequent diagnoses to ear/hearing visit diagnoses in emergency departments (ED) and ambulatory clinics across the United States. Methods: We compared International Classification of Diseases version 10 (ICD10) coded diagnoses following online self-triage for ear/hearing concerns with those from national ED and ambulatory clinic samples. We used data from the Centers for Disease Control (CDC) National Hospital Ambulatory Medical Care Survey (NHAMCS) and National Ambulatory Medical Care Survey (NAMCS) for comparison. Using matched ear/hearing diagnostic categories for those aged 1 and over, we compared self-triage diagnosis frequencies with national ED and ambulatory diagnosis frequencies. Results: Following ear/hearing self-triage, there were 1092 subsequent office visits with a primary diagnosis code. For five frequently diagnosed ear/hearing conditions (i.e., suppurative and nonsuppurative otitis media [OM], otalgia, otitis externa, and cerumen impaction), there was a strong correlation between diagnosis counts made following self-triage and estimated counts of national ED visit diagnoses (r = 0.94; CI 95% [0.37 to 0.99]; p = .016, adjusted r2 = 0.85). Seven diagnoses were available to compare with the national ambulatory sample; correlation was r = 0.79; CI 95% [0.08 to 0.97]; p = .037, adjusted r2 = 0.54. For ages 1 and over, estimated hospital admissions from the national ED visits for ear/hearing were 0.76%, CI 95% [0.28-2.1%]; estimated total national ear/hearing ED visits were 7.5 million (for 4 years, 2016 through 2019). Conclusion: The strong correlation of ear-related self-triage diagnoses with national ED diagnoses and the low hospitalization risk for these diagnoses suggests that there is an opportunity for self-triage of ear/hearing concerns to decrease ED visits for these symptoms.

Self-Triage Use, Subsequent Healthcare Utilization, and Diagnoses: A Retrospective Study of Process and Clinical Outcomes Following Self-Triage and Self-Scheduling for Ear or Hearing Symptoms.

Background: Self-triage is becoming more widespread, but little is known about the people who are using online self-triage tools and their outcomes. For self-triage researchers, there are significant barriers to capturing subsequent healthcare outcomes. Our integrated healthcare system was able to capture subsequent healthcare utilization of individuals who used self-triage integrated with self-scheduling of provider visits. Methods: We retrospectively examined healthcare utilization and diagnoses after patients had used self-triage and self-scheduling for ear or hearing symptoms. Outcomes and counts of office visits, telemedicine interactions, emergency department visits, and hospitalizations were captured. Diagnosis codes associated with subsequent provider visits were dichotomously categorized as being associated with ear or hearing concerns or not. Nonvisit care encounters of patient-initiated messages, nurse triage calls, and clinical communications were also captured. Results: For 2168 self-triage uses, we were able to capture subsequent healthcare encounters within 7 days of the self-triage for 80.5% (1745/2168). In subsequent 1092 office visits with diagnoses, 83.1% (891/1092) of the uses were associated with relevant ear, nose and throat diagnoses. Only 0.24% (4/1662) of patients with captured outcomes were associated with a hospitalization within 7 days. Self-triage resulted in a self-scheduled office visit in 7.2% (126/1745). Office visits resulting from a self-scheduled visit had significantly fewer combined non-visit care encounters per office visit (fewer combined nurse triage calls, patient messages, and clinical communication messages) than office visits that were not self-scheduled (-0.51; 95% CI, -0.72 to -0.29; P < .0001). Conclusion: In an appropriate healthcare setting, self-triage outcomes can be captured in a high percentage of uses to examine for safety, patient adherence to recommendations, and efficiency of self-triage. With the ear or hearing self-triage, most uses had subsequent visit diagnoses relevant to ear or hearing, so most patients appeared to be selecting the appropriate self-triage pathway for their symptoms.

Self-Scheduling Process Efficiency and Utilization of Online Self-Scheduling of Lab Tests: A Retrospective Analysis of Self-Scheduled Appointments for COVID Testing.

Introduction: The COVID 19 pandemic increased the need for rapid and accurate diagnostic testing for COVID. When testing became available, a systems response was needed to efficiently accommodate the high-volume flow of patients who needed testing. Self-scheduling of COVID testing was developed to help patients safely and efficiently schedule their COVID testing online or with a mobile app. Methods: We captured the counts of COVID test appointments, time patients spent in scheduling COVID test appointments, appointment lead times, and no-shows for COVID test appointments. For 17 months of self-scheduling, we retrospectively compared self-scheduling with the concurrent staff scheduling of COVID tests. Results: From November 2020 through March 2022 there were 619 104 scheduled appointments for COVID testing with 22% (136 252) being self-scheduled. For asymptomatic self-scheduled COVID tests, accounting for 10.3% (63 605/619 104) of total COVID tests scheduled, median time to self-schedule was 3.1 min, interquartile range (IQR) [2.4,4.7]. For symptomatic self-schedulers accounting for 11.7% (72 647/619 104) of total COVID tests scheduled, the median time to self-triage and self-schedule was 5.8 min, IQR[4.3,8.9]. Self-scheduled COVID appointments increased to 44% (42 387/97 086) of the total COVID appointments during the peak month of January 2022. Median appointment lead time for symptomatic self-scheduled COVID test appointments was 6.6 h compared to 2.9 h (P < .0001) for symptomatic staff scheduled appointments. However, adjusting for the 24% (32 194/135 252) that self-scheduled during hours when testing was unavailable, the median appointment lead time for symptomatic self-scheduled patients dropped to 3.6 h. No-shows were 2.5% for self-scheduled appointments compared to 3.0% no-shows that were staff scheduled (odds ratio 0.83, P < .0001). Conclusion: COVID testing was self-scheduled for a large percent of scheduled COVID tests, taking patients only a few minutes to complete. Self-scheduling use increased over time, associated with a decreasing use of staff scheduled appointments and lower no-shows.

Neurophysiology during peripheral nerve surgery.

Electrophysiological monitoring of the peripheral nervous system during a variety of surgeries provides useful information that supplements and complements preoperative assessment. Monitoring improves localization and understanding of the underlying pathophysiology of peripheral nerve lesions leading to more rational treatment decisions and improved outcomes. Monitoring is accomplished by adaptation of routine electrodiagnostic techniques (i.e., nerve conduction studies, evoked potentials, and electromyography) with special attention to technical factors including electrical and movement artifact. These techniques have been successfully applied during surgery for entrapment neuropathies, traumatic nerve injury and repair, peripheral nerve tumors, and adjacent structure procedures that risk peripheral nerve injury. A clear understanding of the anatomy and neurophysiology is necessary, as is understanding and performing the difficult technical aspects of these studies to provide accurate information to enhance patient outcome and recovery. As in any intraoperative neurophysiologic monitoring (IONM) setting, constant and accurate communication between the IONM team, surgeon, and anesthesia team is critically important to meet these goals.

The Effect of Automated Mammogram Orders Paired With Electronic Invitations to Self-schedule on Mammogram Scheduling Outcomes: Observational Cohort Comparison.

BACKGROUND: Screening mammography is recommended for the early detection of breast cancer. The processes for ordering screening mammography often rely on a health care provider order and a scheduler to arrange the time and location of breast imaging. Self-scheduling after automated ordering of screening mammograms may offer a more efficient and convenient way to schedule screening mammograms. OBJECTIVE: The aim of this study was to determine the use, outcomes, and efficiency of an automated mammogram ordering and invitation process paired with self-scheduling. METHODS: We examined appointment data from 12 months of scheduled mammogram appointments, starting in September 2019 when a web and mobile app self-scheduling process for screening mammograms was made available for the Mayo Clinic primary care practice. Patients registered to the Mayo Clinic Patient Online Services could view the schedules and book their mammogram appointment via the web or a mobile app. Self-scheduling required no telephone calls or staff appointment schedulers. We examined uptake (count and percentage of patients utilizing self-scheduling), number of appointment actions taken by self-schedulers and by those using staff schedulers, no-show outcomes, scheduling efficiency, and weekend and after-hours use of self-scheduling. RESULTS: For patients who were registered to patient online services and had screening mammogram appointment activity, 15.3% (14,387/93,901) used the web or mobile app to do either some mammogram self-scheduling or self-cancelling appointment actions. Approximately 24.4% (3285/13,454) of self-scheduling occurred after normal business hours/on weekends. Approximately 9.3% (8736/93,901) of the patients used self-scheduling/cancelling exclusively. For self-scheduled mammograms, there were 5.7% (536/9433) no-shows compared to 4.6% (3590/77,531) no-shows in staff-scheduled mammograms (unadjusted odds ratio 1.24, 95% CI 1.13-1.36; P<.001). The odds ratio of no-shows for self-scheduled mammograms to staff-scheduled mammograms decreased to 1.12 (95% CI 1.02-1.23; P=.02) when adjusted for age, race, and ethnicity. On average, since there were only 0.197 staff-scheduler actions for each finalized self-scheduled appointment, staff schedulers were rarely used to redo or "clean up" self-scheduled appointments. Exclusively self-scheduled appointments were significantly more efficient than staff-scheduled appointments. Self-schedulers experienced a single appointment step process (one and done) for 93.5% (7553/8079) of their finalized appointments; only 74.5% (52,804/70,839) of staff-scheduled finalized appointments had a similar one-step appointment process (P<.001). For staff-scheduled appointments, 25.5% (18,035/70,839) of the finalized appointments took multiple appointment steps. For finalized appointments that were exclusively self-scheduled, only 6.5% (526/8079) took multiple appointment steps. The staff-scheduled to self-scheduled odds ratio of taking multiple steps for a finalized screening mammogram appointment was 4.9 (95% CI 4.48-5.37; P<.001). CONCLUSIONS: Screening mammograms can be efficiently self-scheduled but may be associated with a slight increase in no-shows. Self-scheduling can decrease staff scheduler work and can be convenient for patients who want to manage their appointment scheduling activity after business hours or on weekends.

Mapping of critical events in disease progression through binary classification: Application to amyotrophic lateral sclerosis.

BACKGROUND: The progression of many degenerative diseases is tracked periodically using scales evaluating functionality in daily activities. Although estimating the timing of critical events (i.e., disease tollgates) during degenerative disease progression is desirable, the necessary data may not be readily available in scale records. Further, analysis of disease progression poses data challenges, such as censoring and misclassification errors, which need to be addressed to provide meaningful research findings and inform patients. METHODS: We developed a novel binary classification approach to map scale scores into disease tollgates to describe disease progression leveraging standard/modified Kaplan-Meier analyses. The approach is demonstrated by estimating progression pathways in amyotrophic lateral sclerosis (ALS). Tollgate-based ALS Staging System (TASS) specifies the critical events (i.e., tollgates) in ALS progression. We first developed a binary classification predicting whether each TASS tollgate was passed given the itemized ALSFRS-R scores using 514 ALS patients' data from Mayo Clinic-Rochester. Then, we utilized the binary classification to translate/map the ALSFRS-R data of 3,264 patients from the PRO-ACT database into TASS. We derived the time trajectories of ALS progression through tollgates from the augmented PRO-ACT data using Kaplan-Meier analyses. The effects of misclassification errors, condition-dependent dropouts, and censored data in trajectory estimations were evaluated with Interval Censored Kaplan Meier Analysis and Multistate Model for Panel Data. RESULTS: The approach using Mayo Clinic data accurately estimated tollgate-passed states of patients given their itemized ALSFRS-R scores (AUCs > 0.90). The tollgate time trajectories derived from the augmented PRO-ACT dataset provide valuable insights; we predicted that the majority of the ALS patients would have modified arm function (67%) and require assistive devices for walking (53%) by the second year after ALS onset. By the third year, most (74%) ALS patients would occasionally use a wheelchair, while 48% of the ALS patients would be wheelchair-dependent by the fourth year. Assistive speech devices and feeding tubes were needed in 49% and 30% of the patients by the third year after ALS onset, respectively. The onset body region alters some tollgate passage time estimations by 1-2 years. CONCLUSIONS: The estimated tollgate-based time trajectories inform patients and clinicians about prospective assistive device needs and life changes. More research is needed to personalize these estimations according to prognostic factors. Further, the approach can be leveraged in the progression of other diseases.

Impact of Web-Based Self-Scheduling on Finalization of Well-Child Appointments in a Primary Care Setting: Retrospective Comparison Study.

BACKGROUND: Web-booking of flights, hotels, and sports events has become commonplace in the travel and entertainment industry, but self-scheduling of health care appointments on the web is not yet widely used. An electronic health record that integrates appointment scheduling and patient web-based access to medical records creates an opportunity for patient self-scheduling. The Mayo Clinic developed and implemented a feature in its Patient Online Services (POS) web and mobile platform that allows software-managed self-scheduling of well-child visits. OBJECTIVE: This study aims to examine the use of a new self-scheduling appointment feature within POS in both web and mobile formats and determine the use characteristics, outcomes, and efficiency of self-scheduling compared with staff scheduling. METHODS: Within a primary care setting, we collected 13 months of all appointment activity for the well-child visit for children aged 2-12 years. As these specific appointment types are for minors, self-scheduling is performed by parents or other proxies. We compared the appointment actions of scheduling and cancelling for both self-scheduled and staff-scheduled appointments. The frequency in which patients were using self-scheduling outside of normal business hours was quantified, and we compared no-show outcomes of finalized appointments. RESULTS: Of the 1099 patients who performed any self-scheduling actions, 73.1% (803/1099) exclusively used self-scheduling and self-cancelling software. For those with access to self-scheduling (patients registered with the Mayo Clinic POS), 4.92% (1201/24,417) of all well-child appointment-scheduling actions were self-scheduled. Staff scheduling required more than a single appointment step (eg, schedule, cancel, reschedule) in 28.32% (3729/13,168) compared with only 6.93% (53/765) of self-scheduled appointments (P<.001). Self-scheduling appointment actions took place outside of regular business hours 29.5% (354/1201) of the time. No-shows accounted for 3.07% (28/912) of the self-scheduled finalized appointments compared with 4.12% (693/16,828) of staff-scheduled appointments, which is a nonsignificant difference (P=.12). Staff-scheduled finalized appointments (that allowed for scheduling appointments for more than 12 weeks in the future) revealed a potential demand of 11.15% (1876/16,828) for appointments with longer lead times. CONCLUSIONS: Self-scheduling can generate a significant number of finalized appointments, decreasing the need for staff scheduler time. We found that 29.5% (354/1201) of the self-scheduling activity took place outside of the usual staff scheduler hours, adding convenience value to the scheduling process. For exclusive self-schedulers, 93.1% (712/765) finalized the appointment in a single step. The no-show rates were not adversely affected by the self-scheduling.

Improving accuracy of myasthenia gravis autoantibody testing by reflex algorithm.

OBJECTIVE: To improve myasthenia gravis (MG) autoantibody testing. METHODS: MG serologic tests with confirmatory or refuting clinical-electrodiagnostic (EDX) testing and cancer evaluations were reviewed over 4 years (2012-2015). All patients had acetylcholine receptor-binding (AChR-Bi), modulating (AChR-Mo), and striational (STR) autoantibody testing, and negatives reflexed to muscle-specific kinase (MuSK). Thymoma and cancer occurrences were correlated with STR and reflexed glutamic acid decarboxylase 65 (GAD65), ganglionic acetylcholine receptor (α3), collapsin response mediating protein-5, and voltage-gated potassium channel complex autoantibodies. RESULTS: Of 433 samples tested, 133 (31%) met clinical-EDX criteria for MG. Best sensitivity (90%) occurred at AChR-Bi >0.02 nmol/L, leaving 14 negative (6 ocular MG, 7 generalized MG, 1 MuSK MG) with specificity 90% (31 false-positives). Using AChR-Mo antibodies (>20% loss), specificity was better (92%, 24 false-positives), but sensitivity dropped (85%). Specificity improved (95%) by testing AChR-Mo when AChR-Bi are positive, resulting in 45% reduction of false-positives (31-17), maintaining AChR-Bi 90% sensitivity. Cutoff values recommended by area under the curve analysis did not outperform this approach. AChR-Bi and AChR-Mo values were significantly higher in true-positives. CT evaluations in 121 MG samples revealed 16 thymomas. Historical or subsequent cancers occurred in 22. STR and reflexed autoantibodies were not more common in MG with thymoma or other cancers. Full-body CT (n = 34) was performed in those with STR and reflex autoantibody positivity, but without additional cancers found. CONCLUSION: Accuracy of MG serologic testing is improved by reflexing AChR-Bi-positive cases to AChR-Mo. STR and other reflexed cancer evaluation autoantibodies did not provide value beyond standard CT chest imaging at the time of MG diagnosis. Diagnostic certainty is informed by AChR-Bi and AChR-Mo with higher values increasing specificity.

Nerve Pathology Distinguishes Focal Motor Chronic Inflammatory Demyelinating Polyradiculoneuropathy From Multifocal Motor Neuropathy.

OBJECTIVES: The objective of the study is to distinguish the mechanisms of disease for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN), which we believe to be fundamentally different. However, distinguishing the mechanisms is more difficult when the presentation of CIDP is motor-predominant, focal, or asymmetric. METHODS: We describe 3 focal, motor-predominant, representative cases that could be interpreted on clinical and/or electrophysiological grounds as either MMN or focal CIDP, and present pathological findings. RESULTS: We highlight pathological differences in these cases, and provide an argument that CIDP and MMN are distinct entities with different pathophysiological mechanisms-chronic demyelination for CIDP, and an immune-mediated attack on paranodal motor axons for MMN. CONCLUSIONS: Based on clinical evaluation, electrophysiology, and nerve biopsy pathology, we can divide the conditions into inflammatory demyelinating neuropathy (focal CIDP) versus chronic axonal neuropathy (MMN). The divergent pathological findings provide further evidence that CIDP and MMN are fundamentally different disorders.

Tollgate-based progression pathways of ALS patients.

OBJECTIVE: To capture ALS progression in arm, leg, speech, swallowing, and breathing segments using a disease-specific staging system, namely tollgate-based ALS staging system (TASS), where tollgates refer to a set of critical clinical events including having slight weakness in arms, needing a wheelchair, needing a feeding tube, etc. METHODS: We compiled a longitudinal dataset from medical records including free-text clinical notes of 514 ALS patients from Mayo Clinic, Rochester-MN. We derived tollgate-based progression pathways of patients up to a 1-year period starting from the first clinic visit. We conducted Kaplan-Meier analyses to estimate the probability of passing each tollgate over time for each functional segment. RESULTS: At their first clinic visit, 93%, 77%, and 60% of patients displayed some level of limb, bulbar, and breathing weakness, respectively. The proportion of patients at milder tollgate levels (tollgate level < 2) was smaller for arm and leg segments (38% and 46%, respectively) compared to others (> 65%). Patients showed non-uniform TASS pathways, i.e., the likelihood of passing a tollgate differed based on the affected segments at the initial visit. For instance, stratified by impaired segments at the initial visit, patients with limb and breathing impairment were more likely (62%) to use bi-level positive airway pressure device in a year compared to those with bulbar and breathing impairment (26%). CONCLUSION: Using TASS, clinicians can inform ALS patients about their individualized likelihood of having critical disabilities and assistive-device needs (e.g., being dependent on wheelchair/ventilation, needing walker/wheelchair or communication devices), and help them better prepare for future.

Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis.

INTRODUCTION: A randomized trial demonstrated benefit from thymectomy in nonthymomatous acetylcholine receptor (AChR)-antibody positive myasthenia gravis (MG). Uncontrolled observational and histologic studies suggest thymectomy may not be efficacious in anti-muscle-specific kinase (MuSK)-MG. METHODS: The therapeutic impact of thymectomy was evaluated from data collected for a multicenter, retrospective blinded review of rituximab in MuSK-MG. RESULTS: Baseline characteristics were similar between thymectomy (n = 26) and nonthymectomy (n = 29) groups, including treatment with rituximab (42% vs. 45%). At last visit, 35% of thymectomy subjects reached the primary endpoint, a Myasthenia Gravis Foundation of America (MGFA) post-intervention status (PIS) score of minimal manifestations (MM) or better, compared with 55% of controls (P = 0.17). After controlling for age at onset of MG, rituximab, prednisone, and intravenous immunoglobulin/plasma exchange treatment, thymectomy was not associated with greater likelihood of favorable clinical outcome (odds ratio = 0.43, 95% confidence interval 0.12-1.53, P = 0.19). DISCUSSION: Thymectomy was not associated with additional clinical improvement in this multicenter cohort of MuSK-MG patients. Muscle Nerve 59:404-410, 2019.

Recurrent Brachial Neuritis Attacks in Presentation of B-Cell Lymphoma.

We describe a 51-year-old woman who over 5 years had 9 painful monophasic attacks affecting the brachial plexus before a fascicular plexus biopsy diagnosed large B-cell lymphoma. The initial attacks were responsive to steroids with clinical resolution. At last attack, magnetic resonance imaging showed multifocal T2 hyperintensities and nodular gadolinium enhancement in the right brachial plexus not seen previously. Also seen were similar changes in the thoracic spinal cord, basal ganglia, cerebellum, and brainstem. Positron emission tomography revealed marked hypermetabolic activity of the plexus facilitating targeted fascicular brachial plexus biopsy, making the pathological diagnosis. Neurolymphomatosis affecting the peripheral nervous system typically presents with insidious painful progressive infiltration of nerves, roots, or plexi. Recurrent idiopathic brachial neuritis attacks (ie, Parsonage-Turner syndrome) in contrast most commonly are seen in persons with a family history and a discoverable genetic cause by SEPT9 mutations, which tested negative in this patient. This case illustrates how neurolymphomatosis, which represents a malignant transformation of B cells within peripheral nerves, can sometimes present with paraneoplastic immune-responsive neuritis mimicking Parsonage-Turner syndrome. Recurrence, an immune-refractory course or insidious progressive involvement of the nervous system, should raise suspicion of neurolymphomatosis.

Case of Early Amyotrophic Lateral Sclerosis With Prominent Magnetic Resonance Imaging Plexus Abnormalities.

Amyotrophic lateral sclerosis is a neurodegenerative disorder, which is characterized by progressive upper and lower motor neuron signs and symptoms, resulting in progressive muscle weakness. There are very rare reports of magnetic resonance imaging (MRI) abnormalities within the nerve roots or plexus reported in amyotrophic lateral sclerosis. Here, we report a patient who presented with progressive left arm weakness and was found to have bilateral MRI plexus abnormalities. This report illustrates that MRI plexus abnormalities can be seen in amyotrophic lateral sclerosis, possibly before symptoms, and may have clinical value in the diagnostic process.

Rituximab as treatment for anti-MuSK myasthenia gravis: Multicenter blinded prospective review.

OBJECTIVE: To evaluate the efficacy of rituximab in treatment of anti-muscle-specific kinase (MuSK) myasthenia gravis (MG). METHODS: This was a multicenter, blinded, prospective review, comparing anti-MuSK-positive patients with MG treated with rituximab to those not treated with rituximab. The primary clinical endpoint was the Myasthenia Gravis Status and Treatment Intensity (MGSTI), a novel outcome that combines the Myasthenia Gravis Foundation of America (MGFA) postintervention status (PIS) and the number and dosages of other immunosuppressant therapies used. A priori, an MGSTI of level ≤2 was used to define a favorable outcome. Secondary outcomes included modified MGFA PIS of minimal manifestations or better, mean/median prednisone dose, and mean/median doses of other immunosuppressant drugs. RESULTS: Seventy-seven of 119 patients with anti-MuSK MG evaluated between January 1, 2005, and January 1, 2015, at 10 neuromuscular centers were selected for analysis after review of limited clinical data by a blinded expert panel. An additional 22 patients were excluded due to insufficient follow-up. Baseline characteristics were similar between the rituximab-treated patients (n = 24) and the controls (n = 31). Median follow-up duration was >3.5 years. At last visit, 58% (14/24) of rituximab-treated patients reached the primary outcome compared to 16% (5/31) of controls (p = 0.002). Number needed to treat for the primary outcome is 2.4. At last visit, 29% of rituximab-treated patients were taking prednisone (mean dose 4.5 mg/day) compared to 74% of controls (mean dose 13 mg/day) (p = 0.001 and p = 0.005). CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with anti-MuSK MG, rituximab increased the probability of a favorable outcome.

Expanding the spectrum of subacute diencephalic angioencephalopathy.

We present a patient with subacute diencephalic angioencephalopathy (SDAE), a poorly understood syndrome of progressive confusion, disorientation, and dementia that rapidly deteriorates to severe encephalopathy and death. This is the seventh and most thoroughly investigated report of this exceedingly rare diagnosis to date, and we present the first evidence of a potential sentinel syndrome that may allow for earlier diagnosis and therapeutic intervention. We also review the relevant literature, and highlight new evidence that suggests that SDAE and the related disorder, subacute brainstem angioencephalopathy (SBAE), represent severe and terminal variants of posterior reversible encephalopathy syndrome (PRES). Taken together, we suggest a new understanding of SDAE/SBAE and PRES as a spectrum of hypertension-associated non-inflammatory vascular encephalopathies that are mediated by a loss of venous autoregulation, marked by cytotoxic edema, and that ultimately produce subacute-to-acute diencephalic, brainstem, and posterior fossa injuries with devastating neurologic consequences.