The Increasing Burden of Emergency Department and Inpatient Consultations for "Papilledema".

BACKGROUND: Increasing incidence of idiopathic intracranial hypertension (IIH), overreported radiologic signs of intracranial hypertension, difficult access to outpatient neuro-ophthalmology services, poor insurance coverage, and medicolegal concerns have lowered the threshold for emergency department (ED) visits for "papilledema." Our objective was to examine referral patterns and outcomes of neuro-ophthalmology ED and inpatient consultations for concern for papilledema. METHODS: At one university-based quaternary care center, all adults referred for "papilledema" over one year underwent a standardized ED "papilledema protocol." We collected patient demographics, final diagnoses, and referral patterns. RESULTS: Over 1 year, 153 consecutive patients were referred for concern for papilledema. After papilledema protocol, 89 of 153 patients (58%) had bilateral optic disc edema, among whom 89% (79/89) had papilledema (intracranial hypertension). Of the 38 of 153 (25%) consultations for suspected disorder of intracranial pressure without previous fundus examination (Group 1), 74% (28/38) did not have optic disc edema, 21% (8/38) had papilledema, and 5% (2/38) had other causes of bilateral disc edema. Of the 89 of 153 (58%) consultations for presumed papilledema seen on fundus examination (Group 2), 58% (66/89) had confirmed papilledema, 17% (15/89) had pseudopapilledema, and 9% (8/89) had other causes of bilateral optic disc edema. Of the 26 of 153 (17%) patients with known IIH (Group 3), 5 had papilledema and 4 required urgent intervention. The most common diagnosis was IIH (58/79). Compared with IIH, patients with secondary causes of intracranial hypertension were older (P = 0.002), men (P < 0.001), not obese (P < 0.001), and more likely to have neurologic symptoms (P = 0.002). CONCLUSION: Inpatient and ED consultations for "papilledema" are increasing. Of the 153 ED and inpatient neuro-ophthalmology consultations seen for "papilledema" over 1 year, one-third of patients with optic disc edema of unknown cause before presentation to our ED had new vision- or life-threatening disease, supporting the need for prompt identification and evaluation of optic disc edema in the ED. In the face of limited access to neuro-ophthalmologists, this study supports the need for emergency department access to expert eye-care evaluation or ocular fundus camera for prompt identification of optic disc edema and standardized evaluation for neurologic emergencies.

Neuro-ophthalmology Emergency Department and Inpatient Consultations at a Large Academic Referral Center.

PURPOSE: Prompt neuro-ophthalmology consultation prevents diagnostic errors and improves patient outcomes. The scarcity of neuro-ophthalmologists means that the increasing outpatient demand cannot be met, prompting many emergency department (ED) referrals by non-neuro-ophthalmologists. We describe our quaternary care institution's ED and inpatient neuro-ophthalmology consultation patterns and patient outcomes. DESIGN: Prospective observational study. PARTICIPANTS: Consecutive neuro-ophthalmology ED and inpatient consultation requests over 1 year. METHODS: We collected patient demographics, distance traveled, insurance status, referring provider details, consultation question, final diagnosis, complexity of consultation, time of consultation, and need for outpatient follow-up. MAIN OUTCOME MEASURES: Consultation patterns and diagnoses, complexity, and follow-up. RESULTS: Of 494 consecutive adult ED and inpatient neuro-ophthalmology consultations requested over 1 year, 241 of 494 consultations (49%) occurred at night or during weekends. Of ED consultations (322 of 494 [65%]), 127 of 322 consultations (39%) occurred during weekdays, 126 of 322 consultations (39%) occurred on weeknights, and 69 of 322 consultations (22%) occurred on weekends or holidays. Of 322 ED consultations, 225 of 322 consultations (70%) were patients who initially sought treatment in the ED with a neuro-ophthalmic chief symptom. Of the 196 patients sent to the ED by a health care professional, 148 patients (148/196 [76%]) were referred by eye care specialists (74 optometrists and 74 ophthalmologists). The most common ED referral questions were for papilledema (75 of 322 [23%]) and vision loss (72 of 322 [22%]). A total of 219 of 322 patients (68%) received a final active neuro-ophthalmic diagnosis, 222 of 322 patients (69%) were cases of high or very high complexity, and 143 of 322 patients (44%) required admission. Inpatient consultations (n = 172) were requested most frequently by hospitalists, including neurologists (71 of 172 [41%]) and oncologists (20 of 172 [12%]) for vision loss (43 of 172 [25%]) and eye movement disorders (36 of 172 [21%]) and by neurosurgeons (58 of 172 [33%]) for examination for mass or a preoperative evaluation (19 of 172 [11%]). An active neuro-ophthalmic diagnosis was confirmed in 67% of patients (116 of 172). Outpatient neuro-ophthalmology follow-up was required for 291 of 494 patients (59%). CONCLUSIONS: Neuro-ophthalmology consultations are critical to the diagnosis and management in the hospital setting. In the face of a critical shortage of neuro-ophthalmologists, this study highlights the need for technological and diagnostic aids for greater outpatient access. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Clinical Reasoning: A 55-Year-Old Woman With Recurrent Episodes of Aphasia and Vision Changes.

A 55-year-old woman presented with recurrent episodes of headache, vision changes, and language disturbances. Brain MRI showed multifocal white matter lesions, microhemorrhages, and enlarged perivascular spaces. After an extensive and unrevealing workup, she underwent a biopsy of brain and meninges that revealed thick and hyalinized leptomeningeal and cortical vessel walls that were strongly positive for ß-amyloid by immunohistochemical staining, suggestive of cerebral amyloid angiopathy (CAA). CAA can present as a spectrum of inflammatory responses to the deposition of amyloid-ß in the vessel walls. Her clinical presentation, radiologic, and histopathologic findings supported a diagnosis of probable CAA-related inflammation (CAA-ri). Although an uncommon entity, it is important to recognize it because most patients respond to immunosuppressive therapy.

Ischemic stroke in COVID-19: An urgent need for early identification and management.

OBJECTIVE: In the setting of the Coronavirus Disease 2019 (COVID-19) global pandemic caused by SARS-CoV-2, a potential association of this disease with stroke has been suggested. We aimed to describe the characteristics of patients who were admitted with COVID-19 and had an acute ischemic stroke (AIS). METHODS: This is a case series of PCR-confirmed COVID-19 patients with ischemic stroke admitted to an academic health system in metropolitan Atlanta, Georgia (USA) between March 24th, 2020 and July 17th, 2020. Demographic, clinical, and radiographic characteristics were described. RESULTS: Of 396 ischemic stroke patients admitted during this study period, 13 (2.5%) were also diagnosed with COVID-19. The mean age of patients was 61.6 ± 10.8 years, 10 (76.9%) male, 8 (61.5%) were Black Americans, mean time from last normal was 4.97 ± 5.1 days, and only one received acute reperfusion therapy. All 13 patients had at least one stroke-associated co-morbidity. The predominant pattern of ischemic stroke was embolic with 4 explained by atrial fibrillation. COVID-19 patients had a significantly higher rate of cryptogenic stroke than non-COVID-19 patients during the study period (69% vs 17%, p = 0.0001). CONCLUSIONS: In our case series, ischemic stroke affected COVID-19 patients with traditional stroke risk factors at an age typically seen in non-COVID populations, and mainly affecting males and Black Americans. We observed a predominantly embolic pattern of stroke with a higher than expected rate of cryptogenic strokes, a prolonged median time to presentation and symptom recognition limiting the use of acute reperfusion treatments. These results highlight the need for increased community awareness, early identification, and management of AIS in COVID-19 patients.

Collaborative Comanagement Between Neurohospitalists and Internal Medicine Hospitalists Decreases Provider Costs and Enhances Satisfaction With Neurology Care at an Academic Medical Center.

BACKGROUND AND PURPOSE: The majority of academic medical centers are moving to a neurohospitalist model of care for hospital neurology coverage. Potential benefits over a more traditional academic model of patient care include greater expertise in acute neurologic disease, increased efficiency, and improved availability to patients, providers, and learners. Despite these perceived advantages, switching to a neurohospitalist model can come at substantial financial cost, so finding ways to maximize the positive impact of a limited number of neurohospitalists is very important to the future health of academic neurology departments. Over the past 7 years, we have implemented a model for inpatient neurological care based on an intimate collaborative relationship between the neurology and hospital medicine services at our main academic hospital. Our goal was to optimize the value of care by decreasing cost while improving quality. METHODS: Cost and revenue associated with professional services was evaluated on a yearly basis. As part of ongoing quality improvement efforts, yearly surveys were administered to referring providers during the transition to a collaborative care model in which NHs and medicine hospitalists comanage neurology inpatients. RESULTS: Net operating loss was dramatically decreased upon transition to the new care model. Concomitantly, there was a robust positive impact on perception of overall quality, timeliness, and communication skills of neurology services. CONCLUSIONS: Collaborative comanagement is an effective strategy to improve overall satisfaction with neurology services at a tertiary academic medical center while maintaining financial viability.

Weakness in an Elderly Woman With Asthma and Chronic Sinusitis.

Weakness and sensory changes are common complaints in both the inpatient and the outpatient setting. However, this presentation remains a diagnostic challenge to clinicians due to the many possible underlying etiologies. The initial evaluation of weakness and sensory changes starts a thorough history and physical examination to guide the diagnostic process. In this article, we present the case of an elderly woman with complaints of weakness and sensory changes to highlight a step-wise approach to diagnosis and management.

Perioperative Consultation for Patients with Preexisting Neurologic Disorders.

As the population ages, the prevalence of many neurologic diseases is increasing. At the same time, older patients are undergoing more surgical procedures. This confluence of events puts neurohospitalists in a unique position to provide both pre- and postoperative guidance to minimize complications, improve clinical outcomes, and decrease health care costs in patients with neurologic comorbidities. Early preoperative consultation is recommended for patients with severe, poorly controlled, or decompensated neurologic disease, a recent stroke, or those undergoing procedures with a high risk of neurologic complications. The neurohospitalist's role includes optimizing management of preexisting diseases, such as epilepsy, neuromuscular disorders, Parkinson's disease, dementia, and cerebrovascular disease, as well as providing guidance for perioperative management and clarification of risks. In the postoperative period, the neurohospitalist will frequently be consulted to mitigate any negative impact of neurologic complications that do occur.

Defining the role of the academic neurohospitalist in residency education.

OBJECTIVE: We sought to better understand the potential impact of the burgeoning neurohospitalist model of inpatient care on education of neurology residents and to better define possible roles for "neurohospitalists" in residency education. METHOD: We designed a brief qualitative open-ended survey directed toward academic leaders in neurology and distributed it by e-mail to every academic neurology department in the United States and Canada. RESULTS: Of 83 respondents, 36 (43%) had an active neurohospitalist program and only 10% felt certain they would not have 1 within the next 5 years. All respondents expected to have residents continue to be involved with inpatient care. The main perceived advantage for resident education associated with neurohospitalists was inpatient care expertise, and the main expected disadvantage was decreased exposure to subspecialty attendings. The majority anticipated positive impact on all Accreditation Council for Graduate Medical Education core competencies predominantly based on neurohospitalists' expertise in the inpatient setting. CONCLUSION: The majority of academic neurology departments are expected to have a neurohospitalist program within the next 5 years. There are several perceived advantages and disadvantages to such a program for education of neurology residents. In general, the impact of these programs is expected to improve resident education. Regardless of expectations, neurohospitalists will likely play a prominent role in the education of the next generation of neurologists.

Causes and consequences of degeneration of the dorsal motor nucleus of the vagus nerve in Parkinson's disease.

SIGNIFICANCE: Parkinson's disease (PD) is no longer considered merely a movement disorder caused by degeneration of dopamine neurons in the midbrain. It is now recognized as a widespread neuropathological syndrome accompanied by a variety of motor and nonmotor clinical symptoms. As such, any hypothesis concerning PD pathogenesis and pathophysiology must account for the entire spectrum of disease and not solely focus on the dopamine system. RECENT ADVANCES: Based on its anatomy and the intrinsic properties of its neurons, the dorsal motor nucleus of the vagus nerve (DMV) is uniquely vulnerable to damage from PD. Fibers in the vagus nerve course throughout the gastrointestinal (GI) tract to and from the brainstem forming a close link between the peripheral and central nervous systems and a point of proximal contact between the environment and areas where PD pathology is believed to start. In addition, DMV neurons are under high levels of oxidative stress due to their high level of α-synuclein expression, fragile axons, and specific neuronal physiology. Moreover, several consequences of DMV damage, namely, GI dysfunction and unrestrained inflammation, may propagate a vicious cycle of injury affecting vulnerable brain regions. CRITICAL ISSUES: Current evidence to suggest the vagal system plays a pivotal role in PD pathogenesis is circumstantial, but given the current state of the field, the time is ripe to obtain direct experimental evidence to better delineate it. FUTURE DIRECTIONS: Better understanding of the DMV and vagus nerve may provide insight into PD pathogenesis and a neural highway with direct brain access that could be harnessed for novel therapeutic interventions.

Neurohospitalists enhance resident perception of the educational and clinical value of a night float rotation.

BACKGROUND AND PURPOSE: Neurology residency training programs have been profoundly impacted by recent changes in resident duty hours, workloads, and supervisory requirements. In response, many programs have adopted a night float coverage system to minimize the requirements for overnight call. The majority involves residents working a block of night shifts in what is typically a service-oriented rotation. Recently, concerns have arisen regarding the impact of this design on resident education and patient care. We have developed a novel on-site nighttime neurohospitalist model for the explicit purpose of steepening the initial learning curve for neurology residents in an effort to rapidly improve their neurological skills and, in conjunction, overnight patient care. We surveyed residents after the initiation of this system to assess their perception of the impact of direct overnight supervision on education and patient care. METHODS: As part of ongoing quality improvement efforts, surveys were administered to neurology house staff at a tertiary academic medical center after they had completed service on the night float rotation both with and without an attending in the hospital using a retrospective pre/postdesign. RESULTS: There was a robust positive impact on resident's perception of overall quality, educational value, and clinical quality on the night float rotation with an attending on-site. Despite an overall perception that their autonomy was maintained, residents believed barriers to contact the attending were lower, and attending interaction during critical decision making was more frequent. CONCLUSIONS: Direct overnight supervision by a neurohospitalist enhances the educational value and care quality on overnight resident rotations.

Parkinson's disease is not associated with gastrointestinal myenteric ganglion neuron loss.

Gastrointestinal dysfunction is a prominent non-motor feature of Parkinson's disease (PD) that contributes directly to the morbidity of patients, complicates management of motor symptoms, and may herald incipient PD in patients without motor disability. Although PD has traditionally been considered a disease of dopaminergic neurons in the substantia nigra, analyses of gastrointestinal samples from PD patients have consistently revealed pathology in the enteric nervous system. The relationship of PD pathology to GI dysmotility is poorly understood, and this lack of understanding has led to limited success in developing treatments for PD-related GI symptoms. We have quantitatively compared myenteric neuron density and relative abundance of NO, VIP, and catecholamine neurons between patients with PD and control individuals along the length of the GI tract. In addition, we have examined the frequency of GI α-synuclein neuritic pathology and its co-localization with the same neuronal markers. We have included a comparison with a small population of patients with incidental Lewy bodies found at autopsy. These data indicate that there is no neuronal loss in the myenteric plexus in PD. Lewy body pathology parallels parasympathetic autonomic input from the dorsal motor nucleus of the vagus, not the distribution of extrinsic sympathetic input or intrinsic enteric neurons, and is only rarely co-localized with tyrosine hydroxylase. These data provide a critical background to which further analyses of the effect of PD on the GI tract may be compared and suggest that neuropathology in myenteric neurons is unlikely to be a causative factor in PD-related GI dysmotility.

Alpha-synuclein transgenic mice display age-related slowing of gastrointestinal motility associated with transgene expression in the vagal system.

Gastrointestinal (GI) dysfunction is the one of the most common non-motor symptoms of Parkinson's disease (PD) and occurs in nearly every patient afflicted with this common neurodegenerative disorder. While parkinsonian motor symptoms are caused by degeneration of dopamine neurons in the midbrain substantia nigra, the neurological localization of non-motor symptoms in PD is not known. In this study, we examined a transgenic mouse model of PD in which mutant (A53T) human α-synuclein was expressed under control of the prion promoter (AS mice). We found that gastrointestinal expression of human α-synuclein in this transgenic line was limited to efferent fibers projecting from the dorsal motor nucleus of the vagus nerve (DMV) to the enteric nervous system (ENS). Older transgenic mice had a lower density of human α-synuclein expression in the GI tract, suggesting an age-related disruption of efferent vagal fibers in this model. At the same time, mice developed age-related declines in stool frequency and gastric emptying consistent with those seen in human PD. These behavioral and neuropathological patterns parallel those seen in PD patients and suggest the DMV as a target for further investigation into causes for GI neuropathology and symptomatology in parkinsonism.

Current status and future directions of gene expression profiling in Parkinson's disease.

Parkinson's disease (PD) is a common age-associated neurodegenerative disorder. Motor symptoms are the cardinal component of PD, but non-motor symptoms, such as dementia, depression, and autonomic dysfunction are being increasingly recognized. Motor symptoms are primarily caused by selective degeneration of substantia nigra dopamine (SNDA) neurons in the midbrain; non-motor symptoms may be referable to well-described pathology at multiple levels of the neuraxis. Development of symptomatic and disease-modifying therapies is dependent on an accurate and comprehensive understanding of the pathogenesis and pathophysiology of PD. Gene expression profiling has been recently employed to assess function on a broad level in the hopes of gaining greater knowledge concerning how individual mechanisms of disease fit together as a whole and to generate novel hypotheses concerning PD pathogenesis, diagnosis, and progression. So far, the majority of studies have been performed on postmortem brain samples from PD patients, but more recently, studies have targeted enriched populations of dopamine neurons and have begun to explore extra-nigral neurons and even peripheral tissues. This review will provide a brief synopsis of gene expression profiling in parkinsonism and its pitfalls to date and propose several potential future directions and uses for the technique. It will focus on the use of microarray experiments to stimulate hypotheses concerning mechanisms of neurodegeneration in PD, since the majority of studies thus far have addressed that complicated issue.

Neurochemical phenotypes of myenteric neurons in the rhesus monkey.

Understanding the neurochemical composition of the enteric nervous system (ENS) is critical for elucidating neurological function in the gastrointestinal (GI) tract in health and disease. Despite their status as the closest models of human neurological systems, relatively little is known about enteric neurochemistry in nonhuman primates. We describe neurochemical coding of the enteric nervous system, specifically the myenteric plexus, of the rhesus monkey (Macaca mulatta) by immunohistochemistry and directly compare it to human tissues. There are considerable differences in the myenteric plexus along different segments of the monkey GI tract. While acetylcholine neurons make up the majority of myenteric neurons in the stomach (70%), they are a minority in the rectum (47%). Conversely, only 22% of gastric myenteric neurons express nitric oxide synthase (NOS) compared to 52% in the rectum. Vasoactive intestinal peptide (VIP) is more prominent in the stomach (37%) versus the rest of the GI tract (≈10%), and catecholamine neurons are rare (≈1%). There is significant coexpression of NOS and VIP in myenteric neurons that is more prominent in the proximal GI tract. Taken as a whole, these data provide insight into the neurochemical anatomy underlying GI motility. While overall similarity to other mammalian species is clear, there are some notable differences between the ENS of rhesus monkeys, humans, and other species that will be important to take into account when evaluating models of human diseases in animals.

Gastrointestinal features of Parkinson's disease.

Gastrointestinal (GI) symptoms are among the most common nonmotor manifestations of Parkinson's disease (PD), and they have many important ramifications for patients. The purpose of this review is to raise awareness of the full spectrum of GI symptoms in PD which include weight loss, sialorrhea, dysphagia, nausea, constipation, and defecatory dysfunction. We will discuss their practical significance, and outline a clear approach to their evaluation and management. A brief discussion about the impacts of commonly used medical and surgical PD therapies on GI symptom manifestation is also included.

Animal models of gastrointestinal problems in Parkinson's disease.

Gastrointestinal (GI) symptoms are being increasingly recognized as early, common, and severe features of Parkinson's disease (PD), and they are important determinants of quality of life and disability throughout the disease course. In addition, the concept that GI symptoms could represent "pre-motor" PD has been hotly debated and has recently become a driving force for investigations into the pathophysiology and neuropathology of PD. Clinical data has been supported by many pathological studies demonstrating α-synuclein neuritic pathology and neuronal loss in central and peripheral areas relevant to GI function. As understanding has matured concerning the impact of GI dysmotility on patients and its potential relationship to incipient PD, behavioral and neuropathological examination of the GI tract has become a critical aspect of evaluation and validation of PD animal models. This review will briefly summarize GI symptoms and neuropathology in PD important to model in animals, behavioral and neuropathological examination of the GI tract in animals, and the current state of modeling parkinsonian GI dysfunction in animal model systems.