bluuebunny/crossref_metadata_2025 huggingface dataset:
The Role of Road Transportation in the Flood Evacuation Process
Authors: Borowska-Stefańska Marta , Wiśniewski Szymon
Published: November 2021

Floods, which are among the most dangerous and frequent disasters in the world, are expected to occur more frequently due to climate change. Floods, and flash floods in particular, generate economic, environmental, and social effects. Economic effects include damage to infrastructure, the negative influence upon transportation and communications networks, and an increase in fuel costs, as well as time loss due to traffic delays (congestion) and the necessity of taking alternative routes. It is therefore important to take action both to prevent and to mitigate these effects. In the 21st century there has been a radical change in the approach to the issue of flood protection (as seen in the approach formulated within the 2007 European Floods Directive)—it is no longer believed that there is such a thing as complete protection against floods, but that the damage and loss it inflicts can only be mitigated, and since floods cannot be completely eradicated, societies must learn how to live with them. In the event of a flood, preprepared procedures to counteract and mitigate the effects of the disaster are followed, including the evacuation of people and movable property from affected areas.

Evacuation planning is meant to reduce the number of disaster-related (including flood) fatalities and material losses. Crucially, this type of planning requires a well-defined, optimum evacuation policy for people and households within flood hazard areas. In addition, evacuation modelling is particularly important for authorities, planners, and other experts managing the process of evacuation, as it allows for more effective relocation of evacuees to safety. Modelling can also facilitate the identification of bottlenecks within the transportation system prior to the occurrence of a disaster; that is, it enables us to determine the impact of flood-related road closures, and to comprehend—among other things—the effects a phased evacuation has on traffic load. Furthermore, not only may the ability to model alternative evacuation scenarios lead us to establish appropriate policies, evacuation strategies, and contingency plans, but it might also facilitate better communication and information flow.

Evacuation from flood-hazard areas is a major challenge for the field of flood risk management as well as the fields of traffic engineering and transport planning. This is particularly true when the research has to include not only those journeys directly related to escape from hazardous places, but also their reflexive relationship with the total number of journeys made in the “background” of the evacuation itself.
Environmental Degradation: Estimating the Health Effects of Ambient PM2.5 Air Pollution in Developing Countries
Authors: Sánchez-Triana Ernesto , Larsen Bjorn , Enriquez Santiago , Santos Andreia Costa
Published: November 2021

Air pollution of fine particulates (PM2.5) is a leading cause of mortality worldwide. It is estimated that ambient PM2.5 air pollution results in between 4.1 million and 8.9 million premature deaths annually. According to the World Bank, the health effects of ambient PM2.5 air pollution had a cost of $6.4 trillion in purchasing power parity (PPP) adjusted dollars in 2019, equivalent to 4.8% of global gross domestic product (PPP adjusted) that year.

Estimating the health effects and cost of ambient PM2.5 air pollution involves three steps: (1) estimating population exposure to pollution; (2) estimating the health effects of such exposure; and (3) assigning a monetary value to the illnesses and premature deaths caused by ambient air pollution.

Estimating population exposure to ambient PM2,5 has gone from predominantly using ground level monitoring data mainly in larger cities to estimates of nationwide population weighted exposures based on satellite imagery and chemical transport models along with ground level monitoring data. The Global Burden of Disease 2010 (GBD 2010) provided for the first time national, regional and global estimates of exposures to ambient PM2.5. The GBD exposure estimates have also evolved substantially from 2010 to 2019, especially national estimates in South Asia, the Middle East and North Africa, Sub-Saharan Africa and Latin America and the Caribbean.

Estimation of health effects of ambient PM2.5 has also undergone substantial developments during the last two decades. These developments involve: i) going from largely estimating health effects associated with variations in daily exposures to estimating health effects of annual exposure; ii) going from estimating all-cause mortality or mortality from broad disease categories (i.e., cardiopulmonary diseases) to estimating mortality from specific diseases; and iii) being able to estimate health effects over a wide range of exposure that reflect ambient and household air pollution exposure levels in low- and middle-income countries.

As to monetary valuation of health effects of ambient air pollution, estimates in most low- and middle-income countries still rely on benefit transfer of values of statistical life (VSL) from high-income countries.
Treger, Harvey
Authors: Patterson George T.
Published: November 2021

Harvey Treger (1924–2016) was a pioneer in the social work profession, breaking new ground for social work practice in law enforcement agencies. Under Treger’s leadership, police social work was started as a new specialty area of social work practice. His groundbreaking vision for police social work practice continues to evolve to the present (2021), as progressively more law enforcement agencies either hire or establish collaborations with social workers, and community stakeholders recognize the need for a social work response to community social problems instead of law enforcement.
Rahman, Mohammad Habibur
Authors: Kumar Das Tulshi
Published: November 2021

Mohammad Habibur Rahman taught social work in two public universities in Bangladesh. He was the founder of a social work program in a science and technology university, the first of its kind in the country. Despite social work being a less familiar academic discipline, especially in a science and technology university, Rahman, a professor of social work, was for the first time appointed as the vice chancellor of the same university. Apart from teaching, he wrote a number of books on social work and social development, community development and community organization, and urbanization and urban social services and also conducted research on the socioeconomic and psychological causes of suicide, rural development and social services in Bangladesh, and urban community development. He presented social work-related papers in national and international conferences and also attended training courses and seminars on social work methods, social welfare policy formulation, and advanced planning in different countries.
Senescence in chronic wounds and potential targeted therapies
Authors: Wei Xuerong , Li Minxiong , Zheng Zijun , Ma Jun , Gao Yanbin , Chen Lianglong , Peng Yujie , Yu Shengxiang , Yang Lei
Published: November 2021

Chronic wounds (e.g. diabetic wounds, pressure wounds, vascular ulcers, etc.) do not usually heal in a timely and orderly manner but rather last for years and may lead to irreversible adverse events, resulting in a substantial financial burden for patients and society. Recently, a large amount of evidence has proven that cellular senescence has a crucial influence on chronic nonhealing wounds. As a defensive mechanism, cell senescence is a manner of cell-cycle arrest with increased secretory phenotype to resist death, preventing cells from stress-induced damage in cancer and noncancer diseases. A growing amount of research has advanced the perception of cell senescence in various chronic wounds and focuses on pathological and physiological processes and therapies targeting senescent cells. However, previous reviews have failed to sum up novel understandings of senescence in chronic wounds and emerging strategies targeting senescence. Herein, we discuss the characteristics and mechanisms of cellular senescence and the link between senescence and chronic wounds as well as some novel antisenescence strategies targeting other diseases that may be applied for chronic wounds.
Telemedicine Technologies
Authors: Smith Dean L.
Published: November 2021

This chapter provides an overview of telemedicine technologies, including hardware, software and peripheral devices, along with their use in emergency and other clinical settings. Telemedicine comes in many forms, reflecting the complex ways health care is delivered. The earliest examples of telemedicine were simply phone calls between clinicians. Now, telemedicine encompasses live videoconferences involving the full spectrum of stakeholders in the health care continuum, a wide array of diagnostic devices, as well as an ever increasing number of patient-driven modalities. Lessons learned from the implementation of telemedicine technologies and a checklist of factors for clinicians and health care administrators to consider prior to deploying telehealth solutions are included at the end of the chapter.
Telestroke and Teleneurology
Authors: Gross Hartmut , Switzer Jeffrey A.
Published: November 2021

Evaluation and treatment of acute stroke is the oldest and most widespread application of telemedicine. Telestroke systems allow provision of the same high quality of care provided at specialized stroke centers to patients at emergency departments without stroke coverage. The early treatment achieved with telestroke leads to better functional outcomes in stroke patients, thereby lowering overall cost of patient care. Telestroke networks facilitate optimal care, decrease hospital and physician liability, educate health care professionals, and keep many patients closer to home. Admissions to, rather than transfers from, rural sites retain hospitalization revenues locally and help keep small, financially struggling hospitals viable.
Emergency Ocular Telehealth Services
Authors: Goodwin Anika , Khoo Chloe T. L.
Published: November 2021

The availability of ocular telehealth services in the emergency department and urgent care center settings can facilitate the diagnosis and management of ocular complaints. While the start-up costs involving ophthalmic equipment and training modules may seem high, it is well worth the investment knowing that these services will increase patient satisfaction, improve overall clinical outcomes, and aid in the physician decision-making process in regard to patient transfers for eye emergencies. Although the majority of ophthalmic urgencies can be managed as outpatient, true ophthalmic emergencies may require more immediate evaluation in order to preserve good visual outcome. Gaps in the availability of ophthalmologists needed for the immediate evaluation of eye emergencies can be filled by the implementation of ocular telehealth.
Teletriage
Authors: Laghezza Matthew , Greenwald Peter , Booker Ethan , Mishkin David , Sharma Rahul
Published: November 2021

Under the Emergency Medicine Treatment and Labor Act (EMTALA), every patient who presents to an emergency department must receive a Medical Screening Exam (MSE). One approach to meet EMTALA MSE requirements has been the provider in triage (PIT) model. In most PIT models, an advanced practice provider (APP) will obtain an accurate history along with performing a focused physical examination to expedite care for the patient. With telemedicine quickly becoming a mainstream method of health care delivery, many institutions are considering a combination of a PIT model with telemedicine to improve patient throughput.
Extending Care Team Access to Out-of-Hospital Settings via Telehealth
Authors: Wassenaar Denise , Knight Paul , Hanson Mark A. , Wessler Jeffrey D.
Published: November 2021

This chapter explores the use of telehealth within out-of-hospital (OOH) settings, comprising skilled nursing facilities, home care, independent and assisted living, and palliative care. The transition to value-based care is discussed within the context of its applicability to these OOH settings. As health care focuses on cost containment while still providing quality care, providers are turning to the use of telehealth to improve the management of care of seniors living in both community and residential settings. The role of the emergency physician, through telehealth, is introduced as an addition to the interdisciplinary health team in meeting the goal of avoiding unnecessary emergency department visits, often resulting in hospitalization. As the adoption of telehealth grows, benefits are documented, and payment challenges are overcome, the hope is that it becomes another solution to deliver efficient and cost-effective health care, particularly in OOH settings.
Direct-to-Consumer or On-Demand Telehealth
Authors: Joshi Aditi U. , Waller Sylvan
Published: November 2021

Direct-to-consumer (DTC) or on-demand telehealth describes a patient-initiated medical encounter using technology in real time—mostly related to acute care or urgent complaints. Initially created and offered by private companies, DTC telehealth is now more ubiquitous, as it has been offered by hospitals, medical centers, multi-specialty practices, and insurers. On-demand telehealth services are implemented in an effort to increase health care access, improve satisfaction and efficiency, as well as drive new revenue. This chapter outlines the types of DTC telehealth, considerations when conducting a needs assessments, implementation, business models, and quality assurance.
Emerging and Innovative Technologies
Authors: Aalam Ahmad A. , Tarassoli Sam P. , Drury Damien J. , Carayannis Elias G. , Meltzer Andrew C.
Published: November 2021

To provide acute unscheduled care 24 hours per day and 7 days per week is the core mission of emergency medicine. Emergency telehealth is evolving in scope and complexity, no longer constraining care by the walls of the emergency department (ED). Current audio- and video-based communications will advance to support a complex interplay between enhanced video communication, remote patient monitoring, augmented reality, and machine learning. Many of these technologies already exist or are under development for near-term implementation. For those deploying or planning the deployment of emergency telehealth services, this chapter highlights near-term technologies and applications to be considered.
A Woman With Subacute Painful Vision Loss
Authors: Jitprapaikulsan Jiraporn , Bhatti M. Tariq , Eggenberger Eric R. , Acierno Marie D. , Chen John J.
Published: November 2021

A 51-year-old White woman sought care for vision loss 1 week after a nonspecific upper respiratory tract infection. She reported pain in both eyes exacerbated by eye movement, which lasted for several days, followed by bilateral vision loss to the level of counting fingers–only vision. Optic neuritis was diagnosed, and she was treated with 1 g intravenous methylprednisolone for 3 days. Her vision improved substantially, and the pain resolved during the corticosteroid treatment. However, 1 week later, she woke up with right eye pain and vision loss. She was again treated with 5 days of intravenous methylprednisolone, with visual improvement nearly back to baseline. Two weeks later, she had recurrence of painful vision loss in both eyes. A diagnosis of chronic relapsing inflammatory optic neuropathy was made. Tests for serum angiotensin-converting enzyme, antineutrophil cytoplasmic antibody, antinuclear antibody, Lyme disease, syphilis, tuberculosis, and aquaporin-4-immunoglobulin G antibodies were negative. Serum was definitively positive for myelin oligodendrocyte glycoprotein-immunoglobulin G antibodies at a titer of 1:1,000. Myelin oligodendrocyte glycoprotein-immunoglobulin G–associated recurrent optic neuritis was diagnosed. After her diagnosis of recurrent corticosteroid-dependent optic neuritis associated with myelin oligodendrocyte glycoprotein-immunoglobulin G positivity, the patient was treated with 5 days of intravenous methylprednisolone. The eye pain resolved, and her vision returned to normal. At follow-up evaluation, the patient’s visual acuity, color vision, and visual fields were normal in both eyes, but there was mild bilateral optic disc pallor. She has not had recurrent demyelinating episodes while on chronic immunotherapy. Optic neuritis is an inflammatory demyelination of the optic nerve manifesting as acute to subacute vision loss, classically associated with pain with eye movement. The long-term prevention and prognosis depend on the cause of the optic neuritis.
Rapidly Progressive Numbness and Weakness After Soft-Tissue Abscess
Authors: Sechi Elia , Wingerchuk Dean M.
Published: November 2021

A previously healthy 45-year-old man had development of neck pain and swelling, followed 1 week later by fevers, chills, and night sweats. Cervical computed tomography showed a left-sided cervical soft-tissue abscess. The patient was treated with oral cephalexin for 10 days, without benefit. Fine-needle aspiration biopsy of the mass showed granulomatous inflammation and a heterogeneous lymphocyte population without evidence of malignancy. Meropenem and gentamicin were started. Ten days later, he had development of acute urinary retention, numbness and weakness in the lower extremities, and numbness in the upper extremities. At symptom nadir 2 days later, he required the aid of a walker to ambulate. Lhermitte sign and erectile dysfunction were also present. The patient was admitted to the hospital. Spinal cord magnetic resonance imaging showed a longitudinally extensive, nonenhancing, T2-hyperintense lesion predominantly affecting the ventral and lateral parenchyma of the cervical and thoracic spinal cord. Cerebrospinal fluid examination showed a white blood cell count of 581 cells/µL with 42% neutrophils, 35% lymphocytes, and 22% monocytes, increased protein concentration (109 mg/dL), and normal glucose concentration. A diagnosis of postinfectious idiopathic transverse myelitis was made. The patient was treated with intravenous immunoglobulin, intravenous methylprednisolone, and broad-spectrum antibiotics, with improvement of both the abscess and his neurologic symptoms. After discharge, he was able to walk unassisted. At follow-up evaluation 6 months after the initial evaluation, neurologic examination showed only mild weakness of the left iliopsoas muscle and brisk reflexes in the lower extremities. Acute transverse myelopathies are a heterogeneous group of spinal cord disorders characterized by acute or subacute signs and symptoms of spinal cord dysfunction, typically a combination of sensory, motor, and autonomic manifestations. Underlying causes include vascular, infectious, neoplastic, postirradiation, traumatic, and inherited/metabolic, and inflammatory processes.
New-Onset Right-Sided Numbness and Double Vision
Authors: Tobin W. Oliver
Published: November 2021

A 40-year-old right-handed man sought care for right hand numbness, right-sided facial numbness, and diplopia progressing to maximal severity over 10 days. At his worst he was unable to write. His symptoms remained maximal for 4 weeks. He was hospitalized and treated with 5 days of intravenous methylprednisolone. He improved to approximately 95% of normal over 4 weeks. He had residual mild right-sided facial and right leg numbness.

Antibodies to JC polyoma virus and varicella-zoster virus were positive, which indicated prior exposure to these viruses. Total 25-hydroxyvitamin D level was low at 8.2 ng/mL. Optical coherence tomography findings were normal. Magnetic resonance imaging of the brain performed 3 months after the onset of symptoms demonstrated a T2-hyperintense lesion in the left midbrain peduncle extending into the upper pons, without gadolinium enhancement. A small area of T2 hyperintensity was seen in the right frontal deep white matter. Follow-up brain magnetic resonance imaging showed almost complete resolution of the left midbrain peduncle lesion with persistence of the right frontal deep white matter lesion. Spinal fluid analysis showed 1 white blood cell/µL with 95% lymphocytes, protein 35 mg/dL, 0 unique oligoclonal bands, and normal immunoglobulin G index (0.54).

A diagnosis of clinically isolated syndrome-first episode of multiple sclerosis was made.

After detailed discussion with the patient, he elected to commence disease-modifying therapy with fingolimod. He underwent routine monitoring with magnetic resonance imaging of the brain and cervical and thoracic spine on an annual basis, without any further relapses at 5-year follow-up.

Patients with typical demyelinating syndromes may not always fulfill the diagnostic criteria for multiple sclerosis. After careful exclusion of other mimicking conditions, most notably aquaporin-4-immunoglobulin G– and myelin oligodendrocyte glycoprotein-immunoglobulin G–associated disease, a diagnosis of clinically isolated syndrome may be made.
A Septuagenarian With Progressive Hemiparesis
Authors: Kassa Roman , Keegan B. Mark
Published: November 2021

A 78-year-old man with no pertinent medical history sought care for an 18-month history of progressive right lower extremity weakness, gait impairment, and falls. On neurologic examination, he had a hemiparetic gait. He had normal higher cognitive function and cranial nerve function. Motor examination showed decreased bulk over the right hand with no fasciculations, mild spasticity over the right leg, and right hemiparesis with an upper motor neuron pattern. Deep tendon reflexes were brisk throughout his limbs, and he had an extensor plantar reflex on the right side. He had impaired vibratory sense at the toes, with otherwise normal sensory and coordination examinations.

Magnetic resonance imaging (MRI) of the brain showed ovoid periventricular and punctate subcortical and deep white matter T2 hyperintense foci. Some of these had corresponding T1 hypointensity. MRI of the cervical spine showed 1 eccentrically located T2 hyperintense lesion over the right lateral aspect of C2. Cerebrospinal fluid analysis showed no pleocytosis, an increased protein concentration of 66 mg/dL, and 4 unique oligoclonal bands.

A diagnosis of primary progressive multiple sclerosis, very late onset, was made.

With any diagnosis of late-onset multiple sclerosis, a decision about whether multiple sclerosis disease-modifying agents are indicated should be carefully considered. Our older patient had a progressive disease course, and neuroimaging studies did not reveal evidence of active disease. Based on this, a decision was made to monitor him clinically and radiologically. Management of spasticity with regular daily stretching exercises was discussed with him.

A first clinical manifestation of multiple sclerosis can occur at a later-than-typical age. Most studies consider an onset at age 50 years or older to be late-onset multiple sclerosis, whereas first symptoms occurring at age 60 years or older are commonly referred to as very late–onset MS.
Recurrent Demyelinating Episodes
Authors: Collazo I. Vanessa Marin
Published: November 2021

A 28-year-old woman with a history of migraine, type 2 diabetes, irritable bowel syndrome, and mild anxiety sought care for right monocular painful vision loss presenting over 3 days. Two years earlier, she had had back paresthesias for 1 week which were triggered by flexion of the neck suggestive of Lhermitte sign. She also reported intermittent bilateral arm paresthesias for 2 years. She had almost daily headaches, treated with naproxen daily. She reported no bladder, bowel, or sexual dysfunction or episodes of vertigo, diplopia, or unexplained nausea, vomiting, or hiccups.

Magnetic resonance imaging of the brain revealed multifocal T2 fluid-attenuated inversion recovery white matter hyperintensities throughout the brainstem, cerebellum, and bilateral periventricular, subcortical, and juxtacortical areas. Magnetic resonance imaging of the cervical and thoracic spinal cord showed intramedullary cord signal T2 hyperintensities peripherally located at C1 and on the left side at C3-C4.

The patient was diagnosed with relapsing-remitting multiple sclerosis with activity on the basis of her clinical presentation and magnetic resonance imaging findings.

She received comprehensive education about the disease and the role of disease-modifying therapy to decrease the risk of further clinical and radiographic activity. After discussion of the risks and benefits of the various agents, the patient started treatment with dimethyl fumarate. Oral vitamin D3 supplementation was recommended. Healthy lifestyle changes, including maintaining a healthy diet, normal weight range, regular exercise, and sleep hygiene were recommended.

Disease-modifying therapies for relapsing-remitting multiple sclerosis are primarily used to reduce the occurrence of clinical relapses and the appearance of new T2 or enhancing lesions on magnetic resonance imaging of the brain and spine. Long-term treatment with disease-modifying therapy is associated with lower morbidity and mortality rates.
Breakthrough Disease While on Multiple Sclerosis Immunomodulatory Therapy
Authors: Carter Jonathan L.
Published: November 2021

A 36-year-old woman with a history of relapsing-remitting multiple sclerosis was evaluated for new multiple sclerosis symptoms accompanied by new, enhancing, white matter lesions on brain magnetic resonance imaging. Her multiple sclerosis presented with L’hermitte sign when she was 24 years old. She had onset of bilateral lower extremity and left upper extremity tingling at age 26 years. Magnetic resonance imaging and cerebrospinal fluid examination at the time were supportive of the diagnosis of multiple sclerosis, and disease-modifying therapy was recommended by her neurologist. She initiated therapy with dimethyl fumarate at age 30 years after several further relapses. Surveillance magnetic resonance imaging showed new gadolinium-enhancing lesions on brain magnetic resonance imaging on each of 3 consecutive yearly scans.

Urine culture and sensitivity tests were performed to rule out occult urinary tract infection; results of this testing were negative. magnetic resonance imaging of the brain concurrently showed new enhancing white matter lesions.

The patient was diagnosed with clinical and radiographic breakthrough disease activity while receiving therapy for multiple sclerosis.

The patient was treated with 5 days of intravenous methylprednisolone for her relapse. After discussion with the patient, it was decided to transition therapy from dimethyl fumarate to ocrelizumab infusions for her breakthrough disease activity. This decision was further supported by the patient’s concerns that she might be entering an early progressive phase of the disease.

In patients with spinal-predominant multiple sclerosis, or with symptoms potentially indicating new spinal cord involvement, it may be necessary to include spinal cord imaging to assess for new disease activity.
Weakness and Dysarthria After Radiosurgery
Authors: McKeon Andrew
Published: November 2021

A 60-year-old woman with a history of Sjögren syndrome had an episode of painful left eye vision loss. Brain magnetic resonance imaging showed an arteriovenous malformation adjacent to the left ventricular atrium. Although this was considered an asymptomatic lesion, the patient underwent stereotactic radiosurgery to reduce the risk of future growth and hemorrhage. Within days of the surgery, speech disturbance and weakness of the right arm and leg developed. Examination indicated a subcortical language deficit and an upper motor pattern of paresis of right-sided limbs.

Considered in the differential diagnosis were new hemorrhage from the arteriovenous malformation, ischemic stroke, radiation-induced necrosis, abscess, and demyelinating disease. Magnetic resonance imaging of the head with and without gadolinium contrast was used to evaluate for these possibilities. Brain magnetic resonance imaging after onset of speech and motor symptoms demonstrated new areas of confluent T2 signal abnormality in the brainstem and deep white matter of the left hemisphere, with some accompanying enhancement but without evidence of hemorrhage or acute stroke. Slow conduction was noted in the left optic nerve on visual evoked potentials.

The patient was diagnosed with optic neuritis, with subsequent evolution to multiple sclerosis in the setting of radiosurgery.

The patient received intravenous methylprednisolone with full recovery of language function and partial recovery of the hemiparesis, which improved with rehabilitation such that she could walk without a gait aid. Because the patient had more than 1 episode, she was treated with interferon beta-1a for further prevention of multiple sclerosis relapse.

Chronic neurotoxicity leading to subcortical dementia occurs in approximately 25% of patients undergoing whole-brain radiotherapy. Histologically, demyelination, as well as necrosis, can be a prominent feature. Among patients with multiple sclerosis or a clinically isolated syndrome, the risk of demyelinating events appears to increase after brain radiotherapy, within the field of treatment.
Personality Changes, Cognitive Decline, and Jerking Movements
Authors: Chiriboga A. Sebastian Lopez
Published: November 2021

A 64-year-old man had development of abnormal movements characterized by grimacing of the left hemiface and posturing of the ipsilateral arm. He had a generalized tonic-clonic seizure, followed by cognitive decline requiring assistance for most activities of daily living. He was readmitted to the hospital for neurologic evaluation.

His heart rate was 120 beats/min, he was afebrile. On neurologic examination, he had a Mini-Mental State Examination (MMSE) score of 16/30. The patient was oriented to person only, and he had multiple, frequent, left hemibody jerks during examination.

Cerebrospinal fluid analysis indicated increased protein concentration but otherwise normal findings. Laboratory abnormalities included a serum sodium and antibodies to leucine-rich, glioma-inactivated protein 1. Electroencephalography revealed diffuse slowing and bilateral temporal lobe epileptiform discharges. Magnetic resonance imaging of the brain showed T2/fluid-attenuated inversion recovery hyperintensities in the bilateral hippocampi and amygdalae and gadolinium enhancement in the same regions. Brain 18F-fludeoxyglucose–positron emission tomography demonstrated hypermetabolism in the mesiotemporal lobes bilaterally.

A diagnosis of limbic encephalitis associated with leucine-rich, glioma-inactivated protein 1-immunoglobulin G antibodies was. The unilateral facial and arm movements were consistent with faciobrachial dystonic seizures and are pathognomonic of the disorder.

The patient’s seizure frequency did not improve with antiepileptic drug therapy. The patient was treated with intravenous methylprednisolone, followed by plasmapheresis. This resulted in substantial improvement of his cognitive dysfunction and decrease in the faciobrachial dystonic seizures frequency. Therapy was transitioned to oral prednisone and the patient was discharged from the hospital.

This patient had classic features of leucine-rich, glioma-inactivated protein 1 antibody encephalitis: faciobrachial dystonic seizures, personality changes, subacute cognitive decline, hyponatremia, and improvement with immunotherapy.
Rapidly Progressive Memory Loss, Mood Change, Mutism, and Abnormal Movements
Authors: Shah Shailee S. , Grill Marie F.
Published: November 2021

A 24-year-old woman sought care for 2 weeks of disorientation and short-term memory difficulties, as well as diffuse tremor of all extremities. She returned with further decline in memory and new severe headaches. She had intermittent agitation and emotional outbursts of crying or laughing, insomnia, spells consisting of disorganized speech and episodes of intermittent right gaze deviation with facial twitching and lip smacking. She was nearly mute. Her appetite had decreased and she had not had a bowel movement in several days. She was noted to have significant tachycardia and was intermittently febrile. Within several days she became unresponsive to all external stimuli, with nonpurposeful eye movements and frequent dyskinesias observed, and ultimately required ventilator support.

Testing of the cerebrospinal fluid showed 236 white blood cells/µL, mildly increased protein concentration of 50 mg/dL, and normal glucose values. Electroencephalography initially demonstrated generalized slowing and generalized periodic epileptiform discharges and was also notable for an extreme delta brush pattern. Bilateral ovarian masses were identified on pelvic ultrasonography, and subsequent computed tomography of the abdomen and pelvis showed bilateral teratomas. An autoimmune encephalitis autoantibody panel was positive for antibodies targeting the N-methyl-d-aspartate receptor in the serum and cerebrospinal fluid, by both cell-based and immunofluorescence assays.

The patient was diagnosed with anti- N-methyl-d-aspartate receptor encephalitis.

The patient initially received intravenous methylprednisolone, followed by intravenous immunoglobulin. Benzodiazepines and propranolol were used to manage agitation and dysautonomia. Antiepileptic drugs were initiated for seizures. She required mechanical ventilation and parenteral nutrition given her persistent profound encephalopathic state. She underwent left ovarian cystectomy and right salpingo-oophorectomy.

This patient’s history highlights the progressive clinical features characteristic of anti- N-methyl-d-aspartate receptor encephalitis and the long but often complete or near-complete recovery.
Behavioral and Cognitive Changes Followed by Coma
Authors: Zekeridou Anastasia
Published: November 2021

A 60-year-old woman sought care for behavioral and cognitive changes over 1 week. She became irritable, could not run her daycare facility, and had short-term memory loss and. She was admitted to the hospital, where decreased consciousness developed over 2 to 3 days. She became comatose and needed to be intubated because of an aspiration event.

Her clinical examination at admission showed short-term memory and attention deficits and generalized hyperreflexia without any meningeal signs or myoclonic jerks. She also had evidence of left axillary and retroclavicular lymphadenopathy.

Magnetic resonance imaging of the brain showed T2 and fluid-attenuated inversion recovery bilateral hyperintensities in the caudate nuclei and putamina, without any diffusion abnormalities. Cerebrospinal fluid analysis showed lymphocytic, increased protein, and cerebrospinal fluid -restricted oligoclonal bands. Electroencephalography showed diffuse slowing with no epileptiform activity. Neural autoantibody testing was positive for immunoglobulin G antibodies to α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor in the serum and cerebrospinal fluid by indirect tissue immunofluorescence and antigen-specific cell-based assays. Because of suspicion for paraneoplastic neurologic disease, positron emission tomography of the body was performed and showed hypermetabolic axillary, retropectoral, and retroclavicular lymph nodes. Lymph node biopsy showed metastatic melanoma of unknown primary.

A diagnosis of paraneoplastic anti- α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis was made.

The patient received treatment for melanoma (vemurafenib and cobimetinib) and encephalitis (intravenous methylprednisolone) and plasma, followed by rituximab. She remained intubated and unresponsive but subsequently recovered substantially. By 3 months the patient had minimal short-term memory loss; at 2-year follow-up her function had returned to baseline and her cancer was in remission.

Anti-α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis can be severe, necessitating a protracted immunotherapy course and cancer treatment.
Episodic Hemiparesis, Cognitive Decline, and Seizures in a Woman With Pernicious Anemia
Authors: Valencia-Sanchez Cristina , McKeon Andrew
Published: November 2021

A 46-year-old woman with a history of pernicious anemia, sought care for intermittent episodes of weakness in her right upper extremity and speech difficulties followed by a headache. It was initially thought that she had migraine headaches. Blood tests showed increased thyrotropin and low free thyroxine levels, and she was diagnosed with Hashimoto thyroiditis. She initiated treatment with levothyroxine. One month later, the patient had a confusional episode and over the course of the following 2 months, the cognitive difficulties progressed. She was brought to the emergency department after a generalized tonic-clonic seizure. On examination, she was profoundly encephalopathic. Formal bedside cognitive testing could not be obtained.

Findings of magnetic resonance imaging of the brain were normal. Electroencephalography revealed diffuse slowing. Cerebrospinal fluid analysis showed increased protein concentration and lymphocytic pleocytosis. Thyroid peroxidase antibody value was markedly increased. Thyroglobulin antibody value was also increased. Consistent with her history of pernicious anemia, she was positive for gastric parietal cell antibodies.

The clinical presentation was compatible with steroid-responsive encephalopathy associated with autoimmune thyroiditis, also known as Hashimoto encephalopathy.

The patient started levetiracetam therapy and had no further seizures. She received intravenous methylprednisolone. At the completion of treatment, her confusion rapidly and substantially improved. She was discharged home on oral prednisone. She also started pantoprazole, calcium, and vitamin D supplementation and Pneumocystis jirovecii prophylaxis. For long-term immunotherapy, she initiated methotrexate.

Three months later, the patient and her family reported 90% improvement in her cognitive functioning and resolution of the episodes of hemiparesis.

The patient continued tapering prednisone. It was recommended that she continue methotrexate for 5 years before discontinuing.

Steroid-responsive encephalopathy associated with autoimmune thyroiditis, also known as Hashimoto encephalopathy, was initially described as strokelike episodes and subacute encephalopathy months after the onset of autoimmune (Hashimoto) thyroiditis.
Rapidly Progressive Dementia and Thyroid Antibodies
Authors: Kunchok Amy C. , Flanagan Eoin P.
Published: November 2021

A 70-year-old woman sought care for subacute cognitive decline. She initially began to get lost in familiar places. She also reported multiple episodes of hot flashes accompanied by an unusual abdominal sensation that lasted 90 seconds each, which were suspicious for sensory seizures. After symptom onset, her cognition rapidly deteriorated over the subsequent 6 weeks such that she had difficulty with activities of daily living.

On neural autoantibody testing, serum and cerebrospinal fluid were positive for α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor immunoglobulin G autoantibody by tissue-based indirect immunofluorescence assay, which was molecularly confirmed by α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor–specific cell-based assay. Because of the paraneoplastic significance of α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor–immunoglobulin G, a comprehensive search for cancer followed.

18F-Fludeoxyglucose positron emission tomography-computed tomography of the body demonstrated increased 18F-fludeoxyglucose uptake (hypermetabolism) in the right hilum and numerous mediastinal lymph nodes, suggestive of malignancy. Dedicated positron emission tomography-computed tomography brain imaging demonstrated generalized atrophy, without abnormal uptake. Bronchoscopy with lymph node biopsy was undertaken. Small cell carcinoma of the lung was found on histopathologic analysis.

The patient was diagnosed with paraneoplastic autoimmune dementia in the setting of α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor autoantibodies and stage IIIB small cell carcinoma of the lung.

The metastatic small cell carcinoma of the lung was treated with chemotherapy (carboplatin and etoposide) and radiation, with disease resolution. For her cognitive symptoms, she was given intravenous methylprednisolone, with resolution of symptoms.

An insidious onset of cognitive decline in older persons is suggestive of a neurodegenerative dementia. However, a rapidly progressive dementia or a subacute or fluctuating course should raise suspicion for an autoimmune/paraneoplastic cause, which is critical to identify given the potential for reversibility.
A Man With Flulike Symptoms and Hemorrhagic Brain Lesions
Authors: Toledano Michel
Published: November 2021

A 52-year-old man is admitted to a neurosciences intensive care unit during winter for management of seizures requiring mechanical ventilation. Two days earlier he reported cough and myalgia. He was found seated on the couch with altered mental state and was minimally responsive.

Upon arrival to the emergency department he was febrile at 38.8 °C and tachycardic. Complete blood cell count showed leukocytosis (11.1×109 cells/L, neutrophilic predominance). Computed tomography of the head showed an area of hypodensity in the left temporal lobe. During computed tomography, the patient had generalized convulsions requiring lorazepam, fosphenytoin, and levetiracetam, followed by initiation of a continuous midazolam infusion before seizures were controlled.

He was started on broad-spectrum antimicrobials, including acyclovir, and a lumbar puncture was performed. Cerebrospinal fluid protein concentration was 196 mg/dL, and he had 10 white blood cells/µL with lymphocyte predominance. There was no hypoglycorrhachia.

After 24 hours, the patient was weaned from the midazolam infusion and maintained on levetiracetam monotherapy. He was extubated but remained encephalopathic. Magnetic resonance imaging performed the day after admission demonstrated numerous T2 hyperintense lesions throughout both cerebral hemispheres including both mesial temporal lobes and right thalamus.

Nasopharyngeal polymerase chain reaction was positive for influenza virus A, which was later typed further and identified as pandemic 2009 H1N1 virus.

A diagnosis of influenza-associated encephalopathy/encephalitis was made.

The patient was treated with oseltamivir, as well as high-dose intravenous methylprednisolone. His encephalopathy gradually improved. Repeated imaging at 3-month follow-up showed resolution of the previously seen abnormalities. His neurologic examination was normal.

Postinfectious or parainfectious autoimmunity syndromes refer to neurologic signs and symptoms that develop during or after an infection but are not thought to be caused by direct infection of the nervous system.
Stiffness, Spasms, and Frequent Falls in a 41-Year-Old Man
Authors: Devine Michelle F. , Chiriboga A. Sebastian Lopez
Published: November 2021

A 41-year-old man sought care for 3 years of right-sided muscle stiffness. He also had 5- to 10-minute episodes of severe muscle spasms. He noted development of daily episodes of sudden, severe stiffness, often triggered by unexpected stimuli (eg, a touch or loud sound). He started using a walker and stopped driving. He stopped working because of increasing difficulty with mobility and cognition.

On neurologic examination, he had a Kokmen Short Test of Mental Status score of 28/38, with points lost for orientation, attention, calculation, and recall. Cranial nerve examination showed bilateral ptosis and hypometric saccadic eye movements. He had normal strength but diffuse rigidity with increased tone, most severe in the right lower extremity.

Magnetic resonance imaging of the brain indicated right parietal postoperative changes (post hematoma evacuation). Electroencephalography showed dysrhythmia grade 1 over the right frontotemporal region (above the prior hematoma). Cerebrospinal fluid was inflammatory, with mildly increased protein concentration and supernumerary oligoclonal bands.

Movement laboratory evaluation demonstrated an exaggerated startle and abnormal exteroceptive response consistent with central nervous system hyperexcitability.

Neural-specific autoantibody testing was positive for glycine receptor α‎1 subunit-immunoglobulin G in both serum and cerebrospinal fluid.

He was diagnosed with progressive encephalomyelitis with rigidity and myoclonus with positive glycine receptor α‎1 subunit-immunoglobulin G.

Given the immune-mediated cause of progressive encephalomyelitis with rigidity and myoclonus, he was started on intravenous methylprednisolone and concurrent rituximab. The patient markedly improved. His anxiety was still severe, however, and required increased escitalopram and cognitive behavioral therapy to control.

He was tapered off intravenous methylprednisolone and maintained on rituximab. His symptoms eventually resolved. He remained stable at 2-year follow-up after initiating immunosuppression.

Progressive encephalomyelitis with rigidity and myoclonus is considered a variant of stiff-person syndrome. There is clinical overlap between progressive encephalomyelitis with rigidity and myoclonus and classic stiff-person syndrome, which are both characterized by central nervous system hyperexcitability with exaggerated startle, muscle rigidity, and painful spasms.
Progressive Quadriparesis and Cancer
Authors: Sechi Elia , Flanagan Eoin P.
Published: November 2021

A 67-year-old man with a history of cigarette smoking sought care at the emergency department for nonspecific dizziness and fatigue. During evaluation, chest radiography showed a right upper lobe mass, and he subsequently underwent right upper lobectomy. Histologic analysis of resected tissue showed the mass to be small cell lung carcinoma. Progressive myelopathy developed. Adjuvant chemotherapy with carboplatin and etoposide was begun. His neurologic symptoms continued to worsen, with gait imbalance, along with numbness and dysesthesias of the 4 limbs and trunk, with a sensory level at C3-C4. He also reported severe bowel and bladder dysfunction. At his neurologic nadir, he had severe quadriparesis and was wheelchair dependent.

Cerebrospinal fluid analysis showed lymphocytic pleocytosis of 9 lymphocytes/µL, erythrocyte count of 2/µL, and normal protein and glucose values. Serum neural autoantibody screening revealed the presence of collapsin-response mediator protein 5–immunoglobulin G antibodies on both tissue-based indirect immunofluorescence assay and Western blot.

The patient was diagnosed with paraneoplastic myelopathy.

The patient was initially treated acutely with a combination of high-dose oral prednisone and plasmapheresis, without improvement. Subsequently, a combination of intravenous immunoglobulin and rituximab resulted in partial improvement. Soon after treatment discontinuation, his symptoms returned with worsening weakness, numbness, and neuropathic pain. Monthly intravenous immunoglobulin and rituximab were reinitiated, with improvement again noted. Oral corticosteroids, methadone, and high-dose gabapentin were also administered, with mild benefit for neuropathic pain. Follow-up spine magnetic resonance imaging 3 years after symptom onset showed evidence of spinal cord atrophy. At 5-year follow-up he remained in remission from small cell lung cancer but was wheelchair dependent.

Paraneoplastic myelopathy is a rare and underrecognized neurologic disorder that most often manifests before cancer detection, Clinical presentation is generally subacute or slowly progressive over months, but acute onset is possible. Cerebrospinal fluid typically shows lymphocytic pleocytosis, as in this case patient.