bluuebunny/crossref_metadata_2025_split huggingface dataset:
REM sleep behavior disorder (RBD) associated with dementia with Lewy bodies
Authors: Silber Dr. Michael H 
Published: November 2023

Abstract
The diagnosis of RBD was made, and the patient was treated with clonazepam. With a dose of 0.5 mg before sleep, violent behavior largely ceased. Two years after diagnosis, the patient complained of mild memory problems, and examination showed impaired tests of concentration and learning. He had reduced rapid alternating movements of his tongue and fingers, a positive glabellar tap, bilateral palmomental reflexes, and mild upper limb rigidity. Three years later, a more rapid deterioration of cognitive functions commenced. Neuropsychometric testing confirmed a dementia of subcortical type that progressively worsened over the next 2 years. Dementia with Lewy bodies was diagnosed.
Anterior communicating artery aneurysm paraparesis syndrome
Authors: Wijdicks Dr. Eelco F M 
Published: November 2023

Abstract
Cerebral vasospasm and rebleeding are major causes of additional morbidity in patients who survive aneurysmal subarachnoid hemorrhage. There has been a recent emphasis on early surgical or endovascular intervention in the treatment of ruptured cerebral aneurysms. Albeit unproven, there is a perception that this aggressive approach will reduce early rebleeding. If true, cerebral vasospasm will become one of the major remaining causes of deterioration. Early diagnosis of cerebral vasospasm is important and, in certain patients, may lead to effective treatment and prevent permanent infarction.
Hereditary neuropathy with a liability to pressure palsy (HNPP or tomaculous neuropathy)
Authors: Daube Dr. Jasper R 
Published: November 2023

Abstract
The nerve biopsy specimen showed tomaculous neuropathy (Figure), an autosomal dominant, demyelinating neuropathy with liability to pressure palsy (HNPP) that is easily overlooked if the history of recurrent mononeuropathies and a family history of neuropathy are not elicited. FISH analysis confirmed the diagnosis.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
Authors: Flemming Dr. Kelly D 
Published: November 2023

Abstract
The patient initially received treatment with corticosteroids for presumed acute disseminated encephalomyelitis (ADEM). After treatment was initiated, skin biopsy results became available. Electron microscopy showed thickening of the basal lamina from deposits of granular osmophilic material (GOM), consistent with CADASIL. CADASIL is an inherited condition characterized by migraine, recurrent subcortical strokes, and dementia. The disease leads progressively to a subcortical dementia characterized by frontal lobe signs and memory loss. In addition, pseudobulbar palsy, gait disturbance, pyramidal signs, and urinary incontinence may be present. MRI studies generally show increased T2-signal changes in the deep white matter (especially the periventricular area) and basal ganglia. It has been suggested that another useful diagnostic marker is involvement of the anterior temporal pole seen on MRI. Pathologically, there is evidence of widespread myelin pallor of the white matter and multiple small infarcts in the white matter and basal ganglia, which are generally symmetric. The vessels of the white matter and meninges are thickened, and the media of the vessels is characterized by a smudgy granular material and the loss of smooth muscle cell nuclei. GOM is visible with electron microscopy and is of uncertain significance.
Dural arteriovenous fistula (DAVF) of the left medulla
Authors: Crum Dr. Brian A 
Published: November 2023

Abstract
The diagnosis was suggested in part by finding, on the cervical MRI study, dilated vessels within the upper cervical spinal canal extending from the level of the brainstem to C5 (Figure 2). Selective cerebral angiography identified, at the level of the left pontomedullary junction, a small high-flow DAVF supplied by the left vertebral artery, with left meningeal and left anterior inferior and posterior inferior cerebellar artery trunk feeder vessels and a small contribution through collateral vessels from the left external carotid artery.
Retinocochleocerebral vasculopathy (Susac’s syndrome)
Authors: Petty Dr. George W 
Published: November 2023

Abstract
Retinocochleocerebral vasculopathy (Susac’s syndrome) is an acute or subacute syndrome of encephalopathy, visual loss, and hearing loss caused by multiple arteriolar occlusions in the brain, retina, and cochlea. Young adult women are afflicted more often than men, although patients as old as 51 years have been reported. The incidence is unknown. Although this syndrome is considered rare, it is likely encountered more often than it is recognized. Only a small proportion of the patients have the complete clinical triad at the onset of symptoms, making the initial diagnosis difficult. Patients with “partial” forms of the syndrome have been described (e.g., brain and retinal involvement without hearing loss). In many cases of Susac’s syndrome, the initial diagnosis is multiple sclerosis, cardioembolic stroke, Ménière’s disease, systemic lupus erythematosus, migraine, or schizophrenia.
Chronic immune sensory polyradiculopathy
Authors: Sinnreich Drs. Michael , Dyck P James B 
Published: November 2023

Abstract
Several disorders present with sensory ataxia. Included in the pathogenesis are vitamin deficiencies, inherited degenerative conditions, infectious diseases, toxic substances, and inflammatory immune disorders. Sensory ataxia can be caused by varied involvement of the dorsal columns, dorsal root entry zone, dorsal root ganglia, and distal sensory axons. In our patient, peripheral sensory segments proximal to the dorsal root ganglia were affected, rendering the diagnosis of a peripheral nervous system disorder difficult because nerve conduction and electromyographic studies are normal. Thickened nerve roots seen on MRI and slowing of proprioceptive pathways from the lower extremities were helpful in localizing the lesion. A biopsy specimen from a fascicle of a lumbar nerve root showed edema, a marked decrease in the large-fiber population (with a shift to smaller diameter fibers), endoneurial inflammatory cells, and onion bulb formation (Figure 2). Despite the long duration of the patient’s illness, the response to intravenous immunoglobulin treatment was rapid and substantial. After 16 weeks of first biweekly and then weekly intravenous immunoglobulin therapy, the patient had marked and quantifiable improvement. She could walk unaided and drive her car again, which was impossible before treatment because of proprioceptive impairment of the lower extremities. The acroparesthesias resolved.
Cardioskeletal myopathy associated with lysosome-associated membrane protein-2 (LAMP-2) deficiency
Authors: Selcen Dr. Duygu 
Published: November 2023

Abstract
The differential diagnosis in this case is hypertrophic cardioskeletal myopathy associated with mental retardation. Cardiomyopathies can be classified as hypertrophic, dilated, restrictive, and arrhythmogenic. In several disorders, they can be of more than one type, and in a given patient, they may change from one type to another. Cardiomyopathy can precede the onset of an associated skeletal muscle myopathy in some diseases, including Becker muscular dystrophy, Emery-Dreifuss muscular dystrophy, myotonic dystrophy, myofibrillar myopathy, acid maltase deficiency, and Danon disease. The associated mental retardation narrows the diagnosis to myotonic dystrophy and Danon disease.
Squamous carcinoma involving the perineural sheath of the facial nerve
Authors: Auger Dr. Raymond G 
Published: November 2023

Abstract
The patient presented with a progressive disorder of the facial nerve caused by neoplastic infiltration of its perineural sheath (Figure). Nests of high-grade squamous carcinoma cells were found within the buccal nerve in this case. This case demonstrates the need to consider extracranial involvement of the facial nerve in patients presenting with facial weakness. The site of the lesion causing facial paresis in a patient can be deduced clinically by assessing the presence or absence of lacrimation (greater superficial petrosal nerve), hyperacusis (nerve to the stapedius muscle), and taste (chorda tympani). In this patient, taste was normal, suggesting that the lesion was distal to the junction of the chorda tympani and the main trunk of the facial nerve. The patient volunteered the information that he was able to wiggle his ears bilaterally. This was of localizing significance because the branch to the posterior auricular muscle is the first branch of the facial nerve after it exits the stylomastoid foramen. Therefore, the observation that this muscle was normal despite the severe weakness of the other muscles supplied by the facial nerve provided irrefutable evidence that the lesion was in the extracranial portion of the facial nerve.
Hereditary generalized dystonia and tremor secondary to DYT1 gene mutation
Authors: Adler Drs. Charles H , Muenter Manfred D 
Published: November 2023

Abstract
Patients with childhoodor adolescent-onset dystonia or tremor (or both) often have a hereditary disorder. Our patient’s family history of Parkinson’s disease in the father (not evaluated by us) and dystonia in two of four siblings suggested a possible autosomal dominant disorder. DYT-1 dystonia is the most common cause of hereditary, autosomal dominantly inherited dystonia (Table). It is usually characterized by a generalized dystonia that often begins in the lower limbs and progresses over a few years. In some patients only a focal dystonia, usually of the arm, develops. Facial or cervical dystonia at disease onset is extremely rare, and relatively few patients ever have facial dystonia.
Adult acid maltase deficiency
Authors: Bosch Dr. E Peter 
Published: November 2023

Abstract
Type II glycogenosis (glycogen storage disease[GSD-II]) is an autosomal recessive lysosomal storage disorder caused by partial or complete deficiency of acid-glucosidase activity (GAA), or acid maltase. This enzyme defect results in the accumulation of lysosomal glycogen in many tissues, with cardiac and skeletal muscle affected most severely (Figure). The estimated prevalence of GSD-II is low (1 in 40,000 to 100,000 newborns). Three forms of the disease have been recognized. The infantile form (or Pompe’s disease) results from complete or near complete deficiency of GAA and presents within the first 3 months of life with hypotonia, progressive weakness, macroglossia, hypertrophic cardiomyopathy, and hepatomegaly. Its course is invariably fatal, and the child dies of cardiorespiratory failure before age 2 years. The juvenile form presents in the first decade with proximal weakness and mild or no cardiac involvement and leads to respiratory failure in the second or third decade of life. The adult-onset form begins in the third to seventh decade (range, 1865 years). Most patients present with pelvic girdle weakness affecting predominantly the gluteal muscles and thigh adductors, followed by proximal arm weakness. Trunk extensors and abdominal muscles are frequently affected.
Paraneoplastic optic neuritis, polyradiculopathy, and ataxia for which collapsin response mediator protein-5 (CRMP-5) autoantibody serves as a serologic marker
Authors: Cross Dr. Shelley A 
Published: November 2023

Abstract
Paraneoplastic antibody screening showed CRMP-5 autoantibody at a titer of 1/3,840. The glutamic acid decarboxylase (GAD)65 antibody was also positive. The carcinoma-associated retinopathy antibody was negative. Lumbar puncture was not performed. Mediastinoscopy with biopsy revealed small cell lung cancer. This case represents a paraneoplastic subacute encephalomyeloradiculoneuropathy with optic neuritis accompanied by vitreous inflammatory cells (and sometimes by retinitis and intrathecal inflammatory cells) associated with small cell carcinoma. CRMP-5 is its serologic marker. Although CRMP-5-IgG is a marker of autoimmunity initiated by either small cell carcinoma or thymoma, we have not encountered a patient with optic neuritis, retinitis, and thymoma. Cases similar to the one presented here have been reported since 1986, but only recently has the syndrome been fully described, the antibody clearly identified, and the ophthalmologic and neurologic features described in detail.
Molecular Control of Looping
Authors: Kirby Margaret Loewy 
Published: November 2023

Abstract
Even though the right–left axis of the body is established during early gastrulation, cardiac looping is the first visible sign of right–left ASYMMETRY during embryonic development. In vertebrates the heart always loops to the right. Looping directionality correlates with asymmetric expression of several molecular markers, but how these molecules are translated into biomechanical forces that drive looping remains obscure. The looping process itself is important to bring the initially sequentially ordered regions of the heart tube with serial circulation into the correct conformation for chamber specification and septation to create two parallel circulations. During the looping process, the initially midline heart tube undergoes a coordinated progression of bending, rotation, and torsion (Fig. 7.1). The initial steps result in a C-shaped loop whose convexity is directed toward the right side of the body (DeHaan, 1965; Garcia-Pelaez and Arteage, 1993; Icardo, 1996; Stalsberg, 1970). The heart tube goes through two major alterations in shape to achieve the C-shaped loop: ventral bending and rightward rotation (Manner, 2000). However, because the dorsal mesocardium is still intact during C-looping (Fig. 7.1A), little movement is possible except by shape changes at the ventral midline or outer curvature which becomes the right border during C-looping. Thus, the ventral bending is actually ballooning of the newly formed ventral wall rather than bending of the whole tube. In the second part of this movement, the heart tube rotates around its craniocaudal axis, bringing the ventral midline (outer curvature) to the right and the initially left surface of the myocardial tube to form the ventral surface of the C-shaped loop (Manner, 2000). An inner curvature is formed from the dorsal midline on the left side (Fig. 7.1). This is referred to as dextral or D-looping.
Toward an Accounting for Sustainability
Authors: Lawrence Stewart 
Published: November 2023

Abstract
Accounting is a ubiquitous social practice—it touches each of us, every day, in our personal and professional lives. No matter what occupation we take up, there will be no escaping financial responsibility and accountability. Even outside of paid employment, individuals need to be financially responsible and accountable: in families, in social clubs, and in any kind of group activity where resources are acquired and consumed. For centuries, the practice of accounting was limited to providing information relating to the stewardship of financial resources.
Profits of U.S. Defense Contractors
Authors: Higgs Robert , Trevino Ruben 
Published: November 2023

Abstract
Do big defense contractors earn greater returns than other companies? The question has long been controversial, and despite numerous studies, the issue has remained unsettled (Cansler, 1989, pp. 253-4; Rogerson, 1989, pp. 1290-1). Most of the studies have been made by interested parties—the armed services or the Department of Defense. Some of the studies fail to meet professional standards; others are patently tendentious. But even the studies published in professional journals disagree widely.2 The disagreement is hardly surprising, because the various studies are not comparable. They have considered different groups of firms and different periods, and they have employed different measures of profitability.
Institutional Strengths and Limits
Authors: Heinzerling Lisa , Tushnet Mark V 
Published: November 2023

Abstract
In chapter 2, we considered the advantages and disadvantages of private markets in addressing the problem of risk. In chapter 4, through the work of Lon Fuller, we began to consider the strengths and limits of courts as decision- making institutions. We continue and expand on those discussions here. Most signfically we add administrative agencies to the mix of institutions that can be called on to deal with risk. We also examine sociological and historical treatments of the transformation of social problems into legal problems.
Information Provision
Authors: Heinzerling Lisa , Tushnet Mark V 
Published: November 2023

Abstract
We’ve already seen how disclosure (or nondisclosure) of information about risks to human health and life affects the regulation of risk. The economic theory of compensating wage differentials for risky jobs, which we explored in chapter 2, depends on workers knowing what the risks of their jobs are. If workers—or others exposed to risks—are unaware of the risks they face, it is much harder to argue that contractual arrangements should dictate whether the risk should be reduced. In such situations, the government might be justified in requiring disclosure of risks so that contracting parties can decide what actions, if any, to take, based on the information. Information also played a central role in our discussion of institutional limits in chapter 5. There, we saw that gaps in information might lead to a failure to name, blame, or claim—in other words, to identify and to act on one’s legal rights.
Introduction
Authors: Hasbrouck Joel 
Published: November 2023

Abstract
Market microstructure is the study of the trading mechanisms used for financial securities. There is no “microstructure manifesto,” and historical antecedents to the fleld can probably be found going back to the beginning of written language, but at some point, the fleld acquired a distinct identity. As good a starting point as any is the coinage of the term market microstructure in the paper of the same title by Garman (1976): We depart from the usual approaches of the theory of exchange by (1) making the assumption of asynchronous, temporally discrete market activities on the part of market agents and (2) adopt-ing a viewpoint which treats the temporal microstructure, i.e., moment-to-moment aggregate exchange behavior, as an important descriptive aspect of such markets.
Trading Mechanisms
Authors: Hasbrouck Joel 
Published: November 2023

Abstract
This chapter surveys typical trading arrangements and establishes an institutional context for the statistical and economic models to follow. This book focuses on continuous security markets. Whatever their original mechanisms, many and the most visible of these markets presently feature an electronic limit order book. The limit order market, then, is the starting point for the survey. This is probably the most important mechanism, but there are usually at least several alternative paths to accomplishing a trade for any given security. Most security markets are actually hybrids, involving dealers, clearings, one- and two-sided auctions, and bilateral bargaining, all of which are also discussed. The survey emphasizes general features and is not specific to particular securities or a particular country.
Sequential Trade Models
Authors: Hasbrouck Joel 
Published: November 2023

Abstract
In the Roll framework, everyone possesses the same information. New public information can cause the quotes to change, but trades have no impact. Because trades are not informative, there’s no particular need for them to be reported and disseminated in a timely fashion. Trading is not a strategic problem because agents always face the same spread. The costs reflected in dealers’ spreads reflect only expenditures for computers, communications, salaries, business fees, and so on, the sort of expenses that a wholesaler of any good or service might incur.
A Generalized Roll Model
Authors: Hasbrouck Joel 
Published: November 2023

Abstract
Following the economic analysis, we now turn to empirical examina-tion of asymmetric information by developing a generalization of the Roll model. One sensible first step is to allow the efficient price to be par-tially driven by the trade direction indicator variable. A number of models along these lines have been proposed. The developments in this chapter are compatible with and can be viewed as special cases of most of these models. The phrase “generalized Roll model” is not in common use. It is used here to emphasize the roots of the present developments in the Roll model.
Multivariate Linear Microstructure Models
Authors: Hasbrouck Joel 
Published: November 2023

Abstract
Although the trade price is usually the single most important variable in a microstructure analysis, other variables can help explain its dynamics. These include orders, trades, prices of the same security in other markets, prices of related securities, and so on. Inclusion of these variables can enhance the forecasting power of a model. Under certain assumptions, the multivariate models support an attribution of this forecasting power, essentially identifying price changes with particular sources of informa-tion.
Multiple Securities and Multiple Prices
Authors: Hasbrouck Joel 
Published: November 2023

Abstract
Markets for different securities often interact. The standard theory of portfolio optimization is built on the assumption that securities share common-factor value determinants. The value of a derivative securities is generally linked to its underlying asset by arbitrage possibilities. The trading process may reflect these long-term interactions and may exhibit other dependencies as well. Trades may be correlated for non-informational reasons. A trader buying into an index, for example, will place orders in many or all stocks of the index. A trade in stock A, for example, that conveys information about a factor that is also a value determinant of stock B may move the price of B. Arbitrage, too, usually requires trading.
Depth
Authors: Hasbrouck Joel 
Published: November 2023

Abstract
In addition to best bid and offers, most modern financial markets present their customers with full price/quantity schedules, essentially supply and demand curves for instantaneous trade. This chapter explores the determinants of these schedules and what might be learned from their analysis. The stylized empirical fact is that the supply and demand schedules appear to be too steep. As an illustration, recall that in the basic sequen-tial trade model, the price at which an order is executed is equal to the expectation of the security value (conditional on the trade). Because trade prices are equal to expectations, trade price changes mirror the revisions in expectations. In a sequence of three buy orders, for example, the price at which the last order is executed is the expected security value con-ditional on all three orders.
Trading Costs: Retrospective and Comparative
Authors: Hasbrouck Joel 
Published: November 2023

Abstract
This chapter and the next address trading costs and their relation to strat-egy and institutional features. Economic logic suggests a definite ordering for these topics. Most economic data are the outcomes of strategies that are, at least from agents’ exante perspectives, optimal. Thus arises the ideal paradigm of first defining agents’ objectives, then enumerating the feasible strategies, mapping these strategies to costs, determining optimal (cost minimizing) strategies, and finally performing econometric analy-sis of observed outcomes taking into account their dependence on all prior stages of the process. The exposition will follow this sequence but is heavily shaped by the limitations of the data. The present chapter describes the microstructure literature that aims primarily at comparisons of alternative trading venues.