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1

37 WEEKS: One family's journey into cerebral palsy. [Place of publication not identified]: GREEN IVY, 2015.

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2

Bratt, Berneen. No time for jello: One family's experience with the Doman-Delacato Patterning Program. Cambridge, MA: Brookline Books, 1989.

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3

Right brain education: Changing the world, one heart at a time. Bozeman, Mont: The Soul Learning Company, 2009.

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4

Franco, Leporé, Ptito Maurice, and Jasper Herbert H. 1906-, eds. Two hemispheres, one brain: Functions of the corpus callosum : proceedings of the Sixth International Symposium of the Centre de recherche en sciences neurologiques of the Université de Montréal, held in Montréal, Canada, May 16-18, 1984. New York: Liss, 1986.

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5

Romkema, Craig. Embracing the sky: Poems beyond disability. London: Jessica Kingsley Publishers, 2002.

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6

Standards for adjudicating claims presented by veterans suffering from hepatitis C, cerebral malaria, and Persian Gulf illnesses: Hearing before the Subcommittee on Benefits of the Committee on Veterans' Affairs, House of Representatives, One Hundred Fifth Congress, second session, July 16, 1998. Washington: U.S. G.P.O., 1999.

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7

Matsudo-Kiliani, Susanne, and Yukio Matsudo. Cambia le Tue Onde Cerebrali - Cambia il Tuo Karma: Buddismo Nichiren 3. 1. Independently Published, 2018.

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8

I Have Cerebral Palsy (One World). Franklin Watts, 1992.

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9

Z. Visions: A Cerebral Chapbook. Ocean Deep Publishing, 2022.

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10

LEPORE, F. Lepore Two Hemispheres - One Brain - Functions O F the Corpus Callosum. John Wiley & Sons Inc, 1986.

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11

Hasan, David. The Natural History of Cerebral Aneurysms. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0109.

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Subarachnoid hemorrhage (SAH) secondary to rupture of cerebral aneurysms represents a relatively small fraction of strokes (5%) but morbidity and mortality associated with aneurysm rupture remain very high despite advances in the treatment of aneurysmal SAH. Cerebral vasospasm (CV) is the leading cause of delayed morbidity and mortality following aneurysmal subarachnoid hemorrhage, as well as delayed neurological dysfunction 1 to 2 weeks after rupture. Endothelial dysfunction is one of the primary contributing factors to CV following aneurysmal SAH, and this is associated with alterations in intracellular adhesion molecule-1 (ICAM-1), matrix metalloproteinases (MM), and the blood-brain barrier[p63].
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12

Theologis, Tim, and J. M. H. Paterson. Lower limb management in cerebral palsy. Oxford University Press, 2011. http://dx.doi.org/10.1093/med/9780199550647.003.013004.

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♦ All patients with cerebral palsy have lower limb involvement♦ Determination of the pattern of neurological involvement and the degree of selective muscle control is important when considering orthopaedic intervention♦ The neurological insult is non-progressive but the musculoskeletal effects do progress.♦ Specific treatment aims must be identified and realistic targets defined♦ The appropriate treatment must be selected for the child’s age and stage of development♦ It is important to differentiate between dynamic and fixed contractures♦ Surgery is only one of the management options and is rarely successful if considered in isolation.
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13

Mustafa, Nujeen, and Christina Lamb. Nujeen: One Girl's Journey from War-Torn Syria in a Wheelchair. HarperCollins Publishers, 2016.

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14

Nujeen: One Girl's Incredible Journey from War-Torn Syria in a Wheelchair. HarperCollins Publishers Limited, 2016.

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15

Nujeen: One Girl's Incredible Journey from War-Torn Syria in a Wheelchair. HarperCollins Publishers, 2016.

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16

Nujeen: One Girl's Incredible Journey from War-Torn Syria in a Wheelchair. HarperCollins Publishers Limited, 2016.

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17

Pritts, Robin. From CP to CPA: One Man's Triumph Over the Disability of Cerebral Palsy. Weyant Press, Inc., 2002.

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18

Pritts, Robin E. From CP to Cpa: One Mans Triumph over the Disability of Cerebral Palsy. Hefernan Publishing Group, 2003.

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19

Mustafa, Nujeen, and Christina Lamb. Nujeen: One Girl's Incredible Journey from War-Torn Syria in a Wheelchair. Harper Wave, 2016.

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20

Johnson, Jennifer. Heartland weddings: Two contemporary romances under one cover. 2013.

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21

Fortaleciendo La Facultad Cerebral Del Bebe: Hundreds of One-minute Brain Stimulating Activities for Baby's First Years. Beta Center, 2007.

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22

Brown, Keah. Pretty One: On Life, Pop Culture, Disability, and Other Reasons to Fall in Love with Me. Atria Books, 2019.

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23

Pretty One: On Life, Pop Culture, Disability, and Other Reasons to Fall in Love with Me. Atria Books, 2019.

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24

Brown, Keah. The Pretty One: On Life, Pop Culture, Disability, and Other Reasons to Fall in Love with Me. Simon & Schuster Audio and Blackstone Publishing, 2019.

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25

Paddock, Bonner. One more step: My story of living with cerebral palsy, climbing Kilimanjaro, and surviving the hardest race on earth. 2015.

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26

Jill Came Tumbling After : One Caregiver's Journey to Acceptance. Ritamelia Pr, 1996.

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27

Kempe's Operative Neurosurgery. Volume One and Two : Cranial, Cerebral, and Intracranial Vascular Disease / Posterior Fossa, Spinal and Peripheral Nerve. Springer, 2003.

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28

Attems, Johannes, and Kurt A. Jellinger. Neuropathology. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199644957.003.0006.

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This chapter describes the main neuropathological features of the most common age associated neurodegenerative diseases including Alzheimer's disease, Parkinson's disease and dementia with Lewy bodies as well as other less frequent ones such as multiple system atrophy, Pick's disease, corticobasal degeneration, progressive supranuclear palsy, argyrophilic grain disease, neurofibrillary tangle dominant dementia, frontotemporal lobar degeneration with TDP-43 pathology and Huntington's disease. Likewise cerebral amyloid angiopathy, hippocampal sclerosis, vascular dementia and prion diseases are described. A main aim of this chapter is to assist the reader in interpreting neuropathological reports, hence criteria for the neuropathological classifications of the major diseases are provided. One section covers general considerations on neurodegeneration and basic pathophysiological mechanisms of tau, amyloid-β, α-synuclein, TDP-43 and prions are briefly described in the sections on the respective diseases. Finally, one section is dedicated to cerebral multimorbidity and we give a view on currently emerging neuropathological methods.
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29

Schechter, Elizabeth. The Unity Puzzle. Oxford University Press, 2018. http://dx.doi.org/10.1093/oso/9780198809654.003.0001.

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This chapter introduces the major philosophical debate about the split-brain phenomenon. Split-brain surgery severs the major white matter fiber tract connecting the two cerebral hemispheres. A number of individuals who underwent this surgery later agreed to act as participants in experiments designed to reveal its psychobehavioral consequences. The basic finding is that, after they are surgically divided in this way, the two hemispheres cannot interact in all the ways they once could: indeed, split-brain subjects sometimes give the impression of having two minds and spheres of consciousness, one associated with each hemisphere. A split-brain subject nonetheless seems to be one of us, at the end of the day. The aim of the book is to reconcile these apparently opposing intuitions by explaining how a split-brain person could have multiple minds.
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30

Xu, Kui, Joseph C. LaManna, and Michelle A. Puchowicz. Ketogenic Diet, Aging, and Neurodegeneration. Edited by Detlev Boison. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190497996.003.0024.

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The brain is normally completely dependent on glucose, but is capable of using ketones as an alternate energy source, as occurs with prolonged starvation or chronic feeding of a ketogenic diet. Research has shown that ketosis is neuroprotective against ischemic insults in rodents. This review focuses on investigating the mechanistic links to neuroprotection by ketosis in the aged. Recovery from stroke and other pathophysiological conditions in the aged is challenging. Cerebral metabolic rate for glucose, cerebral blood flow, and the defenses against oxidative stress are known to decline with age, suggesting dysfunction of the neurovascular unit. One mechanism of neuroprotection by ketosis involves succinate-induced stabilization of hypoxic inducible factor-1alpha (HIF1α‎) and its downstream effects on intermediary metabolism. The chapter hypothesizes that ketone bodies play a role in the restoration of energy balance (stabilization of ATP supply) and act as signaling molecules through the up-regulation of salvation pathways targeted by HIF1α‎.
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31

Khouri, George, Shelly Ozark, and Bruce Ovbiagele. Common Risk factors for Stroke and Medical Prevention Therapies. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0103.

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Stroke from thrombosis or emboli in cerebral vessels or hemorrhage is one of the most commonly encountered and most devastating neurological diseases. Rapid loss of function occurs due to an interruption of blood supply to the brain, leading to tissue ischemia and cell death. The risk of both ischemic and hemorrhagic stroke doubles for each successive decade after age 55, which is likely independent of other risk factors such as diabetes, hypertension, and hyperlipidemia. Lifestyle modifications, antiplatelet therapy and control of hyperlipidemia and hypertension are the mainstays of prevention.
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32

Alawi, Aws, Michael Reznik, and Jan Claassen. Neurophysiologic Monitoring and Neuroprotection. Edited by David L. Reich, Stephan Mayer, and Suzan Uysal. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780190280253.003.0005.

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One of the main goals of monitoring neurologically ill patients is detection of secondary brain injury early enough to intervene to prevent permanent damage. In some patients with impaired levels of consciousness and those who require sedation, monitoring various brain physiologic parameters by invasive and noninvasive means has become an essential tool in the care of critically ill patients. Integration of multiple physiological parameters provides a more comprehensive physiological assessment of the injured brain and allows real-time, early detection of secondary cerebral injury and intervention to prevent permanent damage. Importantly, these modalities should be interpreted collectively and not in isolation in order to manage acute brain injuries, which are often complex and dynamic at the same time.
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33

Robert, Philippe, Elsa Leone, Hélène Amieva, and David Renaud. Managing behavioural and psychological symptoms in Alzheimer’s disease. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780198779803.003.0009.

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This chapter focuses on the behavioural and psychological symptoms of Alzheimer's disease and the different approaches clinicians can take in their treatment of the condition. The behavioural and psychological symptoms are defined as primary manifestations of cerebral dysfunction, and appear specifically as a result of damage to a system or circuit such as the limbic system or the cortico-subcortical circuits. During the progression of Alzheimer’s disease, the presence of at least one BPSD is common and can vary, depending especially on the severity of the dementia-related syndrome at the time of diagnosis. Management of BPSD should preferentially be based on non-pharmacologic approaches first. Pharmacologic treatments should constitute second line treatment and are to be prescribed only after assessment of the individual risk:benefit ratio.
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34

Osborn, Irene P., and Liang Huang. Interventional Neuroradiology. Edited by David E. Traul and Irene P. Osborn. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190850036.003.0008.

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Interventional neuroradiology is one of the rapidly evolving specialties in medicine that provides minimally invasive and percutaneous treatment of diseases of the brain and spine. The new paradigm for management of a cerebral aneurysm begins with a detailed study of the anatomy and architecture of the lesion. Following this, decisions are made and devices are employed to treat the lesion in the event of a rupture or to prevent a future rupture. The anesthetic implications are different from intracranial clipping and require a different set of priorities. The procedure is performed in the radiology suite with unique concerns such as patient access, lack of movement, and radiation exposure. This discussion will focus on the safe management of endovascular coiling procedures.
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35

Leys, Didier, Charlotte Cordonnier, and Valeria Caso. Stroke. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199687039.003.0067_update_002.

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Stroke is a major public health issue. Many are treatable in the acute stage, provided patients are admitted soon enough. The overall incidence of stroke in Western countries is approximately 2400 per year per million inhabitants, and 80% are due to cerebral ischaemia. The prevalence is approximately 12 000 per million inhabitants. Stroke is associated with increased long-term mortality, handicap, cognitive and behavioural impairments, recurrence, and an increased risk of other types of vascular events. It is of major interest to take the heterogeneity of stroke into account, because of differences in the acute management, secondary prevention, and outcomes, according to the subtype and cause of stroke. In all types of stroke, early epileptic seizures, delirium, increased intracranial pressure, and non-specific complications are frequent. In ischaemic strokes, specific complications, such as malignant infarcts, spontaneous haemorrhagic transformation, early recurrence, and a new ischaemic event in another vascular territory, are frequent. In haemorrhagic strokes, the major complication is the subsequent increased volume of bleeding. There is strong evidence that stroke patients should be treated in dedicated stroke units; each time 24 patients are treated in a stroke unit, instead of a conventional ward, one death and one dependence are prevented. This effect does not depend on age, severity, and the stroke subtype. For this reason, stroke unit care is the cornerstone of the treatment of stroke, aiming at the detection and management of life-threatening emergencies, stabilization of most physiological parameters, and prevention of early complications. In ischaemic strokes, besides this general management, specific therapies include intravenous recombinant tissue plasminogen activator, given as soon as possible and before 4.5 hours, mechanical thrombectomy in case of proximal occlusion (middle cerebral artery, intracranial internal carotid artery, basilar artery), on top of thrombolysis in the absence of contraindication or alone otherwise, aspirin 300 mg, immediately or after 24 hours in case of thrombolysis, and, in a few patients, decompressive surgery. In intracerebral haemorrhages, blood pressure lowering and haemostatic therapy, when needed, are the two targets, while surgery does not seem effective to reduce death and disability.
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36

Auer, Peter, and Ina Hörmeyer. Achieving Intersubjectivity in Augmentative and Alternative Communication (AAC). Oxford University Press, 2017. http://dx.doi.org/10.1093/acprof:oso/9780190210465.003.0013.

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This paper investigates communication, including computer-based speech aids by people with severe cerebral palsy—namely Augmented and Alternative Communication, AAC. The reduced bodily capacities and the “uncontrolled bodies” of CP sufferers make bodily synchronization with their partners a considerable challenge. What is more, the electronic speech aid not only produces a disembodied language (synthetic speech), but also has a massive impact on the mutual corporeal attunement of the participants. It will be shown that these detrimental effects of AAC can lead to a breakdown in temporal, sequential and topical structure, and to interactional failure and lack of understanding. However, there are ways to overcome these risks—for example, a “moderator” who channels and controls co-participants’ activities despite the Augmented/Alternative Communicator’s focus on the machine, even during the production of a complex utterance. Thus the machine can be “embodied,” and the interaction can—despite CP—become an “intercorporeal” one.
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37

Epstein, Charles M. TMS stimulation coils. Edited by Charles M. Epstein, Eric M. Wassermann, and Ulf Ziemann. Oxford University Press, 2012. http://dx.doi.org/10.1093/oxfordhb/9780198568926.013.0004.

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The simplest transcranial magnetic stimulation (TMS) coil is a circular one. The induced current is maximum near the outer edge of the coil while the magnetic field is the maximum under the center of the coil. TMS coils have good penetration to the cerebral cortex. They are commonly placed at the cranial vertex, where they can stimulate both hemispheres simultaneously. The main drawback of circular coils is their lack of focality. Several complex designs for multiloop coils have been proposed to increase the focality or improve the penetration to deep brain structures. This article describes factors of TMS coil design such as mechanical forces and coil lead wires, cooling systems, materials of construction of coil windings, etc. To reduce the risk of lethal electrical shock the entire high-voltage power system, including the lead wires and stimulation coil, must be isolated from earth ground.
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38

Martyna, Majok, Arbery Will, Younger Kelly, Winkler Leah Nanako, and Fox Skylar, eds. ET-Y-MOL-O-GY. Concord Theatricals, 2015.

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39

Steinbock, Bonnie. Opting for Twins in In Vitro Fertilization. Edited by Leslie Francis. Oxford University Press, 2016. http://dx.doi.org/10.1093/oxfordhb/9780199981878.013.27.

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Multiple births are an unfortunate consequence of assisted reproductive technology, causing risks to both fetuses and pregnant women. The central ethical issue raised by multiple pregnancy is the conflict between the fertility patient’s desire to get pregnant and the increased risks to offspring. Although extreme cases in which many embryos are transferred to the woman’s uterus are very rare—and represent negligence—twin pregnancies are still common. Many women undergoing fertility treatment reportedly express a preference for twin pregnancies to reduce the costs and risks of the procedure while increasing their opportunities for having more than one child. However, risks to the offspring are significant and underappreciated, including prematurity, low birth weight, cerebral palsy, and learning disabilities. Even though many multiple pregnancies result in good outcomes, the ethical question is whether the risks are justifiable in order to improve the chances of pregnancy when transfer of a singleton embryo is an available alternative.
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40

Pereira, Jose, and Jennifer Brodeur. The management of bleeding in palliative care. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199656097.003.0087.

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Bleeding is one of the more distressing symptoms experienced by patients with advanced life-threatening illnesses. The prevalence and incidence of bleeding in these patients vary depending on the disease and the illness trajectory. The causes of bleeding in patients with advanced disease are varied and sometimes several aetiologies or aggravating factors occur simultaneously in any given patient. The clinical presentation may be visible, as in haemoptysis or hematemesis, or invisible, as in cerebral haemorrhaging, and volumes may vary, from low-grade oozing to massive and catastrophic haemorrhaging. Catastrophic, terminal haemorrhaging warrants special attention because of its dramatic clinical presentation and the profound distress it causes to patients, families, and caregivers. A number of treatment modalities are available and these can be divided into (a) general measures, (b) local measures, and (c) systemic measures. Unfortunately studies in the palliative care setting comparing various modalities and approaches are generally lacking and guidelines are largely based on case reports and expert opinion.
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41

Guillery, Ray. The role of the brain. Oxford University Press, 2017. http://dx.doi.org/10.1093/oso/9780198806738.003.0001.

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This chapter introduces two interpretations of how we know about the world. One, the standard, sensory-to-motor view, is that physical actions for sounds, lights, tastes, smells, and so on act on our sense organs to produce messages that are sent through the nervous system to the cerebral cortex, where the relevant structures of the world can be recognized and appropriate motor actions can be initiated. The other is an interactive sensorimotor view where the nervous system records our interactions with the world, abstracting our knowledge about the world from these interactions. These two opposing views have rarely been considered in terms of specific neural pathways or the messages that they carry; that is the plan for this book. Each view leads to different sets of interpretations of experiments and to different sets of research proposals. The final part of the chapter explores a well-studied and widely taught clinical condition that illustrates the confusions that can arise when the dual meaning of the driver messages to the thalamus is not recognized.
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42

Schechter, Elizabeth. Self-Consciousness and "Split" Brains. Oxford University Press, 2018. http://dx.doi.org/10.1093/oso/9780198809654.001.0001.

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The largest fiber tract in the human brain is the corpus callosum, which connects the two cerebral hemispheres. A number of surgeries severing this structure were performed on adults in the United States in the second half of the twentieth century. After they are surgically separated from each other in this way, a “split-brain” subject’s hemispheres begin to operate unusually independently of each other in the realms of perception, cognition, and the control of action—almost as if each had a mind of its own. But can a mere hemisphere really see? Speak? Feel? Know what it has done? The split-brain cases raise questions of psychological identity: How many subjects of experience are there within a split-brain subject? How many persons? How many minds? Under experimental conditions, split-brain subjects often act as though they were animated by two distinct conscious beings, evoking the duality intuition. On the other hand, a split-brain subject seems like one of us—not like two of us sharing one body. Split-brain subjects thus also evoke the unity intuition.This book is devoted to reconciling these two apparently opposing intuitions. The key to doing so are facts about the way self-consciousness operates in split-brain subjects. A split-brain subject is composed of two conscious psychological beings that fail to recognize each other’s existence and indeed cannot distinguish themselves from each other. Instead, each must first-personally identify with the split-brain subject as a whole, and in so doing, the two make themselves into one person.
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43

Carlucci, Annalisa, and Paolo Navalesi. Weaning failure in critical illness. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199600830.003.0103.

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Weaning failure has been defined as failure to discontinue mechanical ventilation, as assessed by the spontaneous breathing trial, or need for re-intubation after extubation, so-called extubation failure. Both events represent major clinical and economic burdens, and are associated with high morbidity and mortality. The most important mechanism leading to discontinuation failure is an unfavourable balance between respiratory muscle capacity and the load they must face. Beyond specific diseases leading to loss of muscle force-generating capacity, other factors may impair respiratory muscle function, including prolonged mechanical ventilation, sedation, and ICU-acquired neuromuscular dysfunction, potentially consequent to multiple factors. The load depends on the mechanical properties of the respiratory system. An increased load is consequent to any condition leading to increased resistance, reduced compliance, and/or occurrence of intrinsic positive-end-expiratory pressure. Noteworthy, the load can significantly increase throughout the spontaneous breathing trial. Cardiac, cerebral, and neuropsychiatric disorders are also causes of discontinuation failure. Extubation failure may depend, on the one hand, on a deteriorated force-load balance occurring after removal of the endotracheal tube and, on the other hand, on specific problems. Careful patient evaluation, avoidance and treatment of all the potential determinants of failure are crucial to achieve successful discontinuation and extubation.
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44

Guillery, Ray. The Brain as a Tool. Oxford University Press, 2017. http://dx.doi.org/10.1093/oso/9780198806738.001.0001.

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We don’t perceive the world and then react to it. We learn to know it from our interactions with it. All inputs that reach the cerebral cortex about events in the brain, the body, or the world bring two messages: one is about these events, the other, travelling along a branch of that input, is an instruction already on its way to execution. This second message, not a part of standard textbook teaching, allows us to anticipate our actions, distinguishing them from the actions of others, and thus providing a clear sense of self. The mammalian brain has a hierarchy of cortical areas, where higher areas monitor actions of lower areas, and each area can modify actions to be executed by the phylogenetically older brain parts. Brains of our premammalian ancestors lacked this hierarchy, but their descendants are still strikingly capable of movement control: frogs can catch flies. The cortical hierarchy itself appears to establish and increase, from lower to higher levels, our conscious access to events. This book explores the neural connections that provide us with a sense of self and generate our conscious experiences. It reveals how much yet needs to be learnt about the relevant neural pathways.
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45

Paech, Michael J., and Patchareya Nivatpumin. Postdural puncture headache. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780198713333.003.0027.

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Postdural puncture headache (PDPH) may follow either deliberate or unintentional (accidental) penetration of the interdigitating meninges, the dura and arachnoid mater. It is one of the most common and clinically important complications of regional anaesthesia and analgesia in the obstetric population. The headache develops as a consequence of cerebrospinal fluid loss, low intracranial pressure and cerebrovascular changes in the upright position and can prove debilitating. The diagnosis is clinical, making thorough assessment and regular review all the more important, to revise treatment plans, exclude rare serious pathology such as subdural haematoma, and avoid misdiagnosis. This chapter reviews the pathophysiology, incidence, risk factors (needle, technical and patient related), features, natural history, diagnosis, and management of PDPH. High level evidence supports prevention by using small gauge, non-cutting spinal needles, but other preventative strategies against either unintentional dural puncture or PDPH are poorly supported. The absent or poor efficacy of measures such as bed rest, hydration, cerebral vasoconstrictor therapy, epidural or intrathecal saline injection, intrathecal catheter placement or prophylactic epidural blood patch, is noted. Validation of better evidence supporting epidural morphine or intravenous cosyntropin is required. Symptomatic treatment of PDPH is also unreliable. Very limited evidence that requires substantiation supports a modest benefit from caffeine, gabapentinoids or intravenous hydrocortisone. The intervention of epidural blood patch is highly likely to relieve post-spinal PDPH, but only completely resolves epidural needle-induced PDPH in 30–50% of cases. Much detail about EBP remains undetermined, but delayed intervention and injection of approximately 20 mL of autologous blood appear appropriate.
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46

Leys, Didier, Charlotte Cordonnier, and Valeria Caso. Stroke. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199687039.003.0067.

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Stroke is a major public health issue. Many are treatable in the acute stage, provided patients are admitted soon enough. The overall incidence of stroke in Western countries is approximately 2400 per year per million inhabitants, and 80% are due to cerebral ischaemia. The prevalence is approximately 12 000 per million inhabitants. Stroke is associated with increased long-term mortality, handicap, cognitive and behavioural impairments, recurrence, and an increased risk of other types of vascular events. It is of major interest to take the heterogeneity of stroke into account, because of differences in the acute management, secondary prevention, and outcomes, according to the subtype and cause of stroke. In all types of stroke, early epileptic seizures, delirium, increased intracranial pressure, and non-specific complications are frequent. In ischaemic strokes, specific complications, such as malignant infarcts, spontaneous haemorrhagic transformation, early recurrence, and a new ischaemic event in another vascular territory, are frequent. In haemorrhagic strokes, the major complication is the subsequent increased volume of bleeding. There is strong evidence that stroke patients should be treated in dedicated stroke units; each time 24 patients are treated in a stroke unit, instead of a conventional ward, one death and one dependence are prevented. This effect does not depend on age, severity, and the stroke subtype. For this reason, stroke unit care is the cornerstone of the treatment of stroke, aiming at the detection and management of life-threatening emergencies, stabilization of most physiological parameters, and prevention of early complications. In ischaemic strokes, besides this general management, specific therapies include intravenous recombinant tissue plasminogen activator, given as soon as possible and before 4.5 hours, otherwise aspirin 300 mg, immediately or after 24 hours in case of thrombolysis, and, in a few patients, decompressive surgery. In intracerebral haemorrhages, blood pressure lowering and haemostatic therapy, when needed, are the two targets, but surgery does not seem effective to reduce death and disability.
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47

Leys, Didier, Charlotte Cordonnier, and Valeria Caso. Stroke. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199687039.003.0067_update_001.

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Stroke is a major public health issue. Many are treatable in the acute stage, provided patients are admitted soon enough. The overall incidence of stroke in Western countries is approximately 2400 per year per million inhabitants, and 80% are due to cerebral ischaemia. The prevalence is approximately 12 000 per million inhabitants. Stroke is associated with increased long-term mortality, handicap, cognitive and behavioural impairments, recurrence, and an increased risk of other types of vascular events. It is of major interest to take the heterogeneity of stroke into account, because of differences in the acute management, secondary prevention, and outcomes, according to the subtype and cause of stroke. In all types of stroke, early epileptic seizures, delirium, increased intracranial pressure, and non-specific complications are frequent. In ischaemic strokes, specific complications, such as malignant infarcts, spontaneous haemorrhagic transformation, early recurrence, and a new ischaemic event in another vascular territory, are frequent. In haemorrhagic strokes, the major complication is the subsequent increased volume of bleeding. There is strong evidence that stroke patients should be treated in dedicated stroke units; each time 24 patients are treated in a stroke unit, instead of a conventional ward, one death and one dependence are prevented. This effect does not depend on age, severity, and the stroke subtype. For this reason, stroke unit care is the cornerstone of the treatment of stroke, aiming at the detection and management of life-threatening emergencies, stabilization of most physiological parameters, and prevention of early complications. In ischaemic strokes, besides this general management, specific therapies include intravenous recombinant tissue plasminogen activator, given as soon as possible and before 4.5 hours, otherwise aspirin 300 mg, immediately or after 24 hours in case of thrombolysis, and, in a few patients, decompressive surgery. In intracerebral haemorrhages, blood pressure lowering and haemostatic therapy, when needed, are the two targets, but surgery does not seem effective to reduce death and disability.
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48

Fletcher, Nicholas. Movement disorders. Oxford University Press, 2011. http://dx.doi.org/10.1093/med/9780198569381.003.0926.

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Almost any neurological disorder can produce a disorder of movement but the ‘movement disorders’ include the akinetic rigid syndromes, hyperkinesias, and some tremors. It can sometimes seem, especially with the use of videotape recordings, that diagnosis of movement disorders is mainly a matter of correct visual recognition. Such an approach is not recommended and can lead to mistakes unless, as in other areas of medicine, the history is considered first and the physical signs second. Obvious examples include the family history in Huntington’s disease, developmental history in dystonic cerebral palsy, and neuroleptic drug treatment in patients with tardive dyskinesia. In addition, a single disorder may give rise to several different types of involuntary movement. For example, Huntington’s disease may give rise to an akinetic rigid state, chorea, myoclonus, tics, or dystonia. Patients with Parkinson’s disease taking levodopa may show different types of movement disorder at different times of the day.In akinetic rigid states the diagnostic issue will be whether the patient has idiopathic Parkinson’s disease or one of the other Parkinsonian syndromes. With involuntary movements, the first step in diagnosis is to classify these as dystonia, tics, tremor, chorea, or myoclonus. It must be remembered that involuntary movements are merely physical signs, not diagnostic entities, and that they do not always occur in a pure form; for example, patients with dystonia may have additional choreiform movements or tremor. If more than one form of abnormal movement seems to be present, the diagnosis should be based on the most obvious one. The next step is to decide on the cause of the movements and at this stage the diagnosis must be based upon an accurate and complete history as noted above.The movement disorders are often associated with abnormalities of the basal ganglia and, to some extent, vice versa. This is not entirely correct. Disturbances of basal ganglia function certainly have profound effects on movement with the development of bradykinesia, rigidity, tremor, or the various forms of dyskinesia. However, it is not correct when considering the pathophysiology of movement disorders to regard the basal ganglia as an isolated movement control centre. In fact, they are an important but poorly understood component of a much wider motor system. It is also important to remember that the basal ganglia are involved in the processing of limbic and other cognitive processes which may also be disturbed by basal ganglia dysfunction.
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49

Grant, Robert. Neurocutaneous syndromes. Oxford University Press, 2011. http://dx.doi.org/10.1093/med/9780198569381.003.0235.

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This chapter describes several neurocutaneous syndromes, including tuberous sclerosis, neurofibromatosis, Sturge–Weber syndrome, Von-Hippel–Lindau disease and ataxia telangiectasia amongst others.Tuberous sclerosis, also known as Epiloia or Bournville’s Disease, is an autosomal dominant multisystem disease it usually presents in childhood with a characteristic facial rash, adenoma sebaceum, seizures, and sometimes learning difficulties. Central nervous system lesions in tuberous sclerosis are due to a developmental disorder of neurogenesis and neuronal migration. Other organs such as the heart and kidney are less commonly involved. The condition has very variable clinical expression and two-thirds of cases are thought to be new mutations, therefore it is important to examine and screen relatives. Management may involve many specialists and close co-operation between specialists is essential.The neurofibromatoses are autosomal-dominant neurocutaneous disorders that can be divided into ‘peripheral’ and ‘central’ types, although there is significant overlap. The characteristic features of neurofibromatosis type 1 are café au lait spots, neurofibromas, Lisch nodules, osseous lesions, macrocephaly, short stature and mental retardation, axillary freckling, and associations with several different types of tumours.Sturge–Weber syndrome involves a characteristic ‘port-wine’ facial naevus or angioma associated with an underlying leptomeningeal angioma or other vascular anomaly. It affects approximately 1/20 000 people. There can be seizures, low IQ, and underlying cerebral hemisphere atrophy as a result of chronic state of reduced perfusion and increased oxygen extraction. Patients may present with focal seizures which are generally resistant to anticonvulsant medication and can develop glaucoma.Von-Hippel– Lindau disease is one of the most common autosomal-dominant inherited genetic diseases that are associated with familial cancers. Von-Hippel–Lindau disease is characterized by certain types of central nervous system tumours, cerebellar and spinal haemangioblastomas, and retinal angiomas, in conjunction with bilateral renal cysts carcinomas or phaechromocytoma, or pancreatic cysts/islet cell tumours (Neumann and Wiestler 1991).Other neurocutaneous syndromes discussed include Hypomelanosis of Ito, Gorlin syndrome, Sjogren–Larsson syndrome, Proteus syndrome, Hemiatrophy and hemihypertrophy, Menke’s syndrome, Xeroderma pigmentosum and Cockayne’s syndrome.
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50

Alchi, Bassam, and David Jayne. The patient with antiphospholipid syndrome with or without lupus. Edited by Giuseppe Remuzzi. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0164.

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Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent arterial or venous thrombosis and/or pregnancy loss, accompanied by laboratory evidence of antiphospholipid antibodies (aPL), namely anticardiolipin antibodies (aCL), lupus anticoagulant (LA), and antibodies directed against beta-2 glycoprotein 1 (β‎‎‎2GP1). APS may occur as a ‘primary’ form, ‘antiphospholipid syndrome,’ without any known systemic disease or may occur in the context of systemic lupus erythematosus (SLE), ‘SLE-related APS’. APS may affect any organ system and displays a broad spectrum of thrombotic manifestations, ranging from isolated lower extremity deep vein thrombosis to the ‘thrombotic storm’ observed in catastrophic antiphospholipid syndrome. Less frequently, patients present with non-thrombotic manifestations (e.g. thrombocytopaenia, livedo reticularis, pulmonary hypertension, valvular heart disease, chorea, and recurrent fetal loss).The kidney is a major target organ in both primary and SLE-related APS. Renal involvement is typically caused by thrombosis occurring at any location within the renal vasculature, leading to diverse effects, depending on the size, type, and site of vessel involved. The renal manifestations of APS include renal artery stenosis and/or renovascular hypertension, renal infarction, APS nephropathy (APSN), renal vein thrombosis, allograft vasculopathy and vascular thrombosis, and thrombosis of dialysis access.Typical vascular lesions of APSN may be acute, the so-called thrombotic microangiopathy, and/or chronic, such as arteriosclerosis, fibrous intimal hyperplasia, tubular thyroidization, and focal cortical atrophy. The spectrum of renal lesions includes non-thrombotic conditions, such as glomerulonephritis. Furthermore, renal manifestations of APS may coexist with other pathologies, especially proliferative lupus nephritis.Early diagnosis of APS requires a high degree of clinical suspicion. The diagnosis requires one clinical (vascular thrombosis or pregnancy morbidity) and at least one laboratory (LA, aCL, and/or anti-β‎‎‎2GP1) criterion, positive on repeated testing.The aetiology of APS is not known. Although aPL are diagnostic of, and pathogenic in, APS, a ‘second hit’ (usually an inflammatory event) may trigger thrombosis in APS. The pathogenesis of the thrombotic tendency in APS remains to be elucidated, but may involve a combination of autoantibody-mediated dysregulation of coagulation, platelet activation, and endothelial injury.Treatment of APS remains centred on anticoagulation; however, it has also included the use of corticosteroids and other immunosuppressive therapy. The prognosis of patients with primary APS is variable and unpredictable. The presence of APS increases morbidity (renal and cerebral) and mortality of SLE patients.
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