Handbook of clinical neurology
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Circadian organization of physiology and behavior is an important biologic process that allows organisms to anticipate and prepare for predictable changes in the environment. Circadian disruptions are associated with a wide range of health issues. In patients with neurodegenerative diseases, alterations of circadian rhythms are among the most common and debilitating symptoms. ⋯ Recent studies have examined the neuropathologic status of the different neural components of the circuitry governing the generation of circadian rhythms in neurodegenerative diseases. In this review, we will dissect the potential contribution of dysfunctions in the different nodes of this circuitry to circadian alterations in patients with parkinsonism-linked neurodegenerative diseases (namely, Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy). A deeper understanding of these mechanisms will provide not only a better understanding of disease neuropathophysiology but also holds promise for the development of more effective and mechanisms-based therapies.
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More than one-third of patients with meningiomas will experience seizures at some point in their disease. Despite this, meningioma-associated epilepsy remains significantly understudied, as most investigations focus on tumor progression, extent of resection, and survival. Due to the impact of epilepsy on the patient's quality of life, identifying predictors of preoperative seizures and postoperative seizure freedom is critical. ⋯ Both pre- and postoperative meningioma-related seizures are often responsive to antiepileptic drugs (AEDs), although AED prophylaxis in the absence of seizures is not recommended. AED selection is based on current guidelines for treating focal seizures with additional considerations including efficacy in tumor-related epilepsy, toxicities, and potential drug-drug interactions. Continued investigation into medical and surgical strategies for preventing and alleviating epilepsy in meningioma is warranted.
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Declaration of brain death requires demonstration of irreversible injury to the whole brain including the brainstem. Current guidelines rely on bedside clinical examination to determine that the patient has irreversible coma, absent cranial nerve reflexes, and apnea. Neurophysiologic testing to support the clinical diagnosis of brain death has primarily consisted of EEG and evoked potentials-typically a combination of somatosensory evoked potential and brainstem auditory evoked potential. ⋯ Clinical scenarios in which neurophysiologic testing may aid the declaration of brain death include equivocal results of clinical examination findings, inability to perform some aspects of the neurologic examination, concern for residual sedative effects, suspected spinal cord or neuromuscular injury, and posterior fossa lesions with brainstem involvement. In these scenarios, EEG and evoked potentials may offer supportive evidence for irreversible injury to the whole brain. This chapter also discusses differences between current adult and pediatric guidelines for the role of ancillary testing in brain death.
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Delirium is an acute and transient brain dysfunction that is characterized by disturbances in consciousness, affecting both its content (i.e., attention) and level (i.e., arousal). It affects as many as 50% of those admitted to an intensive care unit (ICU). Once believed to be an inconsequential outcome of critical illness, it is now recognized that delirium is harmful in both the short- and long-term. ⋯ Nonpharmacologic strategies such as those which seek to reduce sensory impairment, sleep deprivation, and immobility are effective. Pharmacologic treatment with antipsychotics, though commonly used, is not supported by findings from placebo-controlled trials. Recent data support from multiple trials support the use of the "ABCDEF bundle" as a means by which to reduce delirium.
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Palliative care is an approach to the care of patients, affected by serious illness, and their families that aims to reduce suffering through the management of medical symptoms, psychosocial issues, spiritual well-being, and setting goals of care. Patients and families affected by a neurodegenerative illness have significant palliative care needs beginning at the time of diagnosis and extending through end-of-life care and bereavement. We advocate an approach to addressing these needs where the patient's primary care provider or neurologist plays a central role. ⋯ Managing distressing medical and psychiatric symptoms is critical to improving quality of life throughout the disease course as well as at end-of-life. Many symptoms are common across illnesses; however, there are issues that are specific to the most common classes of neurodegenerative illness, namely dementia, parkinsonism, and motor neuron disease. Incorporating a palliative approach to care, although challenging in many ways, empowers physicians to provide greater support and guidance to patients and families in making the difficult journey through a neurodegenerative illness.