Handbook of clinical neurology
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The application of targeted temperature management has become common practice in the neurocritical care setting. It is important to recognize the pathophysiologic mechanisms by which temperature control impacts acute neurologic injury, as well as the clinical limitations to its application. Nonetheless, when utilizing temperature modulation, an organized approach is required in order to avoid complications and minimize side-effects. ⋯ Shivering is the most common side-effect of hypothermia and is best managed by adequate monitoring and stepwise administration of medications specifically targeting the shivering response. Due to the impact cooling can have upon pharmacokinetics of commonly used sedatives and analgesics, there can be significant delays in the return of the neurologic examination. As a result, early prognostication posthypothermia should be avoided.
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Critical care medicine came into sharp focus in the second part of the 20th century. The care of acutely ill neurologic patients in the USA may have originated in postoperative neurosurgical units, but for many years patients with neurocritical illness were admitted to intensive care units next to patients with general medical or surgical conditions. Neurologists may have had their first exposure to the complexity of neurocritical care during the poliomyelitis epidemics, but few were interested. ⋯ Most neurointensivists had a formal neurology training. This chapter is a brief analysis of the development of the specialty critical care neurology and how it gained strength, what it is to be a neurointensivist, what the future of care of these patients may hold, and what it takes for neurointensivists to stay exemplary. This chapter revisits some of the earlier known and previously unknown landmarks in the history of neurocritical care.
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The human prion diseases comprise Creutzfeldt-Jakob disease, variably protease-sensitive prionopathy, Gerstmann-Sträussler-Scheinker disease, fatal familial insomnia, and kuru. Each is a uniformly fatal rare neurodegenerative disease in which conformational changes in the prion protein are thought to be the central pathophysiologic event. The majority of cases of human prion diseases occur worldwide in the form of sporadic Creutzfeldt-Jakob disease and a minority of around 10-15% are associated with mutations of the prion protein gene, termed PRNP, in the forms of genetic Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia. ⋯ Despite having a high public profile, human prion diseases are both rare and heterogeneous in their clinicopathologic phenotype, sometimes making a diagnosis challenging. A combined clinical, genetic, neuropathologic, and biochemical approach to diagnosis is therefore essential. The intensive study of these diseases continues to inform on neurodegenerative mechanisms and the role of protein misfolding in more common neurodegenerative diseases such as Parkinson disease and Alzheimer disease.
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Dural arteriovenous fistulas are a heterogeneous group of lesions that comprise 10-15% of intracranial vascular malformations. The treatment strategy is devised after careful consideration of the arterial supply, venous drainage, clinical presentation, and risk of progression, hemorrhage, or neurologic decline. With recent advancements in endovascular technology, the majority of dural arteriovenous fistulas can be treated with either transarterial or transvenous embolization. Those that cannot be fully treated by endovascular means are approached with either adjuvant surgery or radiotherapy.
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Spinal cavernous malformations are intramedullary vascular lesions. They have low pressure and flow, so they may take many years to present with clinical symptoms. Because of their relatively benign nature, surgical intervention is not always indicated. ⋯ Sensory symptoms correlated with worse outcome. Given the natural history of spinal cavernous malformations, surgery may be considered for symptomatic patients, when general medical health and lesion location permit safe resection. The severity of symptoms must also be considered, as the natural history of the disease can be benign.