Handbook of clinical neurology
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Review Case Reports
Natural history of cerebral cavernous malformations.
Cerebral cavernous malformations (CCM) are vascular abnormalities of the central nervous system with an incidence of 0.4-0.5% and an annual rate of hemorrhage ranging from 0.7% to 1%. Most lesions are located in the cerebral hemisphere but some occur in deeper locations such as the basal ganglia and pons. The most common symptoms during presentation are headache, seizures, and focal neurologic deficits. ⋯ This finding, however, is not consistent in all natural history studies evaluated. During follow-up, the most important and consistent risk factor for rebleed was a prior hemorrhage. Here, we provide an indepth but concise review of the literature regarding the natural history of CCMs.
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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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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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Acute traumatic spinal cord injury (SCI) is a devastating disease process affecting tens of thousands of people across the USA each year. Despite the increase in primary prevention measures, such as educational programs, motor vehicle speed limits, automobile running lights, and safety technology that includes automobile passive restraint systems and airbags, SCIs continue to carry substantial permanent morbidity and mortality. Medical measures implemented following the initial injury are designed to limit secondary insult to the spinal cord and to stabilize the spinal column in an attempt to decrease devastating sequelae. ⋯ We discuss initial triage, immobilization, and transportation of the patient by emergency medical services personnel to a definitive treatment facility. Upon arrival at the emergency department, we review initial trauma protocols and the evidence-based recommendations for radiographic evaluation of the patient's vertebral column. Finally, we outline closed cervical spine reduction and various aggressive medical therapies aimed at improving neurologic outcome.
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Decompressive surgery to reduce pressure under the skull varies from a burrhole, bone flap to removal of a large skull segment. Decompressive craniectomy is the removal of a large enough segment of skull to reduce refractory intracranial pressure and to maintain cerebral compliance for the purpose of preventing neurologic deterioration. Decompressive hemicraniectomy and bifrontal craniectomy are the most commonly performed procedures. ⋯ The ethical predicament of deciding to go ahead with a major neurosurgical procedure with the purpose of avoiding brain death from displacement, but resulting in prolonged severe disability in many, are addressed. This chapter describes indications, surgical techniques, and complications. It reviews results of recent clinical trials and provides a reasonable assessment for practice.