Reviews in neurological diseases最新文献

A 62-year-old woman with no known psychiatric illness had a 1.5-year history of progressive personality and language changes, leading to a loss of functional independence. Laboratory results revealed elevated autoimmune antibodies. She did not improve on high-dose steroid therapy and continued to deteriorate to her death, 2.5 years after symptom onset.

Intracerebral hemorrhage (ICH) is the most devastating type of stroke with the greatest mortality rate. Unfortunately there are no clinically proven therapies, and treatment is typically supportive. Given the poor prognosis, many families are faced with the decision to limit or withdraw care from those who have had an ICH. Many clinical grading systems have been developed to help stratify patients with ICH and aid in predicting prognosis. The ICH scale was the first to be developed to predict 30-day mortality and has been validated by different cohorts around the world. Since its inception in 2001, numerous other scales have been developed to predict mortality, and more importantly, functional outcome. However, in the development of these scales, patients who had care withdrawn were included in the analysis. It has been shown that the concept of poor prognosis leads to do-not-resuscitate orders, less invasive care, and eventually death. Most of these patients have care withdrawn within the first two hospital days, despite evidence to suggest early interventions may improve outcomes. As a result, the most recent guidelines suggest waiting more than 24 hours before deciding to withdraw care. The use and interpretation of these ever-advancing scales may allow physicians to better predict outcome and assist families in making important care-related decisions.

Epilepsy is one of the most common neurologic conditions seen by obstetricians, primary care physicians, and neurologists. It is present in three to five per 1000 births, and most women with epilepsy (WWE) can expect to have a normal pregnancy and delivery. The clinician's goal is to establish the best seizure control with the fewest possible number of antiepileptic drugs (AEDs) prior to pregnancy. Clinicians need to be aware of how the pharmacokinetic features of AEDs change during the pregnancy and postpartum period. During pregnancy AED concentrations may decrease, thereby increasing the risk of seizure deterioration. Levels of some AEDs must be monitored and the doses adjusted routinely during pregnancy and after birth. Understanding and applying these principles will ensure better seizure control during and after pregnancy and minimize the risk to the mother and the fetus due to recurrent seizures and fluctuating AED levels. This review emphasizes significant changes in pharmacokinetics of AEDs, the importance of monitoring serum concentration of AEDs, and routine dose adjustment prior to conception and during pregnancy and the postpartum period.

We describe an unusual case of cerebral intravascular lymphomatosis wherein the patient presented with multiple embolic strokes predominantly in the posterior circulation. Using this case as an illustration, we review the literature of this malignancy, which consists of extranodal diffuse large B-cell lymphoma. For patients with recurrent stroke-like events without cardiac risk factors, the accurate diagnosis requires a high index of suspicion by the neurologist and a brain biopsy specimen demonstrating lymphoma cells within the lumen of cerebral blood vessels. Intravascular lymphomatosis can be treated with systemic chemotherapies, but the response rate and pattern of relapse remain unknown.

Worsening memory is a common complaint in the elderly and predictably causes affected individuals and their families to wonder whether the underlying cause is Alzheimer disease, the most common form of dementia. Alzheimer disease is a devastating illness that unavoidably leads to a complete loss of independence and, as a result, substantial emotional, physical, and financial distress for patients and their families. The causes and severity of memory impairment in the elderly are diverse, however, so any given case might not necessarily be secondary to a neurodegenerative disorder such as Alzheimer disease. Consequently, it is critical to rule out potentially reversible causes of dementia and to initiate treatment while cognitive and functional deficits are still mild and more likely to respond to treatment. Furthermore, identifying the etiology and defining a suitable treatment plan early in the course of dementia allows patients to be more actively involved in the management of their disease and is more likely to improve quality of life for both patients and caregivers. This review presents the etiology of dementia in the elderly, describes the diagnostic process, and discusses current therapeutic strategies, including pharmacological agents, nonpharmacological interventions, safety assessments, legal issues, and caregiver needs.

Narcolepsy is a neurologic disorder characterized by excessive daytime sleepiness and manifestations of disrupted rapid eye movement sleep stage. The pathologic hallmark is loss of hypocretin neurons in the hypothalamus likely triggered by environmental factors in a susceptible individual. Patients with narcolepsy, in addition to excessive daytime sleepiness, can present with cataplexy, sleep paralysis, sleep fragmentation, and hypnagogic/hypnopompic hallucinations. Approximately 60% to 90% of patients with narcolepsy have cataplexy, characterized by sudden loss of muscle tone. Only 15% of patients manifest all of these symptoms together. Narcolepsy can be misdiagnosed as a psychiatric disorder or even epilepsy. An appropriate clinical history, polysomnogram, Multiple Sleep Latency Test, and, at times, cerebrospinal fluid hypocretin levels are necessary for diagnosis. The treatment of narcolepsy is aimed toward the different symptoms that the patient manifests. Excessive daytime sleepiness is treated with amphetamine-like or non-amphetamine-like stimulants. Cataplexy is treated with sodium oxybate, tricyclic antidepressants, or selective serotonin and norepinephrine reuptake inhibitors. Sleep paralysis, hallucinations, and fragmented sleep may be treated with benzodiazepine hypnotics or sodium oxybate. Patients with narcolepsy should avoid sleep deprivation, sleep at regular hours, and, if possible, schedule routine napping.

Delirium is sometimes defined as acute onset of either overactivity or underactivity. This article reviews the nature and clinico-anatomical locations of lesions in patients with reduced activity. The term abulia is used to describe global underactivity. Abulia is customarily explained by interruptions in frontal-subcortical circuitry. These interruptions can occur with lesions in the frontal lobes, caudate nuclei, midbrain, and thalamus. The article describes the anatomy of frontal and subcortical circuits and reviews in detail individual cases and series of patients with reduced initiative and activity who have had localized central nervous system lesions.

Pontine infarcts account for 25% of lacunar strokes. The primary morphologies are wedge-shaped tegmental, basal, and tegmentobasal infarcts, caused by disease of the paramedian basilar branches, and smaller, circumscribed lacunar infarcts attributed to lipohyalinosis. Roughly 60% of infarcts are paramedian. Both typical and atypical lacunar syndromes are seen with pontine infarcts, pure motor hemiparesis being the most common, followed by sensorimotor stroke and ataxic hemiparesis. Eye movement abnormalities and neuropsychological deficits are also commonly seen. Short-term functional prognosis is usually good with lacunar morphology; rostral, lateral or tegmental locations predict a more favorable prognosis.