摘要:

BACKGROUND‐Cardiac involvement is a common and well‐recognized feature of many neuromuscular diseases. The types of involvement vary; some are severe and may lead to death, whereas others are mild and often subclinical. Cardiac dysfunction generally manifests during the course of an underlying neuromuscular disease and increases in severity with a longer duration of disease. Cardiac symptoms rarely present as initial manifestations of neuromuscular disease.REVIEW SUMMARY‐Cardiac dysfunction in neuromuscular diseases generally presents with any or all of three cardinal features: heart failure, cardiac arrhythmias, and/or conduction disorder. Dystrophinopathies and Friedreich's ataxia commonly present with cardiomyopathy, whereas myotonic muscular dystrophy and Kearns‐Sayre syndrome present with heart block and a variety of arrhythmias. New diagnostic cardiac technologies and recent molecular, genetic, and biochemical advances have not only helped in accurate diagnosis but have made significant contributions to under‐standing the pathophysiology and prognosis of many cardiomyopathies. In many cases, it is now possible to predict the severity of heart disease directly by DNA analysis.CONCLUSION‐Cardiac manifestations of neuromuscular disease are heterogeneous. There is a high incidence of heart disease in many neuromuscular diseases, but the majority of patients are asymptomatic or mildly symptomatic during the early stages. Early diagnosis of cardiac dysfunction and appropriate treatment may prolong survival and improve quality of life in these patients. It is important to remember that an unexplained cardiac dysfunction could indicate the existence of an underlying neuromuscular disorder.(THE NEUROLOGIST 6:67‐82, 2000)

ISSN:1074-7931    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

BACKGROUND‐Effective management of the medical and surgical aspects of patients who are treated in neurological intensive care units (NICUs) depends on periodic neurological assessment of brain function. This assessment can be aided by application of various monitoring tools on a periodic or continuous basis.REVIEW SUMMARY‐Intracranial pressure monitoring is standard in NICUs and allows for the continuous measurement of cerebral perfusion pressure. Transcranial Doppler can provide information about pending cerebral vasospasm after subarachnoid hemorrhage and can measure cerebral vasoreactivity. Jugular venous oximetry monitoring has been applied to patients with head injury. Paired measurement of both intracranial pressure and jugular venous oxygen content provides useful information to manage blood pressure, ventilation, and osmotherapy in patients with head injury. Continuous EEG may identify unsuspected nonconvulsive status epilepticus and when applied to all NICU patients may provide important information for medical decision making. Evoked potentials are useful in establishing prognosis for both adults and neonates.CONCLUSIONS‐The continuous measure of brain function of patients in NICUs is technologically feasible and devices that provide this information in real time are now commercially available. It is hoped that these devices can improve patient outcome and hospital efficiency.(THE NEUROLOGIST 6:83‐97, 2000)

ISSN:1074-7931    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

BACKGROUND‐Muscle overactivity (e.g., spastic dystonia and pathologic antagonistic cocontraction), in addition to motor weakness and muscle shortening, is one of the three cardinal disabling features in patients with damage to central motor pathways. Muscle overactivity is usually not distributed equally throughout the body; instead, it predominates in certain muscles. As a result, imbalance often exists between hypoactive agonists and relatively hyperactive antagonists. Treatments targeting overactivity in specific muscles (local treatments) may therefore be preferable to generalized therapies. This article reviews the physiological action, pharmacology, risks, and indications of the most promising of these local treatments, botulinum toxin (BTX).REVIEW SUMMARY‐BTX is a neurotropic protein that blocks acetylcholine release from all cholinergic terminals, including peripheral motor nerves, which results in flaccid paralysis when injected into muscle. Seven serotypes of BTX are produced by several species of Clostridium, and some have been purified for therapeutic purposes. The paralyzing action of BTX is temporary, and recovery of function occurs after terminal nerve sprouting and reformation of neuromuscular junctions. No muscle or nerve destruction occurs after BTX injection into muscle, as opposed to neurolytic agents (e.g., alcohol and phenol), and clinical tolerance is usually excellent. To target specific muscles, the stimulation technique seems more effective than passive EMG monitoring. Because BTX blocks all cholinergic synapses, it has also been used in autonomic disorders as an antimuscarinic agent.CONCLUSIONS‐BTX is a well‐tolerated, local pharmacologic treatment for muscle overactivity, and its range of licensed indications will likely increase, particularly among spastic conditions. However, pharmaco‐economic considerations mandate that controlled comparative studies between BTX and other neurolytic agents are performed. The genetic synthesis of a biologically active BTX may render this treatment more cost‐effective in the future.(THE NEUROLOGIST 6:98‐115, 2000)

ISSN:1074-7931    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

BACKGROUND‐Alzheimer's disease (AD) is the most common form of dementia, comprising an estimated 60 to 80% of the dementing illnesses. As a result of increasing life expectancy and the rising incidence of dementia with advancing age, dementia is a major public health concern. The costs of treatment for AD in the United States rank it among major diseases, but its true costs are not well understood. Pharmacological treatment for AD has been available only in the past 7 years, and much needs to be learned about the current and potential benefits of AD treatment.REVIEW SUMMARY‐The costs of caring for patients with AD can be divided into two categories: informal and formal. Informal care is largely provided by family caregivers. The costs associated with informal care involve the time spent in caregiving that constitutes lost opportunities for paid employment. Formal care includes acute medical services as well as day care, respite care, and especially long‐term care. These generate what are referred to as formal costs, which are definitely higher for patients with AD than for other comparably aged patients. The introduction of cholinesterase inhibitors to treat AD has raised questions about their economic costs and benefits. Although virtually all clinical trials of anti‐AD drugs are of short duration, economic modeling has made it possible to gain some insight into the economic benefits of anti‐AD drugs over a longer time period. Four studies that describe economic costs and benefits of anti‐AD therapy are reviewed. Because of the uncertainty about the duration and size of clinical effects of cholinesterase inhibitors, the estimates of costs and benefits span a range from cost‐saving to expensive. Varying estimates also arise for the impact of anti‐AD drugs on formal versus informal costs. If anti‐AD therapies were to be shown to reduce formal costs such as nursing home placement, the impact of pharmacological intervention in AD could be dramatic. Economic modeling shows, however, that anti‐AD drugs need to reduce informal costs to achieve more sizable economic impact.CONCLUSIONS‐Given the efficacy of cholinesterase inhibitors and the current data on the duration of their effects, the estimated economic benefits of this class of drugs are modest. Developing economic models depict what order of magnitude of drug effects are necessary before substantial economic benefits could possibly occur.(THE NEUROLOGIST 6:116‐125, 2000)

ISSN:1074-7931    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

BACKGROUND‐Nervous system manifestation of sarcoidosis (neurosarcoidosis) may occur in 5 to 10% of patients who have systemic sarcoidosis. Neurosarcoidosis may also occur in patients who have not had a previous diagnosis of sarcoidosis (primary neurosarcoidosis). We discuss the clinical presentation of primary neurosarcoidosis, treatment response, and outcome in this study.REVIEW SUMMARY‐Records of 22 patients with neurosarcoidosis who were not known to have systemic sarcoidosis at the time of neurologic presentation were reviewed. The clinical presentation and diagnostic procedures that led to the diagnosis in these patients are summarized. Neurologic presentations were highly variable. Neuroimaging was abnormal in all patients when contrast was used; meningeal enhancement was the most common finding. Spinal fluid was abnormal in 95% of patients. Seventy percent of chest x‐rays and 80% of chest CT scans performed at the time of presentation were abnormal. All 12 gallium scans that were performed were abnormal. Diagnosis was confirmed by biopsy of neural tissue in 36% and of non‐neural tissue in 64%. Initial response to oral corticosteroids was favorable, but more than half of the patients at 1 year had deteriorated.CONCLUSIONS‐The evaluation of patients with primary neurosarcoidosis can be optimized by combining clinical suspicion, based on presenting symptoms and signs, with spinal fluid and neuroimaging studies. Appropriate use of chest x‐ray and CT and gallium scans frequently leads to diagnosis from lymph node or lacrimal gland biopsy.(THE NEUROLOGIST 6:126‐133, 2000)

ISSN:1074-7931    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1074-7931    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1074-7931    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1074-7931    

出版商:OVID    

年代:2000

数据来源: OVID