摘要:

BACKGROUND‐Rett syndrome (RS) is a neurologic disorder that primarily affects girls. A genetic marker has recently been identified. Although RS is associated with a devastating loss of function between infancy and the fifth year of life, its course is relatively static thereafter, setting it apart from most neurodegenerative and genetic disorders of childhood. Clinical, neuropathological, and neurochemical studies call attention to RS as a neurodevelopmental disorder.REVIEW SUMMARY‐Rett syndrome is a distinct clinical entity, with a unique cluster of clinical signs and symptoms observed in a large number of girls in all ethnic groups. RS affects early development first with stagnation (stage 1), then with devastating cognitive and motor regression (stage 2), partial recovery (stage 3), and cognitive stability (stage 4). Epidemiologic studies have supported the hypothesis that RS is genetically determined. After years of research, it is now known that RS is caused by mutations in the methyl‐CpG‐binding protein‐2 gene on chromosome Xq28. Neuropathological studies point to developmental abnormalities dating back to the late second or third trimester in utero. However, the major impact of the disease occurs during postnatal brain growth, involving synapse formation. Alterations in the dopaminergic, cholinergic, and glutaminergic neurotransmitters play an important role in growing nerve processes and morphogenesis in the developing nervous system.CONCLUSIONS‐The identification of a biological marker, combined with improved understanding of the pathogenetic mechanisms, provide hope for therapeutic intervention sufficiently early to alter the course of irreversible brain damage.(THE NEUROLOGIST 7:73‐81, 2001)

ISSN:1074-7931    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

BACKGROUND‐Neurologic complications of acquired immunodeficiency syndrome (AIDS) are diverse and include opportunistic infections of the central and peripheral nervous systems, lymphoma, and primary neurologic disorders directly related to human immunodeficiency virus‐1 (HIV‐1) infection.REVIEW SUMMARY‐This article will review the etiology, pathogenesis, clinical features, and treatment of the most common opportunistic infections of the central and peripheral nervous systems in patients with AIDS. These include toxoplasmic encephalitis, cryptococcal meningitis, progressive multifocal leukoencephalopathy, syphilis, and cytomegalovirus infection. Primary central nervous system lymphoma occurs in patients with AIDS more frequently than in the general population but may be decreasing in incidence with the advent of highly active antiretroviral therapy. Primary neurologic disorders, which result from direct or indirect effects of HIV infection on the nervous system, will be reviewed. These include HIV‐related dementia, myelopathy, and neuropathy. Understanding the pathophysiology of these entities is important to develop effective treatments.CONCLUSIONS‐AIDS‐related neurologic disease is a significant cause of morbidity and mortality. However, a growing number of neurologic disorders can now be treated with highly active antiretroviral therapy, and increasing use of these agents has led to a significant decline in the incidence of opportunistic infections of the nervous system and primary neurologic disorders, such as HIV‐related dementia.(THE NEUROLOGIST 7:82‐97, 2001)

ISSN:1074-7931    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

BACKGROUND‐Several neurologic signs that are considered pathologic in younger patients are known to appear in the absence of pathology in older patients. At the same time, disorders of the nervous sytem are a major cause of morbidity in the aging population. Dementia is a particularly common condition in the aging population, with a steadily increasing incidence with age.REVIEW SUMMARY‐The neurologic findings in the elderly are reviewed, starting with the noncognitive portion of the neurologic examination and proceeding to cognitive changes. This literature review raises several questions regarding the nature of brain aging and late‐life dementia, which are explored additionally in a review of the findings of the Oregon Brain Aging Study. This is a longitudinal study of exceptionally healthy subjects older than 85 years of age in which longitudinal clinical and neuropsychologic data are complemented by longitudinal volumetric brain magnetic resonance imaging data. Neuropathologic examination of the brain has also been completed for the first cohort of brains coming to autopsy.CONCLUSIONS‐The neurology of healthy aging is characterized by the appearance of specific neurologic signs, a very gradual decline in some cognitive functions, and minimal loss of brain volume. With increasing age, even in the healthiest elderly, there is an increased incidence of dementia, which is characterized by increased rates of cognitive decline and brain volume loss. The pathology associated with this dementia is classical Alzheimer disease pathology. It is possible for elderly subjects to be spared cognitive decline, brain volume loss, and Alzheimer pathology, even into the eleventh decade. Although this phenotype represents the exception rather than the rule, additional investigations of healthy aging hold promise for elucidating the mechanisms responsible for both pathologic and successful brain aging.(THE NEUROLOGIST 7:98‐112, 2001)

ISSN:1074-7931    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

BACKGROUND‐Migraine is a common condition, and effective abortive treatment is the most important aspect of its management. The triptans represent a new class of abortive antimigraine medications. They are highly effective and generally well tolerated and safe, especially in their oral form. However, even though they are relatively selective in their vasoconstrictor activity, they are contraindicated in patients who have uncontrolled hypertension or coronary artery disease.REVIEW SUMMARY‐On the basis of the time the triptans take to reach their maximum plasma level, they fall into two categories: slower acting and faster acting. The slower‐acting triptan is naratriptan, and the faster‐acting ones are rizatriptan, sumatriptan, and zolmitriptan. The faster‐acting triptans accomplish in 2 hours what naratriptan accomplishes in 4 hours.CONCLUSIONS‐The faster‐acting triptans are comparable in their efficacy, speed of onset, tolerability, and duration of action and, therefore, can be used interchangeably. When one triptan is found to be ineffective or not well tolerated, another should be tried. The oral triptans are best administered at mild‐to‐moderate headache intensity. They then provide full freedom from headache at 2 hours at percentages similar to those observed for headache relief in clinical studies.(THE NEUROLOGIST 7:113‐121, 2001)

ISSN:1074-7931    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

BACKGROUND‐Epidural hemorrhage can be associated with a rapid and fatal deterioration if not promptly treated. Epidural hematoma is usually associated with trauma; however, it has rarely been associated with several atraumatic conditions, including hemophilia, afibrinogenemia, severe thrombocytopenia, vascular malformations of the dura mater, and central nervous system infectious processes. Multiple intracranial epidural hemorrhages after lumbar puncture in the setting of coagulopathy have not been reported previously.REVIEW SUMMARY‐Presented is a patient who suffered multifocal acute intracranial epidural hematomas after a diagnostic lumbar puncture. A 26‐year‐old woman with promyelocytic leukemia developed severe headaches while undergoing chemotherapy. After a lumbar puncture, the patient became unresponsive with a dilated pupil. Head computed tomographic scan demonstrated multiple epidural hematomas. Although the patient had immediate recovery of neurologic function after emergent evacuation, she died of sepsis related to immunosuppression.CONCLUSIONS‐Early recognition of spontaneous epidural hematomas can result in a favorable neurologic outcome if prompt evacuation is performed. Physicians should be aware of the potential risk of this occurrence.(THE NEUROLOGIST 7:122‐126, 2001)

ISSN:1074-7931    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1074-7931    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1074-7931    

出版商:OVID    

年代:2001

数据来源: OVID