摘要:

BACKGROUNDCerebral autosomal dominant arteriopathy with subcortical intarcts and leukoencephalopathy (CADASIL) is an inherited arterial disease of the brain. The affected gene was mapped in 1993 to chromosome 19. This mapping was essential in defining the natural history of the disease.REVIEW SUMMARYCADASIL can start with attacks of migraine with aura occurring at a mean age of 30 years. The most frequent clinical manifestations are subcortical transient ischemic attacks or completed strokes usually observed between 40 and 50 years of age. The stroke events are sometimes associated with severe mood disturbances. The disease leads progressively to a subcortical dementia associated with pseudobulbar palsy and urinary incontinence. Mean age at death is 65 years. The severity of the clinical presentation is greatly variable between and within families. Magnetic resonance imaging (MRI) is always abnormal in symptomatic subjects. On T2-weighted images areas of increased signals in white matter and basal ganglia and on T1-weighted images, it shows a decreased signal in the same regions. Signal abnormalities can be observed a long time before the onset of clinical manifestations, as they are present in asymptomatic young family members. The severity of the MRI findings dramatically increases with age. Pathologic studies show a widespread pallor of white matter and multiple small intarcts in both the white matter and basal ganglia, associated with a small artery disease of the brain. Electron microscopy studies show that the medium of the small arteries is thickened by a granular, eosinophilic, and nonamyloid material of undetermined origin that surrounds the smooth muscle cells. These ultrastructural abnormalities have been observed in other arteries, particularly in muscular and skin arteries. The gene of CADASIL is mapped to chromosome 19 in close vicinity to the locus of familial hemiplegic migraine and to that of acetazolamicle-responsive cerebellar paroxysmal ataxia.CONCLUSIONCADASIL should be suspected in subjects with a history of unexplained subcortical ischemic strokes, attacks of migraine with aura, mood disorders, or subcortical dementia whenever they are associated with MRI signal abnormalities in white matter and basal ganglia. These findings should prompt a genealogical study including all first-and second-degree relatives. CADASIL is an underdiagnosed cause of subcortical strokes.

ISSN:1074-7931    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

BACKGROUNDCarotid atherosclerotic disease is part of generalized atherosclerosis and may remain asymptomatic or present as transient ischemic attacks or stroke. The surgical treatment is endarterectomy.REVIEW SUMMARYCarotid artery atherosclerosis primarily involves the carotid bifurcation, a surgically accessible site. Since the first report of carotid endarterectomy in 1954, controversy has surrounded this surgery. The clinical presentation of patients with carotid disease, diagnostic testing, and medical and surgical management are reviewed. Selection of patients, practical issues in perioperative management, including the use of anticoagulants, and the timing of surgery for patients who have a completed stroke are discussed.CONCLUSIONSCarotid endarterectomy has been performed for more than 40 years. The operation is indicated for asymptomatic patients with 60% or more stenosis and for symptomatic patients with 70% or more stenosis, in whom the expected morbidity and mortality are less than 3% and 6%, respectively. Careful patient selection is critical to outcome. Long-term management remains risk factor control.

ISSN:1074-7931    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

BACKGROUNDThe relationship between epilepsy and behavior is one of the most controversial areas of study in neurology. The clinician is faced with a bewildering array of studies that present dogmatic and yet often contradictory points of view.REVIEW SUMMARYThis review presents a practical approach to the literature of psychiatric and psychosocial issues in epilepsy, highlighting the strengths as well as the methodologic difficulties of key studies. The first section summarizes current understanding of the complex relationship of epilepsy with global psychopathology, the temporal lobe personality syndrome, psychosis, depression, anxiety, and aggressive behavior. The effect of behaviors on seizures and the topic of nonepileptic seizures are also highlighted. The second section discusses important issues involved in pharmacologic therapies, including potential adverse behavioral effects of antiepileptic drugs, the risks of lowering seizure threshold with the use of psychotropic drugs, and common drug interactions between anticonvulsants and psychotropic agents. We conclude with a discussion of quality of life in epilepsy.CONCLUSIONCareful analysis of the complex literature of psychiatric issues in epilepsy provides practical and useful clinical guidelines for diagnosis and management.OVERVIEWFew subjects in neuropsychiatry have generated as much interest and controversy as the relationship of psychopathology to epilepsy. This review is designed to be a source of practical clinical information useful in the diagnosis and management of psychopathology in epilepsy. It is also a guided tour through a very complicated literature; we note salient studies that have advanced our understanding of this topic, as well as methodological difficulties that leave many questions unresolved.

ISSN:1074-7931    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

BACKGROUNDPatients with myalgia arc referred to neuromuscular centers for diagnosis and treatment where muscle biopsy is often performed to identify a possible organic or metabolic detect in the muscle. A review of large series that addresses the value of muscle biopsy in myalgia shows a wide range of morphological changes in the muscle (0 to 50%). In this review, I present my experience and that of others to provide a logical framework for the evaluation of such patients.REVIEW SUMMARYThe wide range of abnormalities seen in the muscles in different series of cases is related to selection of patients, interpretation of morphological changes in the muscle, and the extent of studies performed with the muscle tissue, such as ultrastructural studies and enzymes measurement.CONCLUSIONIn my series of cases, five patients were found to have primary muscle disease. The present review indicates that for patients with myalgia who have a normal physical examination and laboratory studies, a routine study of muscle tissue does not provide enough information. However, muscle biopsy remains a valuable tool in thoroughly evaluated patients to help identity suspected morphological, biochemical, or ultrastructural abnormalities.

ISSN:1074-7931    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

BACKGROUNDThe role of intravenous (IV) cyclophosphamide (CTX) for patients with progressive multiple sclerosis (MS) remains controversial, despite numerous controlled and uncontrolled clinical trials.OBJECTIVETo present the results of open label treatment with CTX and intravenous methylprednisolone (MP) in 17 consecutive patients with corticosteroid-resistant, fulminant MS.METHODOpen-label, nonblind, noncontrolled consecutive series of patients meeting the following criteria were treated with CTX and MP. Fulminant MS was defined as objectively documented, continuous deterioration by ≥1.5 points on the Kurtzke Expanded Disability Status Scale (EDSS) for ≥ 3 months. Corticosteroid resistance was defined as continued deterioration for 2 months after high-dose IV MP. After completion of the CTX/MP treatment protocol, all patients were subsequently treated with maintenance immunotherapy at the discretion of their treating neurologist. Each patient was followed by a single neurologist, who determined the EDSS according to a standard protocol at successive visits.RESULTSBaseline EDSS scores at the time of CTX/MP treatment ranged from 6.0 to 8.5. There were no serious complications of treatment. Follow-up time ranged from 12 to 24 months (median 24 months, mean 22 months). At 12 months, 1 3 of 1 7 (75.5%) patients were stable or improved by ≥1.0 EDSS point. At 24 months, 9 of 13 (69%) of patients were stable or improved.CONCLUSIONThis consecutive case series suggests that CTX/MP represents an effective therapeutic option for those rare MS patients with a fulminant progressive course who are refractory to corticosteroid therapy. The limitations related to the uncontrolled nature of the study and comparisons with other CTX studies are discussed.

ISSN:1074-7931    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1074-7931    

出版商:OVID    

年代:1997

数据来源: OVID