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991.
ACTH gel and corticosteroids were given to 28 clinically definite multiple sclerosis (MS) patients to determine whether de novo central nervous system (CNS) IgG synthesis (rate and cerebrospinal fluid [CSF] IgG oligoclonal bands) could be eradicated. The most effective treatments were ACTH gel and ACTH gel followed by prednisone, all 11 patients had a significant reduction in rate (p < 0.05), which became normal in eight patients (< 3.3 mg per day). In order of effectiveness, the other drugs used were: dexamethasone or prednisone given orally, and hydrocortisone administered intrathecally. For most treatments, reduction of the rate of CNS IgG synthesis occurred within days and persisted for months after cessation of treatment. The MS CNS immune reaction was not eradicated when IgG synthesis rate became normal, because CSF IgG oligoclonal bands persisted. None of the chronic progressive, severely disabled patients demonstrated significant change in neurologic function or persistent adverse effects.  相似文献   
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Noonan syndrome: a review   总被引:7,自引:0,他引:7  
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Neuropsychiatric features characterize frontotemporal dementia (FTD). The authors evaluated the neuropsychiatric features of 53 FTD patients and retrospectively applied the Consensus Criteria for this disorder. Only one-third of the patients met Consensus Criteria for FTD on presentation. Most had early disengagement with poor insight; however, more than half retained socially appropriate interpersonal conduct and emotional expression. Supportive features, including compulsive-like acts and speech changes, were common presenting features, and 20% developed the Klüver-Bucy syndrome on 2-year follow-up. Consensus Criteria for FTD offer guidelines for diagnosis, but further refinement is needed, particularly for patients who lack early changes in social interpersonal conduct.  相似文献   
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Social impairments have long been recognized as a core feature of schizophrenia. Poor social, self-care, and vocational functioning are criteria for a diagnosis of schizophrenia in most diagnostic systems. Consequently, improving the social behaviors of persons with schizophrenia has been a key target of psychiatric rehabilitation techniques.One such technique, social skills training, has demonstrated effectiveness in yielding skill acquisition, durability, and generalization (1) and has been recognized as a psychosocial treatment of choice for schizophrenia (2). Nevertheless, a number of limitations to the benefits that may be achieved through skills training have been described. For example, the cognitive impairments of persons with schizophrenia, such as poor sustained attention and verbal memory deficits, have been shown to limit the acquisition of social skills (3). Moreover, one study showed that patients with negative symptoms, such as apathy, anhedonia, and amotivation, have an impaired capacity to benefit from social skills training (4). However, the results of a more recent study suggest that only persons with primary, deficit-type negative symptoms-not those with negative symptoms secondary to positive psychotic symptoms, depression, or extrapyramidal side effects-are unable to benefit from conventional social skills training (5).Recognizing these obstacles to implementing a social skills training program for persons with schizophrenia, Torres and his colleagues have created a board game called El Tren (The Train). Several characteristics of El Tren make it ideal for use with this population. First, the game is behaviorally oriented and has an emphasis on positive reinforcement and shaping. Second, it is sensitive to the cognitive limitations of the participants, emphasizing repetition and procedural learning. Third, it is designed to overcome participants' negative symptoms by being entertaining and fun. In this month's column, these authors describe El Tren and present the results of a randomized controlled study of its efficacy.  相似文献   
999.
Purpose: The purpose of this study is to evaluate the MR findings in patients with long-term ankylosing spondylitis (AS) and acute cervical spine fractures. Materials and methods: The magnetic resonance imaging (MRI) studies of five patients with AS and acute cervical spine fractures were retrospectively reviewed for the presence of cervical spine instability, spinal cord compression, and epidural hematoma. Results: Spinal fractures were unstable in all five patients. Three patients had neurological symptoms and abnormal signal within the spinal cord. All patients with neurological deficits had epidural hematomas posterior to the dural sac. Conclusion: MRI is useful for assessment of the integrity of intervertebral disks and spinal ligaments and, therefore, of the instability of the spinal fracture. MRI is mandatory in patients with neurological symptoms, especially in those with a symptom-free interval and those with neurological deterioration after established spinal cord injury, when suspicion for epidural hematoma is high. Electronic Publication  相似文献   
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