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克雅氏病 疾病

Overview

克雅氏病(CJD)是一种罕见的疾病, degenerative and fatal brain disorder caused by an infectious agent known as a "prion.“通常, the disease occurs at about age 60 and 90 percent of patients diagnosed with CJD die within a year.

在早期阶段, 症状可能包括记忆力减退, 行为变化, 缺乏协调性和视力问题.

随着病情的发展, mental deterioration becomes more severe and involuntary movements, 失明, 手臂和腿部无力,然后可能出现昏迷.

克雅氏病可能以三种方式发生:

  • Inherited, when there's a family history or a genetic mutation associated with the disease. About 5 to 10 percent of cases in the United States are hereditary.
  • Sporadically without apparent cause and no known risk factors. This is the common form of 克雅氏病 disease and accounts for about 85 percent of cases.
  • Transmitted through infection when exposed to brain or nervous system tissue, 通常是通过医疗程序.

There is no evidence that 克雅氏病 disease is contagious or that it can be spread through casual contact with a patient. Since 1920, less than 1 percent of the reported cases were acquired through infection.

The disease is part of a family of diseases known as transmissible spongiform encephalopathies (TSE). 海绵状指的是被感染的大脑的外观, which becomes filled with holes until it resembles a sponge under a microscope. 克雅氏病是这些疾病中最常见的. Other forms occur in animals such as bovine spongiform encephalopathy (BSE), which develops in cows and often is referred to as "mad cow" disease. Similar diseases occur in cats, elk, deer, goats, sheep and exotic zoo animals.

我们治疗克雅氏病的方法

作为痴呆症领域的国际领导者, UCSF specialists are working to find new treatments and a cure for 克雅氏病 disease. We offer the full scope of diagnostic tests and therapies to control 症状 and keep patients as comfortable as possible.

奖 & 识别

迹象 & 症状

克雅氏病 disease (CJD) affects many areas of the brain. 加州大学旧金山分校, CJD is sometimes called the "great mimicker" because it causes 症状 that occur in many other neurological diseases. 最初的症状差别很大,可能包括以下内容:

  • 行为和性格的改变
  • 混乱和记忆问题
  • 抑郁症
  • 失眠
  • 缺乏协调
  • 奇怪的身体感觉
  • 视力问题

随着疾病的发展, patients may experience a rapidly progressive dementia and in most cases involuntary and irregular jerking movements called myoclonus.

患者也可能出现惊恐和僵硬. In advanced stages of the disease, patients have difficulties with movement, swallowing and talking. In the final stage, patients lose all mental and physical function and may lapse into a coma. 许多病人死于感染,如肺炎.

The average duration of disease — from the onset of 症状 to death — is four to six months. 90%的病人在一年内死亡. 有些病例进展很快, 只持续了几周就去世了, 还有一些可能会持续两三年, 特别是如果疾病发生在很小的时候.

诊断

克雅氏病 disease (CJD) is typically a rapidly progressive dementia. Early diagnosis is important because the underlying cause of the dementia may be treatable.

如果怀疑患有克雅氏病,你可能会接受一系列的检查. Your doctor will conduct a neurological examination and other tests such as a 脊椎抽液 to rule out more common and treatable forms of dementia and an 脑电图(EEG) 来记录大脑的电模式, which can identify a specific abnormality that sometimes occurs in CJD.

计算机断层扫描(CT) of the brain can help rule out the possibility that 症状 are caused by other problems such as a stroke or a brain tumor.

最有效的诊断工具之一是 核磁共振成像 brain scan, which can reveal patterns of abnormal brain signals characteristic of CJD.

In rare cases, when the diagnosis is not clear, a brain biopsy is performed. A neurosurgeon removes a small piece of tissue from the abnormal area of the brain and the tissue is examined by a neuropathologist. Generally, an MRI is sufficient and this procedure is not necessary.

The 加州大学旧金山分校记忆与衰老中心 offers consultations to doctors from throughout the world who are treating patients with CJD. 如果你想让你的十大赌博平台排行榜咨询加州大学旧金山分校, 让你的十大赌博平台排行榜把你的病历传真给你的十大赌博平台排行榜. 迈克尔Geschwind电话:(415)476-4800. 十大赌博平台排行榜 at the 内存 and Aging Center will review your records.

核磁共振影像(最好是光碟)可寄至:

Dr. 迈克尔Geschwind
加州大学旧金山分校记忆与衰老中心
帕纳萨斯大道350号.905室
旧金山,加州94117

治疗

Currently, there is no cure for 克雅氏病 disease (CJD). 研究人员已经测试了许多药物, 包括无环鸟苷, amantidine, 抗生素, 抗病毒药物, 干扰素和类固醇. 没有一个显示出持续的益处.

Treatment is aimed at alleviating 症状 and making the patient as comfortable as possible. 如果疼痛发生,药物可以帮助缓解疼痛. The drugs clonazepam and sodium valproate may help relieve myoclonus or irregular, jerking movements.

Researchers at the 加州大学旧金山分校记忆与衰老中心 are trying to identify compounds for treatment or a cure for CJD and other diseases caused by the infectious particles called prions.

加州大学旧金山分校健康 medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.

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