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首页> 外文期刊>The Internet Journal of Neurology >An Autopsy Proved A Case Of Creutzfeldt-Jakob Disease Presenting With Depression And Catatonia
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An Autopsy Proved A Case Of Creutzfeldt-Jakob Disease Presenting With Depression And Catatonia

机译：验尸证明一例克雅氏病伴抑郁症和卡塔尼亚病

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Creutzfeldt-Jakob disease (CJD) is increasingly being reported over the last three decades as a result of heightened awareness for the disease. Various studies have reported an annual incidence of 0.5-1.5 cases of CJD per million of general population. However, in our country, like other developing countries the disease is still underreported. Here, we describe our clinical experience with an autopsy proved case of CJD. Introduction Prion diseases, or transmissible spongiform encephalopathies, are a group of fatal neurode-generative brain diseases, which include scrapie and bovine spongiform encephalopathy (BSE) in animals and kuru and Creutzfeldt-Jacob disease in human. Prion diseases are generally characterised by progressive dementia and ataxia. At the time of death, the brain shows spongiform degeneration due to loss of neurons accumulation of the abnormal protease-resistant form (PrP RES) of the normal protease-sensitive cellular glyco-protein (PrP SEN).CJD may have three different origins: sporadic, familial, due to mutations in the prion gene, or infectious, due to iatrogenic exposure to infectious brain material.This paper describes the clinical experience with an autopsy proved case of CJD managed at the Department of Neurology, Razi Hospital.This paper describes the clinical experience with an autopsy proved case of CJD managed at the Department of Neurology, at the Razi Hospital in Iran. Case report A 56 years old woman was refered to our Hospital in October 2003 with a 2 months history of depressive symptoms accompanied by confusion and disorientation. In the past three months, her family members noted her insomnia, loss of appetite and periodic agitation. She was anxious in some days and had some delusions especially in the form of guilt and jealousy. Her interest and pleasure in almost all activities diminished remarkabely.She refered to a psychiatrist and the diagnosis of depression was made and treatment with antidepresants (Nortriptyllin 75mg /day and halopridol 0.5mg/day ) was prescribed. Some improvement in the symptoms was seen but after a few weeks her problems were more complicated. Her speech and responsivness became less and less and eventually she developed complete muteness. During examination, she was in catatonic stupor state. There was lack of spontaneous speech or movement, absence of goal-directed behavior, stiffness and rigidity in all body movements. The family history and her past medical history were negative for similar disorder, surgery and any other medical problems. The CT scan and MRI were normal. The EEG showed generalized background slowing with a few irregular sharp waves. Biochemical and microbiological studies of CSF were normal. Routine blood counts and chemistries were normal. Psychiatric consultation was requested and a diagnosis of “bipolar catatonia” was made and electrocunvulsive therapy (ECT) was started. After three ECTs the patient's state became deteriorated and she intubated for airway support. During last days of addmision some brief and localized myoclonus was noticed in her hand but there was no gross polymyoclonus in history and serial examinations of patient. In the final stage of the disease, the patient lost all her mental and physical functions. The serial EEGs showed generalized periodic sharp waves, 0.5 to 1 cycles per second, typical for Creutzfeldt-Jakob disease (Figure 1).
机译：在过去的三十年中，越来越多的人报道了克雅氏病（CJD）。各种研究报告说，每百万普通人群每年发生0.5-1.5克雅加德病。但是，在我们国家，与其他发展中国家一样，该疾病的报告率仍然较低。在这里，我们以尸检证明的CJD病例描述我们的临床经验。简介Pri病毒病或可传播的海绵状脑病是一组致命的神经变性脑病，包括动物的瘙痒病和牛海绵状脑病（BSE）以及人的库鲁病和克雅氏病。 on病毒疾病通常以进行性痴呆和共济失调为特征。死亡时，由于正常蛋白酶敏感性细胞糖蛋白（PrP SEN）的异常蛋白酶抗性形式（PrP RES）的神经元积聚，大脑显示出海绵状变性.CJD可能有三个不同的起源：由于the病毒基因突变引起的散发性家族性感染，或由于医源性暴露于感染性脑材料而引起的传染性。本文描述了拉齐医院神经内科经尸检证实的CJD病例的临床经验。尸检的临床经验证明是由伊朗拉兹医院神经内科管理的CJD病例。病例报告2003年10月，一名56岁的妇女被转诊到我院，有2个月的抑郁症状，伴有混乱和迷失方向。在过去的三个月中，她的家人注意到她失眠，食欲不振和周期性躁动。她在某些日子里焦虑不安，并且特别是在内of和嫉妒的形式上有一些幻想。她对几乎所有活动的兴趣和愉悦度明显降低。她向精神病医生求助，对抑郁症进行了诊断，并开了抗抑郁药（去甲肾上腺素75毫克/天和吡咯烷醇0.5毫克/天）。症状有所改善，但几周后她的问题更加复杂。她的言语和责任感越来越少，最终她变得完全安静。在检查过程中，她处于紧张性木僵状态。在所有身体运动中都缺乏自发的言语或动作，目标导向的行为，僵硬和僵化。家族病史和她过去的病史对类似的疾病，手术和任何其他医学问题均不利。 CT和MRI均正常。脑电图显示全身背景减慢，并伴有一些不规则的尖锐波。脑脊液的生化和微生物学研究正常。常规血常规和化学检查正常。要求进行精神科咨询，并诊断为“双相性卡塔尼亚病”，并开始进行电刺激治疗。经过三个ECT后，患者的状态恶化，并向其插管以支持气道。在成瘾的最后几天，她的手上发现了一些短暂而局部的肌阵挛，但患者的病史和连续检查中均未见明显的多肌阵挛。在疾病的最后阶段，患者失去了所有的身心功能。连续脑电图显示出普遍的周期性尖波，每秒0.5到1个周期，这对于Creutzfeldt-Jakob病是典型的（图1）。

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