Mehndiratta M M, Bajaj B K, Gupta M, Anand R, Tatke M, Seryam S, Nehru R, Puri V, Khwaja G A
Department of Neurology, G.B. Pant Hospital, New Delhi-110002, India.
Neurol India. 2001 Dec;49(4):338-41.
Creutzfeldt-Jakob disease (CJD) is increasingly being reported over the last three decades as a result of heightened awareness of the disease. Various studies have reported annual incidence of 0.5-1.5 cases of CJD per million of general population. In India, the disease is still under reported. Over the period spanning from 1968-1997, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore recorded 69 cases of CJD from different parts of India in the CJD registry. This paper describes the clinical experience with cases of CJD managed at the Department of Neurology, G.B. Pant Hospital, New Delhi from 1990-1998. In this series, the mean age of the patients was 53.80 (+/- 7.32) years and there were 5 females and 5 males. Myoclonus was present in all the cases and abnormal behaviour with or without other features was the presenting complaint in 7 of the 10 patients, while one patient of CJD had cerebellar ataxia as the presenting feature. One patient with occipital variant of CJD presented with acute onset cortical blindness and myoclonic jerks. One of the patients had acute psychosis precipitated by emotional stress at the onset. Extrapyramidal features were noted in 7 of the 10 patients before death. The mean duration of symptoms from the onset of disease to death was 6.6 (+/- 6.11) months. Classical EEG changes were observed in all the patients, except in one possible case of occipital variant of CJD, where we did not have access to EEG record. Brain biopsy could be undertaken in 3 patients, and in 2 patients the features of subacute spongiform encephalopathy (SSE) were noted.
在过去三十年中，由于对克雅氏病（CJD）的认识提高，该病的报告病例越来越多。各项研究报告称，普通人群中CJD的年发病率为每百万人口0.5 - 1.5例。在印度，该病的报告仍然不足。在1968年至1997年期间，班加罗尔的国家心理健康和神经科学研究所（NIMHANS）在CJD登记处记录了来自印度不同地区的69例CJD病例。本文描述了1990年至1998年在新德里GB潘特医院神经科治疗的CJD病例的临床经验。在这个系列中，患者的平均年龄为53.80（±7.32）岁，有5名女性和5名男性。所有病例均出现肌阵挛，10例患者中有7例以异常行为（伴有或不伴有其他特征）为首发症状，而1例CJD患者以小脑共济失调为首发特征。1例枕叶变异型CJD患者表现为急性皮质盲和肌阵挛性抽搐。其中1例患者在发病时因情绪压力引发急性精神病。10例患者中有7例在死亡前出现锥体外系特征。从疾病发作到死亡的平均症状持续时间为6.6（±6.11）个月。除1例可能为枕叶变异型CJD的病例（我们无法获取其脑电图记录）外，所有患者均观察到典型的脑电图变化。3例患者进行了脑活检，其中2例发现亚急性海绵状脑病（SSE）的特征。
