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神经退行性疾病中的嗅觉

Olfaction in neurodegenerative disorder.

作者信息

Hawkes Christopher

机构信息

Essex Centre for Neuroscience, Oldchurch Hospital, Romford, UK.

出版信息

Adv Otorhinolaryngol. 2006;63:133-151. doi: 10.1159/000093759.

Abstract

There has been gradual increase of interest in olfactory dysfunction since it was realised that anosmia was a common feature of idiopathic Parkinson's disease (IPD) and Alzheimer-type dementia. It is an intriguing observation that a premonitory sign of a disorder hitherto regarded as one of movement or cognition may be that of disturbed sense of smell. In this review of aging, IPD, parkinsonian syndromes, tremor, Alzheimer's disease (AD), motor neuron disease (MND), Huntington's chorea (HC) and inherited ataxia, the following observations are made: (1) olfactory senescence starts at about the age of 36 years in both sexes and accelerates with advancing years, involving pleasant odours preferentially; (2) olfactory dysfunction is near-universal, early and often severe in IPD and AD developing before any movement or cognitive disorder; (3) normal smell identification in IPD is rare and should prompt review of diagnosis unless the patient is female with tremor-dominant disease; (4) anosmia in suspected progressive supranuclear palsy and corticobasal degeneration is atypical and should likewise provoke diagnostic review; (5) subjects with hyposmia and one ApoE4 allele have an approximate 5-fold increased risk of later AD; (6) impaired sense of smell may be seen in some patients at 50% risk of parkinsonism, and possibly in patients with unexplained hyposmia; (7) smell testing in HC and MND where abnormality may be found is not likely to be of clinical value, and (8) biopsy of olfactory nasal neurons reveals non-specific changes in IPD and AD and at present will not aid diagnosis.

摘要

自从认识到嗅觉功能障碍是特发性帕金森病(IPD)和阿尔茨海默病型痴呆的常见特征以来,人们对嗅觉功能障碍的兴趣逐渐增加。一个有趣的现象是,一种迄今被认为是运动或认知障碍之一的疾病的先兆症状可能是嗅觉紊乱。在这篇关于衰老、IPD、帕金森综合征、震颤、阿尔茨海默病(AD)、运动神经元病(MND)、亨廷顿舞蹈病(HC)和遗传性共济失调的综述中,有以下观察结果:(1)嗅觉衰老在36岁左右开始,男女皆然,且随着年龄增长而加速,优先累及令人愉悦的气味;(2)嗅觉功能障碍在IPD和AD中几乎普遍存在、出现早且往往严重,在任何运动或认知障碍出现之前就已发生;(3)IPD患者中嗅觉识别正常的情况罕见,除非患者是震颤为主型疾病的女性,否则应促使重新审视诊断;(4)疑似进行性核上性麻痹和皮质基底节变性患者的嗅觉丧失不典型,同样应引发诊断性复查;(5)嗅觉减退且携带一个载脂蛋白E4等位基因的受试者患后期AD的风险大约增加5倍;(6)嗅觉减退可能在约50%有帕金森病风险的患者中出现,也可能在不明原因嗅觉减退的患者中出现;(7)在HC和MND中进行嗅觉测试可能发现异常,但不太可能具有临床价值;(8)嗅鼻神经元活检在IPD和AD中显示非特异性变化,目前无助于诊断。

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