Hawkes C H, Shephard B C, Geddes J F, Body G D, Martin J E
Department of Clinical Neurology, Ipswich Hospital, Ipswich, IP4 5PD, United Kingdom.
Exp Neurol. 1998 Apr;150(2):248-53. doi: 10.1006/exnr.1997.6773.
In Parkinson's disease and Alzheimer's disease there is profound disorder of olfaction. The extent to which this modality is involved in motor neuron disease (MND) has been studied little. To address this further we assessed olfaction by three methods-a smell identification test ("UPSIT") in 58 patients and 135 controls; olfactory-evoked response (OEP) to H2S in 15 patients, and pathological examination of olfactory bulbs obtained from 8 cadavers. It was found that smell identification compared with the controls was slightly worse overall in the MND group as a whole, but only the bulbar patients scored significantly less on the UPSIT. Patients displayed a subtle defect in cheese odor recognition. OEPs were normal in 9 subjects and delayed in 1 subject. The remaining 5 OEPs were unsuccessful. Histopathological studies of olfactory bulbs showed excess lipofuscin deposition in all 8 cases examined, indicating subclinical neuronal damage. Olfactory neurons with a degree of antioxidant defect may be more susceptible to cellular damage than other neuronal groups because of their direct relationship to environmental agents. Overall we found the degree of olfactory dysfunction in MND to be mild and in contrast with the marked changes described by others.
在帕金森病和阿尔茨海默病中，嗅觉存在严重紊乱。嗅觉在运动神经元病（MND）中的受累程度鲜有人研究。为进一步探讨这一问题，我们采用三种方法评估嗅觉：对58例患者和135名对照进行嗅觉识别测试（“UPSIT”）；对15例患者进行对硫化氢的嗅觉诱发电位（OEP）测试；对8具尸体的嗅球进行病理学检查。结果发现，MND组总体上与对照组相比，嗅觉识别略差，但只有延髓型患者在UPSIT测试中的得分显著较低。患者在奶酪气味识别方面存在细微缺陷。9名受试者的OEP正常，1名受试者的OEP延迟。其余5次OEP测试未成功。嗅球的组织病理学研究显示，在所有8例检查病例中均有过量脂褐素沉积，表明存在亚临床神经元损伤。由于嗅觉神经元与环境因素直接相关，具有一定抗氧化缺陷程度的嗅觉神经元可能比其他神经元群体更容易受到细胞损伤。总体而言，我们发现MND中的嗅觉功能障碍程度较轻，与其他人描述的明显变化形成对比。
