先天性嗅覺全失之磁振造影

Magnetic Resonance Imaging of Congenital Anosmia

江榮山(Rong-San Jiang)

《台灣耳鼻喉頭頸外科雜誌》 48卷1期 (2013/03) Pp. 73-78

https://doi.org/10.6286/2013.48.1.73

摘要

背景：先天性嗅覺全失是嗅覺喪失罕見原因之一，可分為兩型：症候群及孤立型。本研究採討我們診斷為先天性嗅覺全失病患的磁振造影表現。方法：從2007年7月至2012年5月在本科共診斷11名先天性嗅覺全失病患。這些病患主訴有記憶以來並未曾問到任何氣味。他們接受理學檢查來排除罹患鼻及鼻竇炎，也排除罹患鼻息肉或腫瘤。如果病患被懷疑罹患鼻及鼻竇炎、鼻息肉或腫瘤，則安排電腦斷層檢查來確定診斷。他們也接受酚基乙基乙醇氣味查覺閾值試驗來檢查嗅覺功能。磁振造影檢查被安排來檢查嗅球、嗅溝及嗅束。結果：11名病患中男性有8名，女性有3名，年齡分布從9歲至50歲，平均24.2歲。理學檢查在鼻腔均未發現有明顯分泌物，也未發現有鼻息肉或腫瘤。所有病患的酚基乙基乙醇氣味查覺閾值試驗均呈現嗅覺全失。磁振造影檢查發現7名病患左右嗅球均未發育，4名病患左右嗅球則發育不良，5名病患左右均未有嗅溝，5名病患只有一邊有嗅溝，9名病患左右均未有嗅束，1名病患只有一邊有嗅束。結論：本研究顯示先天性嗅覺全失病患的磁振造影影像呈現嗅球未發育或發育不良，同時合併嗅溝及嗅束異常。

關鍵字

先天性嗅覺全失 ； 磁振造影 ； 嗅球 ； 嗅溝 ； 嗅束

並列摘要

BACKGROUND: Congenital anosmia is a rare occurrence and is classified as syndromic and isolated forms. This study reports magnetic resonance imaging (MRI) of our patients with congenital anosmia.METHODS: Eleven patients with congenital anosmia were diagnosed at my department between July of 2007 and May of 2012. These patients all complained of absence of smell as back as they could remember. Physical examinations were done to exclude rhinosinusitis, nasal polyps or nasal tumors. If these diseases were suspected, computed tomography was arranged to confirm the diagnosis. Their olfactory function was evaluated by a phenyl ethyl odor detection threshold test. MRI was performed to evaluate olfactory bulbs, olfactory sulci and olfactory tracts in these patients.RESULTS: Among these 11 patients, there were 8 males and 3 females. The age ranged from 9 years old to 50 years old with a mean of 24.2 years. Physical examinations did not find obvious discharge in nasal cavities, nasal polyps or nasal tumors. The phenyl ethyl odor detection threshold test confirmed complete olfactory loss in all patients. MRI revealed the olfactory bulb was absent bilaterally in 7 patients and hypoplastic bilaterally in another 4 patients. The olfactory sulcus was absent bilaterally in 5 patients and unilaterally in 5 patients. The olfactory tract was absent bilaterally in 9 patients and unilaterally in one patient.CONCLUSIONS: This study shows that MRI of patients with congenital anosmia revealed aplasia or hypoplasia of olfactory bulb with abnormalities of olfactory sulcus and tract.

並列關鍵字

congenital anosmia ； magnetic resonance imaging ； olfactory bulb ； olfactory sulcus ； olfactory tract