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嗅觉发育不全或发育不良:无脑回畸形谱系中的一种变异?

Olfactory dysgenesis or hypoplasia: a variant in the arhinencephaly spectrum?

作者信息

Louis D N, Arriagada P V, Hyman B T, Hedley-Whyte E T

机构信息

Department of Pathology, Massachusetts General Hospital, Boston 02114.

出版信息

Neurology. 1992 Jan;42(1):179-82. doi: 10.1212/wnl.42.1.179.

DOI:10.1212/wnl.42.1.179
PMID:1734299
Abstract

Postmortem examination of a 65-year-old anosmic woman revealed rudimentary olfactory bulbs, an imperforate cribriform plate, and sulcal abnormalities of the orbitofrontal surface of the brain. The remainder of the brain, skull, and body was normal. This abnormality most likely resulted from a local insult to the area of the cribriform plate during early fetal life, occurring after the induction of olfactory bulb differentiation, but before migration and complete differentiation of the olfactory bulbs. Such a lesion can be dated to approximately 11 to 12 weeks gestational age. The malformation may represent another variant in the arhinencephaly spectrum, but is termed "olfactory dysgenesis" or "olfactory hypoplasia" to separate it from true olfactory aplasia and arhinencephaly.

摘要

对一名65岁嗅觉丧失女性进行的尸检显示,嗅球发育不全、筛板闭锁以及大脑眶额表面沟回异常。大脑其余部分、颅骨和身体均正常。这种异常很可能是由于胎儿早期生命中筛板区域受到局部损伤所致,该损伤发生在嗅球分化诱导之后,但在嗅球迁移和完全分化之前。这种病变可追溯到妊娠约11至12周龄。这种畸形可能代表无脑回畸形谱系中的另一种变体,但为了将其与真正的嗅觉发育不全和无脑回畸形区分开来,被称为“嗅觉发育异常”或“嗅觉发育不全”。

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Parkinson's disease in a patient with olfactory hypoplasia: a model case of "body-first Lewy body disease" providing insights into the progression of α-synuclein accumulation.嗅觉发育不全患者的帕金森病:“身体优先型路易体病”的一个典型病例,为α-突触核蛋白聚集的进展提供了见解。
J Neurol. 2023 Oct;270(10):5090-5093. doi: 10.1007/s00415-023-11783-2. Epub 2023 May 29.
2
International consensus statement on allergy and rhinology: Olfaction.国际过敏与鼻科学学会共识声明:嗅觉。
Int Forum Allergy Rhinol. 2022 Apr;12(4):327-680. doi: 10.1002/alr.22929.
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Incidental olfactory aplasia: a case report.
偶发性嗅觉缺失:一例病例报告
J Neurol Neurosurg Psychiatry. 1994 Feb;57(2):240. doi: 10.1136/jnnp.57.2.240.