Reconsidering olfactory bulb magnetic resonance patterns in Kallmann syndrome

Hacquart, Thomas; Ltaief-Boudrigua, A.; Jeannerod, Cécile; Hannoun, Salem; Raverot, Gérald; Pugeat, Michel; Perrière, Aude Brac de la; Lapras, V.; Nugues, F.; Dodé, Catherine; Cotton, François

doi:10.1016/j.ando.2016.12.003

Cited by 8 publications

(7 citation statements)

References 15 publications

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“…Studies have reported that the main characteristic of the olfactory apparatus of Kallmann patients was the absence of OBs and tracts, which were usually bilateral or unilateral (22)(23)(24). In our study, 27 out of 28 KS patients showed bilateral OB aplasia with or without olfactory sulcus aplasia.…”

Section: Discussionsupporting

confidence: 50%

The diagnostic value of the olfactory evaluation for congenital hypogonadotropic hypogonadism

Chen

Mao

et al. 2022

Front. Endocrinol.

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ObjectiveThe aim of this study was to evaluate the diagnostic accuracy of different olfactory evaluation tools in congenital hypogonadotropic hypogonadism (CHH) patients.MethodsSeventy-one CHH patients were prospectively recruited at Peking Union Medical College Hospital between November 2020 and July 2021. The Chinese Olfactory Function Test (COFT) and Self-reported Olfactory Scale (SROS) were adapted as the subjective tools for the evaluation of olfactory function, and magnetic resonance imaging of olfactory apparatus (MRI-OA) was the objective tool. The olfactory bulb volume (OBV) and the olfactory sulcus depth (OSD) were quantified.ResultsBased on the COFT, 36 patients were categorized as having normosmic CHH (nCHH), and the other 35 patients were categorized as having Kallmann syndrome (KS). Among nCHH patients, 35 patients were classified as having normal olfaction and 1 patient had abnormal olfaction by SROS. For KS patients, there were 30 patients grouped into abnormal olfaction, while 5 patients had normal olfaction by SROS. For MRI-OA, 67% (18/27) of nCHH patients showed normal olfactory apparatus, and 33% (9/27) showed bilateral or unilateral olfactory bulb aplasia or hypoplasia. Among KS patients, 96% (27/28) of patients showed bilateral olfactory bulb hypoplasia or aplasia, and 4% (1/28) of patients showed normal olfactory apparatus. All six patients with unilateral olfactory bulb aplasia and three patients with bilateral olfactory bulb aplasia showed normal olfactory function. The accuracy of the SROS in the diagnosis of nCHH and KS was 91.5%, with a sensitivity of 0.857 and a specificity of 0.972, while the accuracy of MRI-OA is 92.7%, with a sensitivity of 0.964 and a specificity of 0.889.ConclusionSROS and MRI-OA both showed high accuracy to distinguish between KS and nCHH. The abnormal structure of the olfactory apparatus was relatively common in nCHH patients. CHH patients with unilateral olfactory bulb aplasia dysplasia usually had normal olfaction. Normal olfaction without apparent olfactory bulbs is rare but occurred in male CHH patients.

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Section: Discussionsupporting

confidence: 50%

The diagnostic value of the olfactory evaluation for congenital hypogonadotropic hypogonadism

Chen

Mao

et al. 2022

Front. Endocrinol.

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“…Recent studies have shown ethmoid bone abnormalities and pituitary anomalies on computed tomography in KS patients. The current study raises the possibility of detecting such findings using MRI ( 9 , 10 ).…”

Section: Discussionmentioning

confidence: 79%

“…MRI is highly valuable in evaluating suspected KS. Data suggest that, in hypogonadotropic hypogonadism, MRI being a non-irradiating technique, should be the first radiological step for investigating the pituitary gland as well as abnormalities of the ethmoid, olfactory bulb and tracts ( 10 ). In patients with suggestive clinical findings, MRI can contribute to the diagnosis of KS by visualizing defects in olfactory bulbs, sulci and tracts ( 4 ).…”

Section: Discussionmentioning

confidence: 99%

A case of Kallmann syndrome associated with a non-functional pituitary microadenoma

Ach

Marmouch

Elguiche

et al. 2018

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryKallmann syndrome (KS) is a form of hypogonadotropic hypogonadism in combination with a defect in sense of smell, due to abnormal migration of gonadotropin-releasing hormone-producing neurons. We report a case of a 17-year-old Tunisian male who presented with eunuchoid body proportions, absence of facial, axillary and pubic hair, micropenis and surgically corrected cryptorchidism. Associated findings included anosmia. Karyotype was 46XY and hormonal measurement hypogonadotropic hypogonadism. MRI of the brain showed bilateral agenesis of the olfactory bulbs and 3.5 mm pituitary microadenoma. Hormonal assays showed no evidence of pituitary hypersecretion.Learning points:The main clinical characteristics of KS include hypogonadotropic hypogonadism and anosmia or hyposmia.MRI, as a non-irradiating technique, should be the first radiological step for investigating the pituitary gland as well as abnormalities of the ethmoid, olfactory bulbs and tracts in KS.KS may include anterior pituitary hypoplasia or an empty sella syndrome. The originality of our case is that a microadenoma also may be encountered in KS. Hormonal assessment indicated the microadenoma was non-functioning. This emphasizes the importance of visualizing the pituitary region in KS patients to assess for hypoplastic pituitary malformations or adenomas.

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“…Brain MRI is performed at baseline to exclude hypothalamic-pituitary lesions, and to assess defects in the olfactory bulbs, corpus callosum, semicircular canals, cerebellum (207,236) and midline (237). KS patients will typically exhibit unilateral or bilateral olfactory bulb agenesis, olfactory tract agenesis and/or gyrus malformation associated with their anosmia/hyposmia (238). However, a few KS patients have normal olfactory structures despite clinically confirmed anosmia.…”

Section: Testicular Ultrasoundmentioning

confidence: 99%

Clinical Management of Congenital Hypogonadotropic Hypogonadism

et al. 2019

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Reconsidering olfactory bulb magnetic resonance patterns in Kallmann syndrome

Cited by 8 publications

References 15 publications

The diagnostic value of the olfactory evaluation for congenital hypogonadotropic hypogonadism

The diagnostic value of the olfactory evaluation for congenital hypogonadotropic hypogonadism

A case of Kallmann syndrome associated with a non-functional pituitary microadenoma

Clinical Management of Congenital Hypogonadotropic Hypogonadism

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