Endocrinol Metab > Volume 25(2); 2010 > Article
Endocrinology and Metabolism 2010;25(2):142-146.
DOI: https://doi.org/10.3803/EnM.2010.25.2.142    Published online June 1, 2010.
A Case of Kallmann's Syndrome with Frontal Lobe Atrophy and Mental Retardation.
Soyoung Hyun, Seungguk Park, Dong Gu Kang, Seung Uk Jeong, Dea Ho Lee, Gwanpyo Koh
1Department of Internal Medicine, Jeju National University School of Medicine, Jeju, Korea. okdom@medmail.co.kr
2Department of Family Medicine, Jeju National University School of Medicine, Jeju, Korea.
Abstract
Kallmann's syndrome is a rare condition, and this is defined as hypogonadotropic hypogonadism and anosmia or hyposmia. The syndrome may be associated with cleft lip, cleft palate, color blindness, skeletal abnormalities, renal agenesis, sensory neural hearing loss, obesity, etc. About 10 cases of Kallmann's syndrome have been reported in Korea, but there are no reports on cases of Kallmann's syndrome with atrophy of the frontal lobe, severe mental retardation and unilateral renal agenesis. We experienced a case of 17-year-old boy with abnormalities of the olfactory system, as was noted on magnetic resonance imaging (MRI). He had an atrophy of the frontal lobe, mental retardation, a micropenis and unilateral renal agenesis. Hormonal assay documented low levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone and thyroid-stimulating hormone (TSH). So, we report here on an unusual case of Kallmann's syndrome along with briefly reviewing the relevant medical literature.
Key Words: Frontal lobe atrophy, Kallmann's syndrome, Mental retardation


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