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Diagnosis and Treatment > Signs and Symptoms

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  • Cognitive problems x
  • Gynaecomastia x
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Taieb Ach Departments of Internal Medicine and Endocrinology, University Hospital Fattouma Bourguiba Monastir, Monastir, Tunisia

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Hela Marmouch Departments of Internal Medicine and Endocrinology, University Hospital Fattouma Bourguiba Monastir, Monastir, Tunisia

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Dorra Elguiche Departments of Internal Medicine and Endocrinology, University Hospital Fattouma Bourguiba Monastir, Monastir, Tunisia

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Asma Achour Departments of Internal Medicine and Endocrinology and Radiology, University Hospital Fattouma Bourguiba Monastir, Monastir, Tunisia

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Hajer Marzouk Departments of Internal Medicine and Endocrinology, University Hospital Fattouma Bourguiba Monastir, Monastir, Tunisia

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Hanene Sayadi Departments of Internal Medicine and Endocrinology, University Hospital Fattouma Bourguiba Monastir, Monastir, Tunisia

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Ines Khochtali Departments of Internal Medicine and Endocrinology, University Hospital Fattouma Bourguiba Monastir, Monastir, Tunisia

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Mondher Golli Departments of Internal Medicine and Endocrinology and Radiology, University Hospital Fattouma Bourguiba Monastir, Monastir, Tunisia

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Summary

Kallmann 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.

Taxonomy:
Clinical Overview, Gland/Organ, Pituitary, Topic, Pituitary, Hormone, LH, Testosterone, Condition/ Syndrome, Cryptorchidism, Gynaecomastia, Hypogonadotrophic hypogonadism, Kallmann syndrome, Microadenoma, Pituitary adenoma, Puberty (delayed or absent), Patient Demographics, Age, Adolescent/young adult, Gender, Male, Ethnicity, White, Country of Treatment, Tunisia, Diagnosis and Treatment, Signs and Symptoms, Anosmia, Cognitive problems, Cryptorchidism, Delayed puberty, Gynaecomastia, Hypogonadism, Micropenis, Investigation, Chromosomal analysis, HCG stimulation, LH, MRI, Testosterone, Ultrasound scan, Medication, Testosterone, Related Disciplines, Genetics, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-18-0027
Volume/Issue:
Volume 2018: Issue 1
Open access
J Bukowczan Regional Pituitary Tumour Service, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, NE1 4LP, UK

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K Lois Regional Pituitary Tumour Service, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, NE1 4LP, UK

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M Mathiopoulou Regional Pituitary Tumour Service, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, NE1 4LP, UK

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A B Grossman Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, OX3 7LE, UK

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R A James Regional Pituitary Tumour Service, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, NE1 4LP, UK

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Summary

Giant prolactinomas are rare tumours of the pituitary, which typically exceed 40 mm in their largest dimension. Impairment of higher cognitive function has been noted post-operatively after transcranial surgery and as a long-term consequence of the radiotherapy treatment. However, there has been little that is reported on such disturbances in relation to the tumour per se, and to our knowledge, there has been none in terms of responsivity to dopamine agonist therapy and shrinkage in these tumours. We present a case of successful restoration of severely impaired cognitive functions achieved safely after significant adenoma involution with medical treatment alone.

Learning points

  • Giant prolactinomas can be present with profound cognitive defects.

  • Dopamine agonists remain in the mainstay first-line treatment of giant prolactinomas.

  • Mechanisms of the reversible cognitive impairment associated with giant prolactinoma treatment appear to be complex and remain open to further studies.

  • Young patients with giant prolactinomas mandate genetic testing towards familial predisposition.

Taxonomy:
Clinical Overview, Gland/Organ, Pituitary, Topic, Pituitary, Hormone, FSH, LH, Prolactin, Testosterone, Condition/ Syndrome, Gynaecomastia, Hyperprolactinaemia, Hypogonadism, Pituitary adenoma, Prolactinoma, Quadrantanopia, Patient Demographics, Age, Adolescent/young adult, Gender, Male, Ethnicity, White, Country of Treatment, United Kingdom, Diagnosis and Treatment, Signs and Symptoms, Amnesia, Cognitive problems, Confusion, Erectile dysfunction, Gynaecomastia, Headache, Hyperprolactinaemia, Hypogonadism, Osteopenia, Visual field defect, Investigation, DEXA scan, FSH, LH, MRI, Prolactin, Psychometric assessment, Testosterone, Visual field assessment, Medication, Cabergoline, Dopamine agonists, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-15-0111
Volume/Issue:
Volume 2016: Issue 1
Open access