Hypothalamic–Pituitary Alterations in Patients With Neurosarcoidosis

Julie Martin-Grace,1 Giovanni Murialdo,2 *Gianluca Tamagno3

1. Acute Medical Unit, St Vincent’s University Hospital, Dublin, Ireland
2. Department of Internal Medicine, San Martino Hospital – University of Genoa, Genoa, Italy
3. Department of Endocrinology/Diabetes, Mater Misericordiae University Hospital – University College Dublin, Dublin, Ireland
*Correspondence to gianlucatamagno@tiscali.it

Disclosure: The authors have declared no conflicts of interest.
Received: 20.03.15 Accepted: 15.05.2015
Citation: EMJ Neurol. 2015;3[1]:51-56.

Abstract

Sarcoidosis is a non-caseating, granulomatous inflammatory disorder that can affect the central nervous system (CNS), including the hypothalamic–pituitary region, although rarely. The clinical manifestations of hypothalamic–pituitary neurosarcoidosis are heterogeneous and require a prompt diagnosis to ensure the most appropriate treatment. We have reviewed the cases of neurosarcoidosis affecting the hypothalamic–pituitary axis published since 2002 and compared them with the cases reported in the literature up to 2002, which were previously meta-analysed by our research group. Since 2002, 64 cases were identified in the literature: 37 cases presented with diabetes insipidus, 36 were found to have secondary amenorrhoea, 30 with hypogonadotropic hypogonadism, 17 with hyperprolactinaemia, 15 with thyroid-stimulating hormone deficiency, and 8 cases of panhypopituitarism. Uncommon manifestations included hyperphagia, sudden death, and thermodysregulation. We confirm that neurosarcoidosis affecting the hypothalamic–pituitary axis is an uncommon manifestation of sarcoidosis. Neither changes in the clinical manifestations and diagnosis nor significantly novel management options have appeared in the last decade. While it is a rare disorder, the involvement of the CNS is an indication to treat sarcoidosis and as the symptoms of CNS involvement, including hypothalamic–pituitary alterations, may precede the diagnosis of sarcoidosis, it is important to include neurosarcoidosis in the differential diagnosis of hypothalamic–pituitary axis dysfunction in order to facilitate prompt and appropriate treatment.

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