The involvement of the hypothalamic and/or the pituitary gland during granulomatous, infiltrative or autoimmune diseases is a rare condition of acquired hypothalamic dysfunction and non-tumoral-non-vascular hypopituitarism. Sarcoidosis is a pathogen-free granulomatous disease which affects both central and peripheral nervous systems in 5-16% of patients. In most cases, nervous involvement by sarcoidosis occurs within a multi-systemic disease, although disease localization limited to the nervous system has also been reported. We observed a 26-year-old Italian woman presenting with low-grade fever, headache, visual disturbances, amenorrhea-galactorrhea syndrome and pituitary failure due to an infiltrative lesion involving the hypothalamus and the pituitary stalk. At first, the diagnosis of "probable" neurosarcoidosis was made according to the clinical picture, magnetic resonance imaging, and the study of cerebrospinal fluid lymphocyte sub-populations. In this case, hyperprolactinemic amenorrhea and galactorrhea combined with blunted responses of gonadotropins to luteinizing hormone releasing hormone and acquired growth hormone failure were to be considered as an infrequent manifestation of neurosarcoidosis limited to the medio-basal brain, without other disease localization. Forty-eight months after disease onset, the occurrence of mediastinal lymph node enlargement and increase of angiotensin converting enzyme in serum allowed us to confirm the diagnosis of sarcoidosis. Neurosarcoidosis is often hard to diagnose, especially when the neurological localization of the disease is not accompanied by other possible systemic signs, and when the lesion is too deep to obtain biopsy confirmation. The study of cerebrospinal fluid and blood lymphocyte sub-populations integrated by magnetic resonance imaging may be helpful for a correct diagnosis.
|Number of pages||7|
|Journal||Annali Italiani di Medicina Interna|
|Publication status||Published - Oct 2001|
ASJC Scopus subject areas
- Internal Medicine