Endocrine aspects of neurosarcoidosis

Abstract

The involvement of the hypothalamus and/or pituitary gland by granulomatous, infiltrative or autoimmune diseases is a rare condition of non-tumoral-non-vascular acquired hypothalamic dysfunction and hypopituitarism. In this paper, we present the case of a 26-year-old woman, who showed an amenorrhea-galactorrhea syndrome with hypogonadotropic hypogonadism due to an isolated hypothalamic-peduncular localization of neurosarcoidosis. Acquired GH deficiency was also demonstrated. This clinical case provided the opportunity for a review of the endocrine aspects linked to brain infiltrative diseases that may affect the hypothalamic-pituitary function, with a focus upon neurosarcoidosis. Sarcoidosis is a pathogen-free granulomatous disease that affects both the central and peripheral nervous system in 5-16% of patients. In most cases, such involvement by sarcoidosis occurs within a multi-systemic disease, but disease localization limited to the nervous system may also be observed. Endocrine manifestations of neurosarcoidosis disclose "chameleon-like" clinical pictures, which are usually expressed by the evidence of hypothalamic dysfunction, diabetes insipidus, adenopituitary failure, amenorrhea-galactorrhea syndrome, in isolated fashion or variedly combined. More rarely, inappropriate anti-diuretic hormone secretion, isolated secondary hypothyroidism, adrenal insufficiency or altered counter-regulation of glucose homeostasis have been reported. Neurosarcoidosis is often hard to diagnose, especially when the neurological localization of the disease is not accompanied by other systemic localizations or by specific signs of the disease, and when the lesion is too deep to obtain bioptic confirmation. The study of cerebrospinal fluid and blood lymphocyte sub-populations, integrated by MRI and nuclear scans (67GalIium uptake and 111Indium-pentetreotide, Octreoscan), may be helpful for a correct diagnosis. Therapy with corticosteroid and immunosuppressive drugs, such as cyclosporine A, and other treatment approaches to neurosarcoidosis are also accounted for.

Sarcoid granulomas in the parathyroid gland - a case of dual pathology: hypercalcaemia due to a parathyroid adenoma and coexistent sarcoidosis with granulomas located within the parathyroid adenoma and thyroid gland

“…Further endocrine manifestations reported in association with sarcoidosis involve the hypothalamus, pituitary and adrenal glands [26]. In our case, the identification of granulomas within the thyroid gland, a rarely reported occurrence, had no clinical significance.…”

An Elderly Patient with Sarcoidosis Manifesting Panhypopituitarism with Central Diabetes Insipidus

“…Based on the review on 285 patients with sarcoidosis (mean age: 43.7, male to female ratio 5/4) [6], mean age of 14 patients with neurosarcoidosis was 44 years old and female patients were predominant (male to female ratio 4/10). Murialdo and Tamagno have specifically reviewed 91 patients with neuroendocrine dysfunctions due to neurosarcoidosis, showing that the age of patients with endocrine manifestations related to hypothalamo-pituitary region ranged from 10 to 66 years (mean age: 33 years) without gender prominence [2].…”

“…Among the various symptoms attributed to neuroendocrine sarcoidosis, polyuria and polydipsia are the most frequent manifestation, as reported in approximately 30% of patients with sarcoidosis involving the central nervous system [2][3][4][5]. However, considering that the central nervous system is affected only in 5% of all cases of sarcoidosis, diabetes insipidus resulting from sarcoidosis is still an uncommon complication [6,7].…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…Cranial neuropathies occur in up to 75% of patients and most commonly involve the optic nerve, although involvement of the facial nerve is considered classic [179,180,182]. Endocrine abnormalities are common in sarcoid and are related to the fact that it tends to involve the hypothalamus and pituitary [183]. Typical findings include diabetes insipidus, adrenal insufficiency, and amenorrhea-galactorrhea syndrome [183].…”

“…Endocrine abnormalities are common in sarcoid and are related to the fact that it tends to involve the hypothalamus and pituitary [183]. Typical findings include diabetes insipidus, adrenal insufficiency, and amenorrhea-galactorrhea syndrome [183]. Myelopathy may occur due to transverse myelitis or granulomatous invasion of the spinal cord itself or of the intradural or extradural space [83,184].…”