Hypopituitarism with Moyamoya Disease Involving the Posterior Pituitary

Abstract

ABSTRACT: Objective: To describe a case of moyamoya disease associated with hypopituitarism.Methods: We present a case of moyamoya disease presenting with hypogonadotrophic hypogonadism and central diabetes insipidus and review the literature.Results: A 22-year-old female presented with secondary amenorrhea with loss of secondary sexual characteristics. Her follicle-stimulating hormone and luteinizing hormone levels were 1.4 mIU/mL and <0.1 mIU/mL, respectively. The results of water deprivation test indicated central diabetes insipidus. Her thyroid-stimulating hormone, thyroxine, and post-Synacthen cortisol levels were normal, and her prolactin was measured as 52.2 ng/mL (normal <20 ng/mL). Contrast-enhanced magnetic resonance imaging showed a partial empty sella with enhanced linear and curvilinear structures in the suprasellar cistern. Magnetic resonance angiography showed narrowing of the apices of the bilateral intracranial carotid, middle cerebral, and anterior cerebral arteries. It also showed collaterals of the middle cerebral artery, predominantly on the left side.Conclusion: Moyamoya disease with hypopituitarism involving the posterior pituitary is rare but can be the result of ischemia due to internal carotid artery narrowing.