Das Liza, Bhansali Anil, Ahuja Chirag Kamal, Korbonits Márta, Dutta Pinaki
Department of Endocrinology, PGIMER, Chandigarh, India.
Department of Radiology, PGIMER, Chandigarh, India.
AACE Clin Case Rep. 2020 May 4;6(5):e207-e211. doi: 10.4158/ACCR-2020-0094. eCollection 2020 Sep-Oct.
Vasculotoxic envenomation is an uncommon cause of hypopituitarism. Most described cases have varying extent of anterior pituitary dysfunction, but posterior pituitary involvement is extremely rare.
Clinical, biochemical, and radiologic evaluation of a young female who presented with secondary amenorrhea was performed. A brief literature review of envenomation-induced hypopituitarism is included.
A 26-year-old female presented with secondary amenorrhea since the age of 20 years. She had normal stature. Her past medical history was significant for a vasculotoxic snakebite 12 years back requiring hemodialysis, but no hormonal testing was done at that time. Current evaluation showed anterior hypopituitarism. An insulin-induced hypoglycemia test confirmed deficiencies of cortisol and growth hormone axes (peak values 348 nmol/L and 0.03 ng/mL). There was no diabetes insipidus. Magnetic resonance imaging revealed a hypoplastic anterior pituitary with an ectopic posterior pituitary. In view of normal stature and secondary amenorrhea, a diagnosis of envenomation-induced hypopituitarism with ectopic posterior pituitary (EPP) was made. A brief literature review of envenomation-induced hypopituitarism showed both acute and delayed presentation, male predominance, and variable lag period (weeks to years). Nearly half of all patients were asymptomatic. The most common axis involved in acute presentation was the cortisol axis, whereas the thyroid and gonadotroph axes were commonly involved in delayed hypopituitarism.
Vasculotoxic envenomation is a rare cause of acquired hypopituitarism. EPP in the index case was probably due to the "axonal dieback" phenomenon and subsequent regeneration of the axons at a more caudal site. This case, being the first instance of acquired EPP following envenomation, expands the spectrum of envenomation-induced hypopituitarism.
血管毒性蛇咬伤是垂体功能减退症的一种罕见病因。大多数已报道的病例存在不同程度的腺垂体功能障碍,但神经垂体受累极为罕见。
对一名出现继发性闭经的年轻女性进行了临床、生化及影像学评估。并对蛇咬伤所致垂体功能减退症进行了简要文献回顾。
一名26岁女性自20岁起出现继发性闭经。她身材正常。其既往病史中,12年前曾被血管毒性蛇咬伤,当时需要进行血液透析,但当时未进行激素检测。目前评估显示为腺垂体功能减退。胰岛素诱发低血糖试验证实皮质醇和生长激素轴存在缺陷(峰值分别为348 nmol/L和0.03 ng/mL)。无尿崩症。磁共振成像显示腺垂体发育不全,神经垂体异位。鉴于身材正常及继发性闭经,诊断为血管毒性蛇咬伤所致垂体功能减退症伴神经垂体异位(EPP)。对蛇咬伤所致垂体功能减退症的简要文献回顾显示,其表现有急性和延迟性,男性居多,且潜伏期长短不一(数周至数年)。几乎一半的患者无症状。急性发病时最常受累的轴是皮质醇轴,而甲状腺轴和促性腺激素轴在延迟性垂体功能减退症中常见受累。
血管毒性蛇咬伤是后天性垂体功能减退症的罕见病因。该病例中的神经垂体异位可能是由于“轴突回退”现象以及随后轴突在更靠尾端的部位再生所致。该病例作为蛇咬伤后获得性神经垂体异位的首例,拓展了蛇咬伤所致垂体功能减退症的范围。