Perikal Parichay J, Jagannatha Aniruddha Tekkatte, Khanapure Kiran S, Furtado Sunil Valentine, Joshi Krishna Chaitanya, Hegde Alangar Satyaranjandas
Department of Neurosurgery, Ramaiah Institute of Neurosciences, Ramaiah Medical College and Hospitals, Bangalore, India.
Department of Neurosurgery, Ramaiah Institute of Neurosciences, Ramaiah Medical College and Hospitals, Bangalore, India.
World Neurosurg. 2018 Oct;118:304-310. doi: 10.1016/j.wneu.2018.07.115. Epub 2018 Jul 25.
Osmotic demyelination syndrome commonly follows rapid correction of hyponatremia. Although pons is a common location, extrapontine locations, such as striatum and thalamus, have been reported.
A 48-year-old woman presented with masked facies, shuffling gait, and pill-rolling tremors suggestive of acute-onset parkinsonism. Hyponatremia was diagnosed following a bout of diarrhea, which was corrected with hypertonic saline. Magnetic resonance imaging of the brain showed a giant pituitary adenoma. Hyperintensities on T2-weighted imaging were also seen at the level of pons and bilateral striatum. Central pontine myelinolysis and extrapontine myelinolysis were diagnosed. Hormonal assay showed hypocortisolism, secondary hypothyroidism, and hypogonadism. The patient was started on levodopa-carbidopa, steroids, and thyroxine. She underwent transnasal pituitary adenoma excision. At 6 months postoperatively, she had recovered completely with normal gait. Repeat imaging showed complete resolution of myelinolysis. At 36 months, she continued to have hypocortisolism and hypothyroidism requiring replacement.
Extrapontine myelinolysis with parkinsonism and asymptomatic central pontine myelinolysis is rare with few cases described in the literature. Our patient had a pituitary adenoma with hyponatremia requiring sodium correction, and we believe that hypopituitarism might have predisposed her to osmotic demyelination. We reviewed relevant literature on extrapontine myelinolysis in suprasellar tumors and the pathophysiology. Hypopituitarism is an underrecognized cause of hyponatremia. When treating a patient with hyponatremia, knowing the pituitary function status is a prerequisite for the physician to prevent osmotic demyelination syndrome.
渗透性脱髓鞘综合征通常在低钠血症快速纠正后发生。虽然脑桥是常见部位，但也有报道称纹状体和丘脑等脑桥外部位也会出现。
一名48岁女性出现面具脸、拖步和搓丸样震颤，提示急性帕金森病。腹泻后诊断为低钠血症，用高渗盐水进行了纠正。脑部磁共振成像显示巨大垂体腺瘤。在脑桥和双侧纹状体水平的T2加权成像上也可见高信号。诊断为中枢性脑桥髓鞘溶解症和脑桥外髓鞘溶解症。激素检测显示皮质醇缺乏、继发性甲状腺功能减退和性腺功能减退。患者开始服用左旋多巴 - 卡比多巴、类固醇和甲状腺素。她接受了经鼻垂体腺瘤切除术。术后6个月，她步态恢复正常，完全康复。重复成像显示髓鞘溶解完全消退。36个月时，她仍有皮质醇缺乏和甲状腺功能减退，需要替代治疗。
伴有帕金森病的脑桥外髓鞘溶解症和无症状性中枢性脑桥髓鞘溶解症罕见，文献中报道的病例很少。我们的患者患有垂体腺瘤并伴有需要纠正钠水平的低钠血症，我们认为垂体功能减退可能使她易患渗透性脱髓鞘。我们回顾了关于鞍上肿瘤中脑桥外髓鞘溶解症及病理生理学的相关文献。垂体功能减退是低钠血症一个未被充分认识的原因。在治疗低钠血症患者时，了解垂体功能状态是医生预防渗透性脱髓鞘综合征的先决条件。
