Central Pontine and Extrapontine Myelinolysis ; Frequent association with pituitary-adrenal axis dysfunction

J Korean Neurol Assoc > Volume 14(2); 1996 > Article

Journal of the Korean Neurological Association 1996;14(2): 548-559.

중심성 교뇌 수초용해 및 교뇌외 수초용해

김지수, 전범석, 윤병우, 이한보*, 박성호*, 나덕렬*, 노재규

서울대학교 의과대학 신경과학교실, 서울특별시립 보라매병원 신경과*

Central Pontine and Extrapontine Myelinolysis ; Frequent association with pituitary-adrenal axis dysfunction

JS Kim M.D., BS Jeon M.D., BW Yoon M.D., HB Lee M.D.*, SH Park M.D.*, DR Na M.D.*, JK Roh M.D.

Department of Neurology, Seoul National University. Boramae City Hospital*

Abstract

Objective and Background : Central pontine area is the original and most frequently reported site of cerebral demyelinating lesions from the systemic disorders. Most common underlying systemic disorders are alcoholism, malnutrition and electrolyte imbalance. Recent imaging techniques as MRI helped the antemortem diagnosis, and increasingly showed that the lesion is not limited to the pons. We report our observation of frequent occurrence of extrapontine lesions in this disorder. Furthermore, we also noted very frequent association of pituitary-adrenal axis dysfunction in these patients. Methods and Results . Between 1988-1995, we diagnosed 14 cases of central pontine and extrapontine myelinolysis (CPM and EPM) based on the clinical informations and MRI findings. They were 2 men and 12 women , and the ages ranged from 19 to 73 years. Underlying diseases were Sheehan's syndrome (4), pituitary adenoma(1), sellar chordoma(1), Addison's disease(1), chronic alcoholism(1), diabetes mellitus(1), hepatoma(1), diabetes insipidus(1) and lymphoma (1). Two patients had nausea and vomiting from unknown cause. Four patients had CPM only, but the other 10 patients had extrapontine lesions(5 with EPM only and 5 with combined CPM and EPM).
Conclusion: Extrapontine involvement is frequent (71.4%) . Pituitary-adrenal axis dysfunction was the main underlying disease. Pretreatment with corticosteroid may have a role to prevent the development of demyelination during the correction of hyponatremia. Key Words . Central pontine myelinolysis, Extrapontine myelinolysis, pituitary-adrenal axis dysfunction