Stetkarova IIvana, Svobodova Zuzana, Soltez Jozef, Svatova Jinda
Department of Neurology, 3rd Faculty of Medicine, Charles University and University Hospital Kralovske Vinohrady, Prague, Czech Republic.
Neuro Endocrinol Lett. 2016 Nov;37(6):415-418.
Osmotic demyelination syndrome (ODMS) is a rare and serious neurologic disorder with acute myelin disintegration, usually in the pontine area (central pontine myelinolysis) and to a lesser extent, even in other areas of the central nervous system (extrapontine myelinolysis). The main underlying mechanism is the change of serum osmolality with quick correction of low mineral levels, mainly hyponatraemia. Clinical manifestation is various and depends on the localization.
We describe an acute isolated extrapontine myelinolysis causing acute onset of parkinsonism in a 61-year-old man who developed quickly progressing parkinsonian syndrome after the rapid correction of hyponatraemia.
Brain MRI revealed lesions only in the striatum, sparing the globus pallidus. Substitution therapy with high doses of levodopa significantly improved his clinical condition.
Extrapontine myelinolysis with isolated affection of basal ganglia is extremely rare. In such case, clinical manifestation of acute severe parkinsonism could be successfully treated by high dose of levodopa.
渗透性脱髓鞘综合征（ODMS）是一种罕见且严重的神经系统疾病，伴有急性髓鞘崩解，通常发生在脑桥区域（中枢性脑桥髓鞘溶解），在较小程度上甚至发生在中枢神经系统的其他区域（脑桥外髓鞘溶解）。主要潜在机制是血清渗透压的变化以及低矿物质水平（主要是低钠血症）的快速纠正。临床表现多样，取决于病变部位。
我们描述了一例急性孤立性脑桥外髓鞘溶解症，该疾病导致一名61岁男性急性帕金森综合征发作，该患者在低钠血症快速纠正后出现快速进展的帕金森综合征。
脑部MRI显示仅纹状体有病变，苍白球未受累。高剂量左旋多巴替代治疗显著改善了他的临床状况。
仅累及基底节的脑桥外髓鞘溶解极为罕见。在这种情况下，高剂量左旋多巴可成功治疗急性重度帕金森综合征的临床表现。
