Aceruloplasminemia With Psychomotor Excitement and Neurological Sign Was Improved by Minocycline (Case Report).
Aceruloplasminemia With Psychomotor Excitement and Neurological Sign Was Improved by Minocycline (Case Report).
复制标题
DOI：
10.1097/md.0000000000003594
复制
发表时间：
2016-05
期刊：
影响因子：
1.6
通讯作者：
Horiguchi J
中科院分区：
文献类型：
--
作者：
Hayashida M;Hashioka S;Miki H;Nagahama M;Wake R;Miyaoka T;Horiguchi J
无铜蓝蛋白血症是一种由铜蓝蛋白基因突变引起的常染色体隐性遗传性铁代谢疾病。在日本，其患病率为1/2，000，000。这是一种神经退行性疾病，MRI显示铁在大脑中积聚。铁过载诱导氧化应激和活性氧的产生，这触发了导致神经元死亡的一系列病理事件。静脉内施用铁螯合剂去铁胺已被提议作为抑制铁积累的方法。患者是一名46岁的日本女性。她在33岁时被诊断出来。去铁胺给药6个月，但由于不良反应而停药。在46岁入院时，精神兴奋是急性发作的。锥体外系症状反映中脑基底节和黑质铁沉积。共济失调和宽基底门反映了小脑齿状核中的铁沉积。抗生素米诺环素150 mg/天成功改善了临床症状。 米诺环素是第二代四环素，由于其化学结构，具有直接清除自由基的特性。据报道，米诺环素在螯合铁的能力方面与去铁胺相似。米诺环素也参与预防促炎细胞因子的上调。米诺环素的铁螯合作用、抗氧化作用和抗炎作用与此有关。
Aceruloplasminemia is an autosomal recessive disorder of iron metabolism caused by mutations in the ceruloplasmin gene. Its prevalence is 1 in 2,000,000 people in Japan. This is a disorder of neurodegeneration with iron accumulation in the brain revealed by MRI. The iron overload induces oxidative stress and generation of reactive oxygen species, which triggers a cascade of pathological events that lead to neuronal death. Intravenous administration of an iron chelator, deferoxamine has been proposed as a method of inhibiting the accumulation of iron. The patient was a 46-year-old Japanese woman. She was diagnosed at the age of 33 years. Deferoxamine was administrated for 6 months but was discontinued due to adverse effects. On admission at the age of 46, psychomotor excitement was acute in onset. The extrapyramidal symptoms reflected iron deposition in the basal ganglia and substantia nigra in the midbrain. Ataxia and a wide-based gate reflected iron deposition in the dentate nuclei of the cerebellum. An antibiotic, minocycline at 150 mg/day successfully ameliorated the clinical symptoms. Minocycline, a second generation tetracycline, has a direct radical scavenging property due to its chemical structure. It has been reported that minocycline is similar to deferoxamine in its ability to chelate iron. Minocycline is also involved in preventing the upregulation of proinflammatory cytokines. The iron-chelating, antioxidant, and anti-inflammatory effects of minocycline were involved in this case.