脑桥出血后继发肥大性下橄榄核变性临床和影像学特点及文献回顾

Clinical and Imaging Features of Hypertrophic Inferior Olivary Degeneration Secondary to Pontine Hemorrhage

目的探讨脑桥出血后继发肥大性下橄榄核变性（hypertrophic inferior olivary degeneration，HOD）的临床及影像学特点。方法回顾分析2009年12月～2013年3月北京航空总医院神经内科和北京天坛医院神经内科住院的3例HOD患者的临床及影像资料，3例患者均为男性，表现为肢体不自主多动、腭肌阵挛、走路不稳等锥体外系症状，均完成颅脑计算机断层扫描（computed tomography，CT）或磁共振成像（magnetic resonance imaging，MRI）检查。结果3例患者既往均有脑桥出血史，其中患者1和2为高血压性脑桥出血，患者3为外伤后脑桥出血，临床症状距脑桥出血的时间分别为8个月、12个月和14个月。3例均以锥体外系症状为主要表现，患者1表现为面下颌、躯干、四肢不自主扭转抖动；患者2仅有言语不清及腭肌阵挛；患者3表现为头晕、走路不稳，查体偏侧肢体共济失调。患者1和2出血部位位于脑桥双侧被盖部，患者3位于脑桥左侧被盖部，3例病变部位均为双侧下橄榄核腹外侧孤立局限性病灶。患者1口服氯硝西泮联合丙戊酸钠1周、患者2单纯口服氯硝西泮1周后症状缓解，患者3应用激素治疗2周后症状有所好转，但体征改变不显著。结论 HOD可由脑桥出血继发，脑桥出血治疗病情稳定后若出现锥体外系症状需注意继发HOD的可能。氯硝西泮和丙戊酸钠对症状控制可能有效，激素疗效不确切。

Objective To investigate the clinical and imaging characteristics of hypertrophic inferior olivary degeneration （HOD） after pontine hemorrhage. Methods The clinical data of three patients with HOD secondary to pontine hemorrhage from December 2009 to March 2013 admitted to Aviation General Hospital and Beijing Tiantan Hospital were retrospectively analyzed. All cases were male who showed extrapyramidal symptoms such as non-autonomic hyperactivity of limbs, palatal myoclonus and walking instability and they all underwent computed tomography （CT） or magnetic resonance imaging （MRI） examination of the brain. Results Those three patients all had history of pontine hemorrhage, 2 cases caused by hypertensive, 1 case after trauma. There appeared clinical symptoms and imaging changes respectively in the 8th, 12th and 14th month after brain stem hemorrhage. Extrapyramidal symptoms were the main clinical manifestations in the 3 cases, with glossolalia accompanied by serious involuntary torsional jitter of torso and limbs in case 1 and only glossolalia with obvious palatal myoclonus in case 2. Case 3 showed the original dizzy and walking unsteadily relapsed after improvement. Bleeding occurred on bilateral pontine tegmentum in 2 cases and only unilateral in 1 case. The characteristics of the lesion site were ventrolateral isolated localized lesions in bilateral olive nucleus. Symptoms gradually ease in one week after oral diazepam combined with valproate in case 1 and oral clonazepam in case 2. For case 3 symptoms improved in two weeks after application of hormone therapy without no signiifcant change of signs. Conclusion HOD can be caused by pontine hemorrhage. The possibility of secondary HOD should be considered if tremor jitter of limbs and other extrapyramidal symptoms appear during the pontine hemorrhage treatment in stable condition. Clonazepam and sodium valproate might play an effective role in controlling the symptoms and the effects of hormone therapy effect has not been deifnite.