脑桥出血后继发肥大性下橄榄核变性的临床及影像学特点

摘要

目的 探讨脑桥出血后继发肥大性下橄榄核变性症(hypertrophic inferior olivary degeneration syndrome,HOD)的临床及影像学特点.方法 对2009年1月至2011年6月在我院确诊的脑桥出血的65例,其中63例患者进行为期1年的随访,并对随访过程中发现有继发性下橄榄核变性症的9例患者的临床资料进行分析.结果 HOD发生影像学改变时仅有3例患者有临床症状,主要表现为眼震、软腭阵挛、躯干粗大震颤.脑桥出血均发生在被盖区.头颅MRI下橄榄核信号改变距离出血时间平均为4.05个月.出血病灶9例中有8例存在含铁血红素沉着.肥大的橄榄核直径多数在0.7～ 0.8 cm左右,下橄榄核信号改变6例为等T1WI,长T2WI改变,3例为长T1WI长T2WI改变,其中2例长T1WI患者出现典型临床症状.7例Flair显像为高信号,1例低信号,1例为等信号.治疗上,1例患者先后予森福罗、氯硝西泮、心得安、氟哌啶醇、美多巴等治疗,其中氯硝西泮及心得安有效.结论 HOD多发生在脑桥被盖部出血1个月后,头颅MRI特征为下橄榄核区的圆形的T2WI长信号改变；予心得安及氯硝西泮可能有效.%Objective To investigate the clinical and imaging features of hypertrophic inferior olivary degeneration syndrome (HOD) after pontine hemorrhage. Method All the patients with hypertrophic inferior olivary degeneration after pontine hemorrhage from January 2009 to June 2011 in hospital were enrolled to this study. Clinical manifestations, imaging and treatment were retrospectively observed. Results Nine cases were diagonosed as having HOD. Among them, only three patients with hemorrhage lesions in the tegmental area had clinical symptoms, presenting with dentatorubral tremor, palatal myoclonus and Nystagmus. The changes of MRI signals in the inferior olivary nucleus occurred at an average of 4.05 months following pontine hemorrhage. The hemosiderin staining was evident in eight patients and the size of hypertrophy Olivary was 0.7- 0.8 cm in diameter. The fluid-attenuated inversion-recovery (FLAIR) imaging revealed high signals in seven cases, low signals in one case and equal signals in one case. Patients were treated with pramipexole, clonazepam, propranolol, haloperidol and Madopar. But only clonazepam and propranolol were effective for HOD. Conclusion HOD often occurs one month following cerebral hemorrhage. Long T2 signal in MRI is the characteristic change of HOD and long T1 signal in MRI may indicate serious damage to the olive. Treatment with propranolol or clonazepam may be effective for HOD.