Bickerstaff's脑干脑炎继发肥大性下橄榄核变性的临床及影像学特点(附1例报告)

Clinical and imaging features of hypertrophic olivary degeneration secondary to Bickerstaff's brainstem encephalitis (report of 1 case)

目的探讨Bickerstaff's脑干脑炎(BBE)继发肥大性下橄榄核变性(HOD)的临床及影像学特点。方法回顾性分析1例BBE继发HOD患者的临床资料。结果本例患者先以复视、构音障碍、口角歪斜等多脑神经受损症状及共济失调为主要表现,后出现饮水呛咳,伸舌受限,软腭、舌肌、面肌震颤及肌张力增高等延髓及锥体外系症状;头颅MRI示入院后第2d时脑干及小脑异常信号并强化,入院后第17d时出现延髓双侧下橄榄核区局限性肥大,且双侧下橄榄核区异常信号较前明显增大。结论 BBE继发HOD的临床表现以多脑神经受损症状、共济失调及锥体外系症状为主,影像学改变为脑干、小脑及下橄榄核区异常信号和下橄榄核区局限性肥大。

Objective To investigate the clinical and imaging characteristics of hypertrophic olivary degeneration （HOD） secondary to Bickerstaff＇s brainstem encephalitis（BBE） . Methods The clinical data of one patient with HOD secondary to BBE was retrospectively analyzed. Results The main symptoms of the case were symptoms of multiple cranial nerves damaged like diplopia,dysarthria and distortions of commissure,and ataxia; then the main symptoms were medulla and extrapyramidal symptoms like irritating and cough with drinking,limited of tongue extension,tremor of palatal,tongue muscle and facial muscle,and hypermyotonia. MRI showed abnormal signals and contrast enhancement at brain stem and cerebellum at 2 d after admission,and then showed the localized hypertrophy at inferior olivary nucleus,and the abnormal signals of inferior olivary nucleus increased obviously at 17 d after admission. Conclusion The main clinical manifestation of HOD secondary to BBE seems to be lots of cranial nerves damaged symptoms,ataxia and extrapyramidal symptoms. The imaging features are abnormal signals at brain stem,cerebellum and inferior olivary nucleus,and localized hypertrophy at inferior olivary nucleus.