Tsuchiya K, Watabiki S, Siojiri T, Sato T
Department of Neurology, Musashino Red Cross Hospital, Tokyo, Japan.
No To Shinkei. 1992 Oct;44(10):893-8.
Three cases (case 1, female, aged 30; case 2, male, aged 32; case 3, male, aged 34) of benign brainstem encephalopathy with truncal ataxia were reported. Two patients had prodromal symptoms Neurological examination revealed truncal ataxia in all cases. As additional neurological signs, anisocoria, mydriasis, nystagmus, ptosis, transient opsoclonus, and facial palsy were seen. There was neither drowsiness nor myoclonus in the three cases. On laboratory examinations, cold agglutination test revealed significant elevation in two cases. The examination of cerebrospinal fluid showed a moderate rise of proteins in one case, but did not revealed pleocytosis in any of the cases. Magnetic resonance imaging of one patient revealed an area of high intensity in the left pontine tegmentum by T2-weighed imaging. The prognosis for all these cases was good, and the reappearance of neurological signs was not present until now. Our cases were different from brainstem encephalitis (Bickerstaff's encephalitis) because of an absence of disturbed consciousness and no pleocytosis in the cerebrospinal fluid. Our cases were also different from "myoclonus-opsoclonus syndrome" because of an absence of myoclonus. We discussed a possibility of a new clinical syndrome which we call "benign brainstem encephalopathy with truncal ataxia".
报告了3例(病例1,女性,30岁;病例2,男性,32岁;病例3,男性,34岁)伴有躯干共济失调的良性脑干脑病。2例患者有前驱症状。神经系统检查显示所有病例均有躯干共济失调。作为额外的神经系统体征,可见瞳孔不等大、瞳孔散大、眼球震颤、上睑下垂、短暂性眼球阵挛和面瘫。3例患者均无嗜睡和肌阵挛。实验室检查中,冷凝集试验显示2例显著升高。脑脊液检查显示1例蛋白质中度升高,但所有病例均未发现细胞增多。1例患者的磁共振成像T2加权像显示左侧脑桥被盖区有高强度区域。所有这些病例的预后良好,至今未出现神经系统体征复发。我们的病例与脑干脑炎(比克斯特法夫脑炎)不同,因为没有意识障碍且脑脊液中无细胞增多。我们的病例也与“肌阵挛 - 眼球阵挛综合征”不同,因为没有肌阵挛。我们讨论了一种新的临床综合征的可能性,我们称之为“伴有躯干共济失调的良性脑干脑病”。