Koshimura I, Takeda N, Ohtomo T, Shimada J, Sugano K, Mori H, Mizuno Y, Sato K
Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan.
No To Shinkei. 1996 Jan;48(1):81-9.
We report a 32-year-old man who developed cerebellar ataxia and a posterior fossa mass 12 years after the radiation therapy for a cerebellar arteriovenous malformation (AVM). The patient was well until 19 years of the age when he had an acute onset of vertigo and vomiting. A spinal tap was performed and the CSF was bloody. He was admitted to another hospital where an arteriovenous malformation was found in the cerebellum by angiography. Four years after the onset, he developed tingling sensation in the distribution of the second division of the right trigeminal nerve. He was admitted to the neurosurgery service of our hospital where the cerebellar AVM was confirmed. He was transferred to University of California where Bragg peak stereotaxic radiotherapy was successfully performed; this utilizes high energy alpha-ray produced by a cyclotron. Three years after the radiotherapy, marked reduction in the size of the AVM was confirmed by angiography. Twelve years after the onset of his initial symptom, he noted unsteadiness of gait. He was readmitted to our neurosurgery service where obstructive hydrocephalus was found. He was treated by ventriculoperitoneal shunting and placement of a Ommaya reservoir. After these therapy, he noted marked improvement in his gait and ataxia. However, in 1993, his unsteadiness of gait recurred, and he was again admitted to our neurosurgery service on June 20, 1993. On admission, T1-weighted MRI revealed a slightly low signal intensity mass lesion in the right cerebellar hemisphere compressing the brain stem; a spotty high signal intensity lesion and another small low intensity lesion were seen within the mass. Vertebro-basilar angiograms revealed upward displacement of the superior cerebellar arteries. No arteriovenous nidus was visualized. On July, 3rd, the cyst was surgically drained and the Ommaya reservoir was removed. Post-operative course was uneventful, however, he developed head tremor after the surgery. Neurologic examination on July 20, 1993 revealed an alert and well oriented man in no acute distress. General physical examination was unremarkable. Neurologic examination revealed no dementia; higher cerebral functions appeared intact. The optic discs were flat, and visual fields were intact. Ocular movements were full but convergence was restricted. Horizontal gaze nystagmus was noted more in the right lateral gaze. Pupils were intact. Facial sensation and facial muscles were intact. Hearing was normal. His voice was of nasal quality. Pharyngeal reflex was diminished. The tongue showed deviation to the left without atrophy. Head tremor at 5 c/s was noted. He was able to stand with support but was unable to walk. No muscle atrophy or weakness was noted. The finger-to-nose and the heel-to-knee tests showed dysmetria and decomposition more on the right. Rapid alternating movements were ataxic on the right. Muscle tone was diminished on the right. Muscle stretch reflexes were normally elicited and were symmetric. The plantar response was flexor bilaterally. Sensation was intact. On July 21, a posterior fossa exploration was performed. After the surgery, he was treated with 30 mg/day of alotinolol which showed no effect on his head tremor. He was then treated with gradually increasing doses of clonazepam; when he received 8 mg/day of clonazepam, his tremor showed marked improvement. He was discussed in a neurologic CPC on the nature of the posterior fossa lesion and his tremor. Opinions were divided between delayed radiation necrosis and a radiation-induced brain tumor. The chief discussant arrived at the conclusion that the patient had delayed radiation necrosis compressing the brain stem and cerebellar hemispheres. Regarding the nature of his tremor, he thought that his head tremor was of cerebellar type of postural tremor. Histologic examination of the biopsied specimen revealed accumulation of relatively fresh blood constituents in the deep area of the cerebellum forming a mass. Most of the
我们报告一名32岁男性,他在接受小脑动静脉畸形(AVM)放射治疗12年后出现小脑共济失调和后颅窝肿块。该患者在19岁之前情况良好,之后急性起病,出现眩晕和呕吐。进行了腰椎穿刺,脑脊液呈血性。他被收治于另一家医院,经血管造影发现小脑存在动静脉畸形。发病四年后,他右侧三叉神经第二支分布区出现刺痛感。他被收治于我院神经外科,经确认存在小脑AVM。他被转至加利福尼亚大学,成功接受了布拉格峰立体定向放射治疗;该治疗利用回旋加速器产生的高能α射线。放射治疗三年后,血管造影证实AVM大小显著缩小。初始症状出现12年后,他注意到步态不稳。他再次入住我院神经外科,发现存在梗阻性脑积水。他接受了脑室腹腔分流术并置入了Ommaya储液囊。经过这些治疗,他的步态和共济失调有了明显改善。然而,1993年,他的步态不稳再次出现,并于1993年6月20日再次入住我院神经外科。入院时,T1加权磁共振成像显示右侧小脑半球有一个信号强度略低的肿块病变,压迫脑干;肿块内可见一个斑点状高信号强度病变和另一个小的低强度病变。椎基底动脉血管造影显示小脑上动脉向上移位。未发现动静脉畸形团。7月3日,对囊肿进行了手术引流并取出了Ommaya储液囊。术后过程顺利,但他术后出现了头部震颤。1993年7月20日的神经系统检查显示,该男子警觉,定向力良好,无急性痛苦。全身体格检查无异常。神经系统检查未发现痴呆;高级脑功能似乎完好。视盘平坦,视野完整。眼球运动正常,但集合受限。右侧凝视时水平凝视性眼球震颤更明显。瞳孔正常。面部感觉和面部肌肉正常。听力正常。他的声音带有鼻音。咽反射减弱。舌头向左偏斜但无萎缩。发现有5次/秒的头部震颤。他在支撑下能够站立,但无法行走。未发现肌肉萎缩或无力。指鼻试验和跟膝试验显示右侧共济失调和动作分解更明显。右侧快速交替动作共济失调。右侧肌张力降低。肌肉牵张反射正常引出且对称。双侧巴宾斯基征呈屈曲反应。感觉正常。7月21日,进行了后颅窝探查术。术后,他接受了每天30毫克的阿替洛尔治疗,对其头部震颤无效。随后,他接受了逐渐增加剂量的氯硝西泮治疗;当他每天服用8毫克氯硝西泮时,震颤有了明显改善。在一次神经科病例讨论会上,对后颅窝病变的性质及其震颤进行了讨论。对于是迟发性放射性坏死还是放射性诱发的脑肿瘤,存在不同意见。主要讨论者得出结论,该患者患有压迫脑干和小脑半球的迟发性放射性坏死。关于他震颤的性质,他认为他的头部震颤属于小脑型姿势性震颤。活检标本的组织学检查显示,小脑深部区域有相对新鲜的血液成分积聚形成肿块。大多数……