Case Report-OPCA-课件.pptVIP

Rong Rong Radiology Department, Peking University First Hospital 52y, M Major symptoms: dizzy, progressive ataxia, slurring of speech, bradykinesia, dysphagia, problems with balance, bladder and sexual function problems for 3 years and progressive for months anarthria , symptoms of false ball paralyze (Forced crying and forced laughing, stiff-faced, deviated mouth) Stellwag’s sign (+) Muscle spasm heel-knee-tibia test not accurate ?Romberg’s sign failed ankle clonus (+) Bilateral Rossolimo’s sign (+) Chaddock’s sign (+) Babbinski’s sign (+) MRI: atrophy of brain stem and cerebellar EEG: Slightly abnormal (right occipal) Hu(-), Yo(-), Ri(-) Localization diagnosis: (1)cerebellar: balance problems, ataxia, cerabellar atrophy; (2) vegetative nervous system: bladder dysfunction; (3) bilateral pyramidal tract: slurring dysfunction, forced crying and laughing, false ball paralyze, possitive pathologic sign; (4) extrapyramidal system: bradykinesia, stiff-faced, muscle spasm Qualitative diagnosis: multiple system atrophy Clinical evidence: Middle aged male, latent, chronic, slow progress, ataxia, anarthria, with vegetative nerves system, extrapyramidal system and pyramidal system disease MRI Olivopontocerebellar atrophy 容蓉 医学影像科 2010-08-27 是一种引起脊髓以上大脑深部区域萎缩的疾病 遗传 散发 散发病例的病因尚未明确 渐进性恶化 男性发病稍高于女性 OPCA是主要累及小脑、脑干和脊髓的进行性神经变性病 遗传和散发两类，临床有多种类型和变异型 运动障碍、感觉障碍和颅神经损害 首发症状以进行性共济失调及双下肢无力者最多,眼球运动障碍也较为常见 主要症状：平衡障碍，构音障碍，运动障碍 其他症状：异常眼球运动，异常运动，肠道或膀胱问题，吞咽困难，肌痉挛，肌肉紧张，神经损害，震颤，性功能障碍 Menzel型，常染色体显性遗传 Fickler-Winkler型，常染色体隐性遗传 Schut-Haymaker型，常染色体显性遗传 伴有视网膜变性，常染色体显性遗传 伴痴呆、眼外肌麻痹、锥体外系体征者，常染色体显性遗传 其他，如伴第5组症状的常染色体隐性遗传和散发病例。儿童期发病，大多数病例呈缓慢进展的病程，首发症状以进行性共济失调及双下肢无力者最多，眼球运动障碍也较为常见。婴儿橄榄体脑桥小脑萎缩极其罕见，以快速退变、并在发病后数月或数年内死亡为特征 小脑半球、桥脑腹侧、延髓橄榄核严重萎缩，红核、黑质、基底节、大脑皮质及脊髓前角也受累 CT：桥脑和小脑中脚萎缩，桥脑周围池扩大，小脑中脚可有低密度区。第四脑室扩大，桥脑-小脑角池扩大。小脑沟增宽，大脑沟亦可轻度增宽 MR：①脑干形态变细，尤以桥脑前后径变小更为明显；②小脑体积变小，小脑沟裂增宽加深，半球小叶变细变直，呈枯树枝状；③脑池及脑室扩大，其中以桥前池增宽最为明显。T1WI 正中矢状面对脑干及小脑萎缩的显示十分清楚。T2WI：桥脑的十字形高信号（“十字征”） Mario Mascalchi et. al. Proton MR Spectrosc