MSA多系萎缩.ppt

corticospinal disorder Babinski sign with hyperreflexia Company Logo Company Logo Other features Sleep disorders(REM) Inspiratory sighs Severe dysphonia New or increased snoring Cold hands and feet Pathologic laughter or crying postural/action tremor Company Logo （五）临床分型(Clinical classification) 三组亚型 依据：临床症状 MSA-P型 MSA-C型 MSA-A型 SND OPCA SDS Company Logo （六）辅助检查(auxiliary examination) 卧立位血压：先测卧位血压，站立时血压下降20-40 mmHg或以上，而心率无明显变化为阳性。 正电子发射计算机体层扫描（PET）：能发现纹状体、黑质、橄榄、脑桥和小脑出现代谢降低区。 肌电图：MSA患者的尿道括约肌或肛门括约肌EMG检查发现为神经元性受损。 血液生化检查：血浆去甲肾上腺素含量测定、24小时尿儿茶酚胺含量测定有明显降低。 Company Logo 影像学检查 头颅CT和MRI：可见脑干、小脑萎缩，环池及第 四脑室扩大； MRI有相对特征的表现： T1像壳核、小脑、脑干萎缩，呈稍低信号； T2像壳核、小脑、脑干萎缩，呈稍高信号； 脑桥十字征：是在T2WI上脑桥十字形异常高信号影，其出现机制可能与脑桥核及脑桥横行纤维变性，胶质增生致含水量增加，而由齿状核发出构成小脑上脚的纤维和锥体束未损害有关。不具有特征性。 Company Logo MRI Company Logo 三、诊断(Diagnosis) 临床上根据成年期缓慢起病、无家族史、临床表现为进展的小脑性共济失调、自主神经功能不全和帕金森样等症状及体征，应考虑本病。 2008 Second consensus statement on the diagnosis of multiple system atrophy Company Logo 4 Clinical features Criteria for MSA Company Logo Criteria for possible MSA A sporadic,progressive,adult(30y)-onset disease characterized by Parkinsonism or Cerebellar syndrome and At least one feature suggesting autonomic dysfunction and At least one of additional features additional features of possible MSA Possible MSA-P or MSA-C Babinski sign with hyperreflexia Stridor Possible MSA-P Rapidly progressive parkinsonism Poor response to levodopa Postural instability within 3y of motor onset Gait ataxia,cerebellar dysarthria,limb ataxia,or cerebellar oculomotor dysfunction Dysphagia within 5y of motor onset Atrophy on MRI of putamen,middle cerebellar peduncle,pons,or cerebellum Hypometabolism on PET in putamen,brainstem,or cerebellum Possible MSA-C Parkinsonism(bradykinesia and rigidity) Atrophy on MRI of putamen,middle cerebellar peduncle,or pons Hypometabolism on PET in putamen Criteria for probable MSA A sporadic,progressive,adult(30y)-onset disease characterized by Autonomic failure and