MSA多系统萎缩课件.pptVIP

Autonomic failure OH(Orthostatic hypotension) A reduction of systolic blood pressure by at least 30 mm Hg or of diastolic blood pressure by at least 15 mmHg(3min heart rate) asymptomatic or symptomatic syncope GD(Genitourinary dysfunction) earliest symptom male patients erectile dysfunction impotence urinary symptoms urinary urgency or retetion, urinary or fecal incontinence Other symptoms loss of sweating,dry mouth, miosis, Vocal cord palsy is an important and sometimes initial manifestation of the disorder; it may cause dysphonia or stridor and airway obstruction * . * * . Motor disorder * Parkinsonism bradykinesia with rigidity, tremor, or postural instability The tremor is usually irregular and postural/action, often incorporating myoclonus, but a classic pill-rolling rest tremor is uncommon. The parkinsonism can be asymmetric. Postural instability, occurs earlier and progresses more rapidly than in PD. Parkinsonism usually responds poorly to chronic levodopa therapy; up to 30% of patients show a clinically significant, but usually waning； The lack of L-dopa effect is probably attributable to the loss of striatal dopamine receptors. * . * Cerebellar ataxia Ataxia of gait limb ataxia cerebellar dysarthria cerebellar oculomotor dysfunction gaze-evoked nystagmus * . * corticospinal disorder Babinski sign with hyperreflexia * . * * . Other features Sleep disorders(REM) Inspiratory sighs Severe dysphonia New or increased snoring Cold hands and feet Pathologic laughter or crying postural/action tremor * * . （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 * * . （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。 * * . 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