Becker K, Keuser R, Weber M, Steffen H M
Klinik II und Poliklinik für Innere Medizin, Universität zu Köln.
Med Klin (Munich). 1995 Jul 15;90(7):398-402.
Vertigo and syncope often occur during orthostatic conditions. A possible cause is asympathicotonic dysregulation with a fall of blood pressure and no increase in heart rate. After the exclusion of secondary disturbances of autonomic innervation. g. in diabetes mellitus, and pharmacological side effects, e.g. tricyclic antidepressants, primary reasons must be considered. We present 2 cases of primary autonomic failure.
Patient 1 had Parkinson-syndrome, impaired bladder function and orthostatic dysregulation with a maximal drop of blood pressure to 60/40 mm Hg. Patient 2 showed orthostatic dysregulation with a minimal blood pressure of 70/30 mm Hg without further symptoms, in a tilt table examination catecholamines were measured during orthostatic conditions. Patient 1 had normal, and patient 2 very low resting catecholamine plasma levels. Neither patient reacted with a rise of plasma catecholamines when tilted to an upright position. These findings are diagnostic of multiple system atrophy (MSA) in patient 1 and pure autonomic failure (PAF) in patient 2.
In PAP only dysautonomic symptoms are found. It has a favourable prognosis. MSA consists of an autonomic dysregulation in combination with parkinsonism, cerebellary and bulbar symptoms, prognosis quo ad vitam is poor. Diagnosis is often established by a tilt table examination and radiology findings, and is often late. Treatment of both diseases consists of pharmacological, e.g. fludrocortisone, vasopressin analogues and ergotamine tartrate as well as non-pharmacological blood pressure stabilizing measures.
直立位时眩晕和晕厥常易发生。一个可能的原因是交感神经张力失调伴血压下降且心率无增加。排除自主神经支配的继发性紊乱,如糖尿病中的紊乱,以及药物副作用,如三环类抗抑郁药的副作用后,必须考虑原发性原因。我们报告2例原发性自主神经功能衰竭病例。
患者1患有帕金森综合征、膀胱功能受损以及直立位失调,血压最大降幅达60/40 mmHg。患者2表现为直立位失调,最低血压为70/30 mmHg,无其他症状,在倾斜试验中于直立位时测量儿茶酚胺。患者1静息时血浆儿茶酚胺水平正常,患者2则极低。两名患者在倾斜至直立位时血浆儿茶酚胺均未升高。这些发现诊断患者1为多系统萎缩(MSA),患者2为纯自主神经功能衰竭(PAF)。
在PAF中仅发现自主神经功能障碍症状。其预后良好。MSA包括自主神经失调并伴有帕金森症、小脑和延髓症状,生命预后较差。诊断通常通过倾斜试验和影像学检查确定,且往往较晚。这两种疾病的治疗包括药物治疗,如氟氢可的松、血管加压素类似物和酒石酸麦角胺,以及非药物性血压稳定措施。