Müller-Vahl K R, Müller-Vahl H G
Neurologische Klinik mit Klinischer Neurophysiologie, Medizinische Hochschule Hannover.
Dtsch Med Wochenschr. 1998 Nov 6;123(45):1343-6. doi: 10.1055/s-2007-1024185.
Two weeks after a mild viral infection a previously healthy 22-year-old woman developed ileus, a severe abnormality of orthostasis, sicca (Sjögren's) syndrome, bilateral miosis and generalized hyphidrosis. Pelvic endoscopy and laparotomy failed to clarify the cause of the mechanical ileus.
Neurological examination revealed an isolated abnormality of the autonomic system, involving both sympathetic and parasympathetic components. Schellong's, Schirmer's and the ninhydrin tests were markedly abnormal. There was no heart rate variation on breathing and a post-Valsalva hypotensive blood pressure overshoot. Further tests failed to find a cause of the neuropathy.
DIAGNOSIS, TREATMENT AND COURSE: The diagnosis of idiopathic panautonomic neuropathy (pandysautonomia) was made. The ileus and hypotension were treated symptomatically with neostigmine, cisapride, midodrine and, initially, with enemas, nasogastric tube feeding and parenteral fluids. The patient was free of symptoms at follow-up examination a year later.
Idiopathic autonomic neuropathy should be considered in the differential diagnosis of functional abnormalities of the sympathetic and/or parasympathetic nervous system, especially in previously healthy young people, in the presence of orthostatic, unexplained gastrointestinal and hidrotic symptoms.
一名既往健康的22岁女性在轻度病毒感染两周后出现肠梗阻、严重直立性异常、干燥（舍格伦）综合征、双侧瞳孔缩小及全身少汗。盆腔内镜检查和剖腹手术未能明确机械性肠梗阻的病因。
神经系统检查显示自主神经系统存在孤立性异常，涉及交感和副交感神经成分。谢隆试验、施密特试验和茚三酮试验均明显异常。呼吸时心率无变化，瓦尔萨尔瓦动作后血压出现低血压过冲。进一步检查未发现神经病变的病因。
诊断、治疗与病程：诊断为特发性全自主神经病变（全自主神经功能不全）。肠梗阻和低血压采用新斯的明、西沙必利、米多君进行对症治疗，最初还采用灌肠、鼻饲和静脉补液。一年后的随访检查时患者无症状。
在鉴别诊断交感和/或副交感神经系统功能异常时，尤其是在既往健康的年轻人中，出现直立性、不明原因的胃肠道和出汗症状时，应考虑特发性自主神经病变。
