Wright R A, Grant I A, Low P A
Department of Neurology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA.
J Auton Nerv Syst. 1999 Jan 15;75(1):70-6. doi: 10.1016/s0165-1838(98)00164-7.
We retrospectively studied 52 patients with sicca complex who presented primarily with peripheral neuropathic symptoms to assess the degree of autonomic involvement and its relationship to somatic neuropathy, serological tests and extraglandular disease. One patient presented with severe panautonomic dysfunction, with another six patients reporting prominent autonomic symptoms, and a further 40 patients having mild or asymptomatic disease. Eighty-seven percent of patients showed abnormalities on an autonomic reflex screen (ARS). Fifty-eight percent of patients showed a mixed pattern of autonomic dysfunction with abnormalities in two or more ARS subgroups of sudomotor, cardiovagal and adrenergic function. Three patients had a cholinergic autonomic neuropathy with sparing of adrenergic function. Tonic pupils were present in 13 patients. Autonomic neuropathy occurred in patients with a variety of somatic neuropathy subtypes, without a clear relationship between the type of autonomic neuropathy and the type of somatic peripheral neuropathy. Extraglandular disease was uncommon, occurring in only four patients. Serological abnormalities (extractable nuclear antigen [ENA], SS-A, SS-B) occurred in 20% of patients. We conclude that: (1) A wide spectrum of autonomic dysfunction is seen in sicca complex. (2) Severe autonomic involvement is uncommon, with most patients showing mildly symptomatic or asymptomatic disease. (3) Overall, characteristic patterns of autonomic dysfunction did not predominate, and there were no clear relationships with the different types of somatic peripheral neuropathy. (4) Clinical and serological evidence of non-neurological extraglandular disease is uncommon in this group of patients.
我们回顾性研究了52例主要表现为周围神经病变症状的干燥综合征患者，以评估自主神经受累程度及其与躯体神经病变、血清学检查和腺体外疾病的关系。1例患者出现严重的全自主神经功能障碍，另有6例患者报告有明显的自主神经症状，还有40例患者有轻度或无症状疾病。87%的患者在自主神经反射筛查（ARS）中显示异常。58%的患者表现为自主神经功能障碍的混合模式，在泌汗运动、心血管迷走神经和肾上腺素能功能的两个或更多ARS亚组中存在异常。3例患者患有胆碱能自主神经病变，肾上腺素能功能未受累。13例患者出现强直性瞳孔。自主神经病变发生在各种躯体神经病变亚型的患者中，自主神经病变类型与躯体周围神经病变类型之间无明确关系。腺体外疾病不常见，仅4例患者出现。20%的患者出现血清学异常（可提取核抗原[ENA]、SS-A、SS-B）。我们得出以下结论：（1）干燥综合征患者可见广泛的自主神经功能障碍。（2）严重的自主神经受累不常见，大多数患者表现为轻度症状或无症状疾病。（3）总体而言，自主神经功能障碍的特征性模式并不占主导，且与不同类型的躯体周围神经病变无明确关系。（4）在这组患者中，非神经腺体外疾病的临床和血清学证据不常见。
