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Journal of the Korean Neurological Association 2000;18(4): 420-424.
신경병증에 나타난 긴장성동공
울산대학교 의과대학 서울중앙병원 신경과학교실
Tonic Pupil in Neuropathy
Kwang-Kuk Kim, M.D.
Department of Neurology, College of Medicine, University of Ulsan, Asan Medical Center
Abstract
B a c k g r o u n d : The clinical and laboratory findings of five patients with tonic pupil (TP) and neuropathy were reviewed for the comprehension of pathogenesis of TP in neuropathy. Methods : Immunological and nerve conduction studies (NCS) were performed in three patients with Sjogren’s syndrome (SS), Miller-Fisher syndrome (MFS), and Adie’s syndrome. Results : Upon initial examination, there were no definite sicca syndromes in patients of SS, despite intolerable sensory symptoms. The TP in MFS was improved after intravenous immunoglobulin. Of the cranial neu-ropathies, trigeminal sensory neuropathy was frequent finding. Deep tendon reflexes were absent in all five patients.Absent sensory nerve action potentials and prolonged R1 and R2 of the blink reflex were detected in two SS patients with syncope and asymmetric sensory loss. Conclusions : These findings in SS patients implicated the possibility of a selective lesion at the level of the dorsal root- or trigeminal- or autonomic- ganglions complicating the TP. In view of the sensory ataxia, opthalmoplegia, areflexia, slow and decreased sensory NCS in the extremity and prolonged R1 and R2, a demyelinating process of postganglionic parasympathetic nerves were suspected to be the cause of the tonic pupil in MFS. Adie’s syndrome along with flushing of the left side of the face and chest after exercise, suggested segmental postganglionic lesions of the sympathetic and parasympathetic peripheral nervous systems. In patients with complicat-ing TP and asymmetric progressive sensory neuropathy, the SS has to be considered even if the patient denies the pres-ence of sicca symptoms at first and SS-A/SS-B autoantibody is negative. J Korean Neurol Assoc 18(4):420~424, 2000 Key Words : Tonic pupil, Sjogren’s syndrome, Autoantibodies, Fisher syndrome