[Autonomic dysfunction in sensory ataxic neuropathy with Sjögren's syndrome]

Sjögren's syndrome (SjS) is occasionally associated with chronic progressive sensory neuropathy, and its major pathology is suggested to be dorsal root ganglionitis with the T-lymphocyte invasion. Autonomic dysfunction is frequently accompanied by SjS-associated sensory neuropathy but its underlying pathology is uncertain. We reported four patients with SjS-associated sensory ataxic neuropathy who satisfied the diagnostic criteria of Sjögren's syndrome and also showed a wide variety of autonomic disturbances. The sensory involvement was initially those of multiple mononeuritis in the distal extremities, but in the advanced stage it involved the area distributed along the spinal segmental dermatomes particularly in the trunk. Complete anhidrosis with discrete segmental distribution, as well as absence of response to cholinergic agents, was observed in three cases. Surface skin temperature recorded by a thermoviewer also showed a segmental involvement along the spinal dermatomes in two cases. Adie's pupils were present in two cases. These features suggested that the segmental involvement of the postganglionic sympathetic ganglion cells could be present in the underlying pathological condition. As for the other autonomic dysfunction, two cases showed severe postural hypotension and three cases had an abnormal heart rate variation (R-R interval). Our cases indicated that SjS-associated sensory ataxic neuropathy also manifest variable autonomic disturbances and suggested that similar pathological process to that involving the dorsal root ganglia would be present in the pathology of sympathetic and ciliary ganglion cells.