A case of primary Sjögren'/INS;s syndrome presented with dysautonomia as initial manifestation

Abstract

Neurological involvement occurs in 10–/INS;42% of the primary Sjö/INS;gren's syndrome (pSS). Distal sensory neuropathy is the most common, pandysautonomia without sensory neuropathy is extremely rare in the pSS as initial manifestation. We describe the case of pSS presented with bladder dysfunction, paralytic ileus and postural intolerance. /INS;A 27-year-old woman developed paresthesia in the both arms 1 week before admission and progressed paresthesia in the legs and trunk below the chest with myalgia. At admission she couldn't urinate and defecate. On examinations she was revealed with /INS;abdominal distension, decreased deep tendon reflexes and sensation for the temperature in the extremities. Autonomic symptoms and signs included no bowel sound during auscultation, increased perspiration on the whole body, bladder distension and postural intolerance with normal pupil sizes and light reflex. Abdomen CT revealed urinary bladder distension and diffuse dilated colon without obstruction suspected paralytic ileus. She had positive anti-SS-A/Ro Ab, anti SS-B/La Ab, positive anti-nuclear antibody (titer 1:40, homogeneous pattern) and anti dsDNA antibody of 38.05 IU, but with negative rheumatoid factor, anti-Sm Ab and Anti-RNP Ab. Salivary scan revealed decreased activity of both the /INS;parotid glands and sublingual glands. Motor and sensory nerve conduction studies of median, ulnar, radial, tibial, peroneal and sural nerves are mild delayed terminal latency with normal amplitude and velocity. During months her symptoms recovered without steroid or IVIG. This case suggests that pSS should be listed in the differential diagnosis of acute autonomic failure as well as chronic form.