Progressive numbness of distal limbs for two years, unsteady gait for two months

Abstract

A 50-year-old female was admitted to our department, complaining of progressive numbness of distal limbs for two years and unsteady gait for two months. “Peripheral neuropathy” was the presumed diagnosis. She has suffered dry mouth for months. Neurological examination revealed proximal upper muscle strength was normal and distal was 5-/5 while muscle strength in lower limbs was normal. Tendon reflexes in all limbs were reduced, and superficial sensation as well as deep sensation in all limbs was also diminished. Deep sensation below T 8-10 was diminished. Romberg’s test was positive with negative pathological reflex. Several sensory nerves action potentials (SNAPs) were diminished or absent with normal compound muscle action potentials (CMAPs). Cervical MRI showed hyperintensities in the dorsal column. Serum anti-Ro/SSA antibody was positive. Tear break-up time was abnormal in either eye (5s, normal range>10s); the rate of saliva production declined 0.02 ml/min (> 1.50 ml/15 min); parotid gland contrast sialography was abnormal; lip biopsy was positive with focal lymphocytic sialadenitis with focus score ≥1. The patient was diagnosed as primary Sjogren's syndrome and sensory neuronopathy. She received oral prednisone in dose of 1mg/(kg·d) for four weeks, then reduce the dosage with 5mg/w to 0.50mg/ (kg·d). Later she reduced the dosage with 2.5mg/per week. At the same time, she got cyclophosphamide (100mg every other day) and hydroxychloroquine (0.20g twice a day). Numbness of limbs and unsteady gait were improved when the patient was discharged. Two month later, during the follow-up, the patient’ gait was slightly improved, but the numbness still existed. DOI: 10.3969/j.issn.1672-6731.2016.11.016