Objective:

To describe a case of inflammatory myelopathy associated with Sjogren's Syndrome in a 73 year old male with features initially suggestive of a spinal arteriovenous fistula (AVF).

Background:

Sjogren’s Syndrome is an autoimmune condition primarily affecting the exocrine glands, however it has been described as a cause of inflammatory myelopathy that can respond to immunotherapy. Chronic progressive myelopathy in older males with low thoracic lesions is typical of spinal AVF, but features can overlap with inflammatory causes.   

Design/Methods:

Case Report

Results:

A 73 year old male presented with progressive gait instability, urinary retention, and leg weakness with claudication evolving over 8 months. T-spine MRI demonstrated a central longitudinally extensive lesion from T6-L1 that was not contrast enhancing. Initial work-up with spinal angiography showed evidence of a Dural AVF originating from the left T9 intercostal artery which was surgically ligated with partial symptom improvement (walking with a walker). Four months later, the patient worsened over 1 month and became wheelchair bound. Repeat angiogram showed no evidence of recurrent or secondary AVF, and MRI showed extension of the T2W hyperintensity to T5-L1. CSF profile demonstrated 4 WBCs, a protein of 154 mg/dl, elevated IgG index, and mirror serum/CSF oligoclonal bands. Serum AQP4-IgG and MOG-IgG were negative. The patient had developed sicca symptoms which persisted for more than 3 months. He also had an elevated SSA. Schirmer’s test was performed and positive. This was followed by a lip biopsy with a FOCUS score of 2 confirming the diagnosis of Primary Sjogren’s Syndrome. The patient was started on steroids and plasmapheresis with partial symptom improvement. 

Conclusions:

Clinicians should consider Sjogren's Syndrome on the differential of patients presenting with progressive myelopathy even in cases with atypical features suggestive of vascular etiologies, and even in groups where Sjogren’s syndrome is less prevalent, as it represents a potentially treatable cause.