Sjogren’s Sensory Neuropathy: A Potentially Treatable Condition with Early Intervention

Objective To describe 2 cases of Sjögren's syndrome sensory neuronopathy (SSSN) in which early intervention resulted in excellent clinical outcomes. Background Acquired sensory neuronopathies (i.e. dorsal root ganglionopathies) are rare sensory neuropathies most commonly associated with Sjögren's syndrome (SS) and paraneoplastic syndromes. Design/Methods We describe 2 patients who presented with painful sensory symptoms, sicca symptoms and with positive SSA and antinuclear antibodies. The first patient presented with perceived symmetric hand weakness (despite no motor nerve conduction abnormalities), total body numbness and paresthesia, as well as sensory ataxia, while the other presented with asymmetric numbness and pain in the left foot and hand. The electrophysiological profiles varied considerably between the 2 patients. The first patient demonstrated complete absence of sensory nerve action potentials (SNAPs) whereas the second patient had only asymmetrical superficial peroneal and sural SNAPs, corresponding with his clinical deficit. The first patient was diagnosed with SSSN, while the other was diagnosed with asymmetrical sensory neuropathy in SS. The first patient was treated with mycophenolate mofetil (MMF) with dramatic symptomatic improvement and near recovery of clinical deficits. The second patient was treated with prednisone, methotrexate, and ultimately switched to MMF and rituximab with significant improvement in symptoms. Results NA. Conclusions SS is commonly associated with sensory neuropathies including sensory neuronopathies. Both patients met diagnostic criteria for SSSN, though with differing severities. To date, there has been no randomized controlled trial evaluating treatments of SSSN. However, our findings suggest that early use of MMF could result in considerable benefit in a disease that is often functionally devastating.