Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome

Abstract

Objective: Sjögren's syndrome is a heterogeneous inflammatory disorder frequently involving peripheral nerves with a wide spectrum of sensory modalities and distribution patterns. The objective of this cross-sectional study was to determine characteristics of Sjögren's syndrome as a cause for severe neuropathy with limb weakness. Methods: One hundred and eighty four patients with polyneuropathy associated with limb weakness underwent routine diagnostics including investigations for Sjögren's syndrome. Forty-four patients with Sjögren's syndrome (ACR-EULAR classification criteria) and severe neuropathy were identified. Results: Sjögren's syndrome was found at a median age of 63 years and the gender distribution showed a balanced female-male ratio of 1:1. Anti-SSA(Ro) antibodies were detected in 48% while seronegative patients were diagnosed with Sjögren's syndrome based on sialadenitis on minor salivary gland biopsy with a focus score ≥1. The majority of patients (93%) were diagnosed with Sjögren's syndrome after neurological symptoms appeared. Limbs were symmetrically involved in 84% of patients (57% tetraparesis, 27% paraparesis). Sensory function was not affected in 11% of patients indicating that Sjögren's syndrome associated neuropathy can present as a pure motor syndrome. Electrophysiological measurements did not reveal pathognomonic findings (23% demyelinating pattern, 36% axonal pattern, 41% both demyelinating and axonal damage signs). More than half of our patients fulfilled the European Federation of Neurological Societies (EFNS) diagnostic criteria for CIDP indicating that distinction between Neuro-Sjögren and other causes of neuropathy such as CIDP is challenging. Interpretation: Our findings show that severe neuropathy with limb weakness is often associated with Sjögren's syndrome. This is of great importance in identifying and understanding the causes of immune mediated polyneuropathy.

Keywords: Neuro-Sjögren; Sjögren's syndrome; chronic inflammatory demyelinating polyneuropathy; motoneuron disease; neuropathy.

Figure 1

Diagnostic approach for evaluation of Sjögren's syndrome in patients with polyneuropathy.

Figure 2

(A) Neuropathy diagnoses prior to Sjögren's syndrome diagnosis. (B) Distribution of electrophysiological damage pattern at initial neurographic analysis.

Figure 3

Distribution of cerebrospinal fluid (CSF) parameters in all study patients: (A) CSF cell counts, (B) CSF total protein, and (C) albumin quotient (CSF albumin/serum albumin × 103). Horizontal bars indicate median values.