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.

Cognitive impairment in patients with Neuro‐Sjögren

“…This, however, aligns with our previously published cohort of patients with Sjögren’s syndrome and severe neuropathy with a 1:1 female/male ratio. The high fraction of male patients further supports the hypothesis that Neuro‐Sjögren patients present with distinct features in comparison to predominantly rheumatological cohorts of patients with Sjögren’s syndrome 3 …”

“…Sjögren’s syndrome is an autoimmune disease with lymphocytic infiltration and consecutive inflammatory destruction of salivary and lacrimal glands causing the typical sicca symptoms in eyes and mouth 1,2 . Extraglandular neurological manifestations of Sjögren’s syndrome are increasingly recognized, forming the entity of Neuro‐Sjögren 3–7 . While peripheral neuropathy and cranial nerve affection 4,5 are the most prevalent neurological symptoms of Sjögren’s syndrome with rates of up to 60%, there have been several studies suggesting the involvement of the central nervous system through spinal cord involvement, headache, 8 and cognitive dysfunction 9–12 .…”

Lung Involvement in Primary Sjögren's Syndrome—An Under-Diagnosed Entity

“…Its prevalence is estimated at ∼60 cases per 1,00,000 population. The clinical hallmarks are xerophthalmia and xerostomia, however ∼30-50% of patients will develop extra-glandular manifestations in a variety of organ systems (1,2). Lung involvement is relatively common, affecting 9-22% and confers major adverse effects on both life quality and mortality, resulting in a 4-fold increase in 10 years mortality (3,4).…”

Is the taste acuity affected by oral dryness in primary Sjögren’s syndrome patients?

“…Furthermore, other possible aetiologies for the taste impairment in this group remain underexplored. Peripheral neuropathy is a well‐documented symptom in SS patients (Seeliger et al, ; Sivadasan et al, ), but whether this also affects the oral cavity and the tongue in particular is not established. Therefore, a study was designed to investigate the effect of oral dryness and the neurological taste threshold on the taste function in SS patients.…”