Two cases of primary Sjögren's syndrome with neurological impairment as initial symptom

Abstract

Objective·To study the clinical features of two primary Sjögren's syndrome (pSS) patients with neurological symptoms as initial manifestations and review the related literature.Methods·The clinical data, response to treatment as well as prognosis of 2 cases were analyzed and followed up.Results·Case 1, female, initially presented progressive gait instability and distal sensory impairment at the age of 50. Clinical manifestations included spastic gait, sensory ataxia, conduction fascicular sensory impairment, as well as urination and defecation dysfunction. At the age of 58, this patient was unable to walk independently. The laboratory findings revealed a positive result for anti-nuclear antibody with a titer of 1∶1 000, anti-Sjögren's syndrome A (SSA)/Ro-52, anti-SSA/Ro-60, and anti-centromere antibodies. Labial salivary gland biopsy showed lymphocytes and plasma cells infiltration into the glandular tissues, interstitium, and lobules, with 2 foci/4 mm2 (lymphocytes >50). The cranial magnetic resonance imaging exhibited bilateral symmetric hyperintensity in the brainstem, characterized by the "snake-eye sign". Electromyography examination revealed axonal impairment of the right peroneal nerve. The patient was treated with high-dose corticosteroid therapy in combination with immunosuppressants. She experienced remarkable improvement. After three months, she was able to walk with aids and take care of herself in daily life. Case 2, female, presented spasmodic torticollis since the age of 81 with unknown reason. She had a history of mild mucosal dryness of mouth and eyes, and painful knee for one year. Laboratory findings revealed positive results for anti-SSA/Ro-52 and anti-SSA/Ro-60 antibodies, as well as hemoglobin of 86 g/L. Labial salivary gland biopsy demonstrated partial atrophy of the acini and the presence of 2 foci/4 mm2 of lymphocytes and plasma cells infiltration into the stroma (lymphocytes>50). Electromyography examination showed reduced conduction velocity in the right median nerve. She got a significant relief after the treatment of immunosuppressants, antispasmodics, and muscle relaxants.Conclusion·Patients presenting initial symptoms such as complex forms of spastic paraplegia and cervical dystonia expand the clinical spectrum of pSS. In clinical practice, it is important to distinguish neurological involvement secondary to pSS from other primary neurological disorders. For patients with neurological impairments but without apparent etiology, it is crucial to screen relevant series of autoimmune antibodies.

Keywords

• primary sjögren's syndrome (pss)
• neurological involvement
• spastic paraplegia
• spasmodic torticollis