Neurosjögren and antiphospholipid syndrome: a case report

Abstract

Background: Sjögren's syndrome is an autoimmune chronic systemic disease, characterized by lymphocytic infiltration of exocrine glands, and exceptionally affects nervous system.Objective: To describe a case of Sjögren, with CNS initial manifestations.Patient and Methods: A 38 years old, Wichi ethnicity, female, admitted with generalized tonic-clonic seizures, continuous partial epilepsy in upper limbs and right eye blindness of sudden onset. MRI shows hiperintense brain lesions, in T2 and FLAIR weighted secuences, with heterogeneous enhancement after contrast. Laboratory: Presence of Anti-SSA/ Anti-SSB, presence of lupus anticoagulant and antiphospholipid antibody, Schirmer test was positive, salivary gland and brain biopsy were performed, fulfilling diagnostic criteria for Sjögren's disease and antiphospholipid syndrome. Central retinal vein thrombosis was diagnosed. Patient signed the informed consent.Results: We highlight features of onset, with inconspicuous clinical sicca syndrome, despite a considerable neurological envolvement. Good clinical and imaginological outcomes were achieved after glucocorticoids, cyclophosphamide, antiepileptic and anticoagulants terapy.Conclusion: This presentation underlines the importance of suspecting this pathology in young patients with vascular and CNS compromised. We emphasized the requirement biopsy before any therapeutic approach to obtain a certain diagnosis.Brain Biopsy: Mononuclear inflammatory elements around small vessels.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Background: Sjögren's syndrome is an autoimmune chronic systemic disease, characterized by lymphocytic infiltration of exocrine glands, and exceptionally affects nervous system. Objective: To describe a case of Sjögren, with CNS initial manifestations. Patient and Methods: A 38 years old, Wichi ethnicity, female, admitted with generalized tonic-clonic seizures, continuous partial epilepsy in upper limbs and right eye blindness of sudden onset. MRI shows hiperintense brain lesions, in T2 and FLAIR weighted secuences, with heterogeneous enhancement after contrast. Laboratory: Presence of Anti-SSA/ Anti-SSB, presence of lupus anticoagulant and antiphospholipid antibody, Schirmer test was positive, salivary gland and brain biopsy were performed, fulfilling diagnostic criteria for Sjögren's disease and antiphospholipid syndrome. Central retinal vein thrombosis was diagnosed. Patient signed the informed consent. Results: We highlight features of onset, with inconspicuous clinical sicca syndrome, despite a considerable neurological envolvement. Good clinical and imaginological outcomes were achieved after glucocorticoids, cyclophosphamide, antiepileptic and anticoagulants terapy. Conclusion: This presentation underlines the importance of suspecting this pathology in young patients with vascular and CNS compromised. We emphasized the requirement biopsy before any therapeutic approach to obtain a certain diagnosis. Brain Biopsy: Mononuclear inflammatory elements around small vessels.