Kaleidoscopic nervous system involvement in the setting of a chronic multisystem dysimmune disorder: report of a remarkable case

Abstract

Background. Neurological manifestations are not an unusual expression of multisystem dysimmune disorders, some etiopathogenic correlations being more common than others. We report here four distinct patterns of recurrent central nervous system (CNS) involvement and neuromuscular junction dysfunction occurring in an autoimmune chronic disease. Our patient presented diagnosis clues suggestive for systemic lupus erythematosus, antiphospholipid syndrome and Sjögren’s syndrome along with clinical findings highly characteristic for two primary vasculitis (i.e. Churg-Strauss syndrome and Behçet’s disease) and an increased susceptibility to infections. To our knowledge no similar case has been described previously. Case presentation. The patient is a Caucasian female of current age 26. Her neurological condition evolved over the last 6 years in a relapsing-remitting manner with recurrent cerebral venous system thrombosis/thrombophlebitis, recurrent myelopathy consistent with acute transverse myelitis, recurrent encephalopathy manifested as psychosis, recurrent pancerebellar syndrome and recent onset myasthenic syndrome. In spite of reaching life-threatening illness severity several times, following each one of the disease flare-ups the patient eventually showed a complete clinical resolution with therapy, as a peculiar and unexpected feature of her repeated CNS autoimmune attacks. Conclusion. When facing a complex and atypical immune disorder involving CNS one should remain cautious in labeling it. In such beyond-guidelines cases several treatment approaches should be tried and reevaluation of different organ involvement is a must.