[A case of progressive systemic sclerosis with Sjögren's syndrome presenting with coma, convulsion and bilateral...

A 35-year-old female with progressive systemic sclerosis (PSS) and Sjögren's syndrome developed consciousness disturbance and generalized seizure after the episode of fever and erythema lasting for 3 weeks. Neurological examination disclosed deep coma and spastic tetraplegia with pathological reflexes. Laboratory data showed mild anemia, severe hypoproteinemia, hypoalbuminemia and increase of protein content in the cerebrospinal fluid. Cranial CT scans obtained after convulsion revealed diffuse brain swelling and bilateral symmetrical hypodensity involving the thalami and posterior limbs of the internal capsule. High-dose corticosteroid therapy and osmotherapy with correction of hypovolemia were started, because severe dehydration, hypoalbuminemia and cerebral vasculitis were suspected to change the vascular permeabilities. Neurological symptoms and CT findings were rapidly improved. Cerebral angiography 4 weeks after convulsion showed definite angitis. The patient was discharged 9 weeks after convulsion. In our case, a possible cause of the characteristic CT findings may be the disturbance of cerebral venous return due to hypovolemia and vasculitis. High-dose corticosteroid therapy may be recommended in patients with collagen disease, who show the bilateral symmetrical thalamic hypodensity on CT scans.