Dados do Trabalho

Título

Reversible cerebellar magnetic resonance imaging abnormality and ataxia as a first manifestation in a patient with systemic lupus erythematosus

RESUMO

Case report
A 36-year-old woman, admitted to the emergency department complaining cognitive changes, in gait imbalance, progressive incoordination, asthenia and weight loss started 3 months ago. At the neurological examination, the patient presented disorientation with inappropriate behavior, gait ataxia and bilateral dysmetria and dysdiadokokinesia.
Initial investigations showed the presence of normochromic normocytic anemia, with positive direct coombs. The FLAIR in brain MRI revealed high signal intensity in the cerebellum symmetrically and bilaterally and the DWI showed restricted diffusion. The study of CSF that had inflammatory characteristics with 35 cells (85%monocytes), proteins 58 mg/dl and glucose 50 mg/dl, the CSF culture did not reveal any signs of infection; the culture was negative at 72 h, cytomegalovirus PCR, human herpesvirus 1 e 2, varicella-zoster virus and Epstein–Barr virus PCR were all negative. Serological profile for hepatitis B, hepatitis C and human immunodeficiency virus were negative. Neoplastic screening with tomography was negative.
Autoimmune profile, however, was positive for antinuclear antibody (1: 640 titers, speckled pattern), anti-DNA antibody (1:40 titer) and anti-Sm antibody reagent with consumed C3, C4 and CH50. Systemic lupus erythematosus, unequivocally fulfilling the Systemic Lupus International Collaborating Clinics classification criteria, was made and corticosteroid treatment was started with prednisone 60 mg per day, after a week of follow-up the patient has a completely improved of ataxia and the brain MRI abnormality.

Discussion
​Neuropsychiatric manifestation is commonly found in patients with SLE, it can manifest as changes in both the central and peripheral nervous systems. The most common manifestations are cognitive dysfunction, headache, seizures, and psychiatric conditions; aseptic meningitis, stroke, encephalopathy, movement disorders, and myelopathy are also seen. Cerebellar involvement in SLE is estimated at less than 2% of cases and more rarely as the first clinical manifestation. We present a patient that had a cerebellar syndrome and MRI abnormalities as the first presentation of SLE. The diagnosis of inflammatory cerebellitis associated with SLE was made supported by mild inflammatory changes in the CSF, clinical changes and abnormalities in neuroimaging rapidly reversible. Cerebellar degeneration due to SLE is not common.

Palavras Chave

ataxia, cerebellitis, systemic lupus erythematosus