Background Sweet's syndrome with involvement of the central nervous system (CNS) is rarely reported.
Methods We describe a Japanese woman with Sweet's syndrome associated with acute-onset encephalitis and review literatures.
Results Examination of the cerebrospinal fluid (CSF) revealed pleocytosis with lymphocytes predominant. Magnetic resonance imaging (MRI) revealed increased signal intensity on T2-weighted scans in the left temporal lobe.
To our knowledge, 22 cases of Sweet's syndrome associated with CNS involvement have been reported. The mean age is 47.6 years (n = 22). Sex distribution (male : female) is 12 : 10 (n = 22). The most common neurologic symptoms are convulsions, headaches, and disturbance of consciousness. CSF cell count is increased with lymphocytes predominant in 8 cases (n = 12). Certain (HLA) types (B54 and Cw1) may be characteristic findings in Sweet's syndrome accompanied with the CNS involvement in Japanese patients, as these were found in four previous cases similar to the present case.
Conclusions Although Sweet's syndrome with neurologic manifestations is rarely reported, it may be needed to investigate neurologic manifestations.