Acute Disseminated Encephalomyelitis: A Gray Distinction

Abstract Background Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory acquired demyelinating syndrome predominantly affecting the white matter of the central nervous system. Methods We describe the case of a 3 year old boy, whose clinical presentation was suspicious for ADEM but initial imaging showed abnormalities confined to the deep gray matter (without evidence of white matter involvement) in the brain. His clinical course was fluctuating and repeat imaging obtained one week after presentation demonstrated interval development of characteristic white matter lesions. Results Treatment with adjunctive intravenous immunoglobulin (IVIg) and high-dose corticosteroids resulted in significant clinical improvement. Conclusions This case of ADEM is novel in demonstrating the initial findings of isolated deep gray matter involvement with delayed appearance of white matter abnormalities, suggesting that repeat imaging is indicated in cases that are clinically suspicious for ADEM but lack characteristic imaging findings