Anti-Adenylate Kinase 5 Encephalitis With Histologic Evidence of CNS Vasculitis.

Encephalitis with antibodies against intracellular antigen adenylate kinase 5 (AK5) is a recently discovered entity; only 16 cases with characteristic clinical, radiologic, and CSF presentation have been described.1,-,3 There is no report of histologic association with vasculitis.\n\nWe present a case of AK5 limbic encephalitis with histologic evidence of CNS vasculitis.\n\nA 58-year-old woman was admitted for investigation of headaches, memory impairment, behavioral changes, and sleep disturbances, progressing over approximately 1 month and impairing her daily activities.\n\nNeuropsychologic examination showed a multimodal disorientation, severe executive and attentional deficits, disinhibition, and severe anterograde memory impairment affecting encoding, ideomotor apraxia, and left multimodal neglect. The rest of the neurologic and general examinations were normal.\n\nBasic laboratory tests were normal. A brain MRI at admission (1 month after symptoms onset) showed bilateral, right predominant, mesiotemporal T2—fluid-attenuated inversion recovery (FLAIR) …