Limbic encephalitis.

ABSTRACT

Limbic encephalitis (LE) is a clinical syndrome defined by subacutely evolving limbic signs and symptoms with structural and functional evidence of mediotemporal damage in the absence of a better explanation than an autoimmune (or paraneoplastic) cause. There are features common to all forms of LE. In recent years, antibody(ab)-defined subtypes have been established. They are distinct regarding underlying pathophysiologic processes, clinical and magnetic resonance imaging courses, cerebrospinal fluid signatures, treatment responsivity, and likelihood of a chronic course. With immunotherapy, LE with abs against surface antigens has a better outcome than LE with abs to intracellular antigens. Diagnostic and treatment challenges are, on the one hand, to avoid overlooking and undertreatment and, on the other hand, to avoid overdiagnoses and overtreatment. LE can be conceptualized as a model disease for the consequences of new onset mediotemporal damage by different mechanisms in adult life. It may be studied as an example of mediotemporal epileptogenesis.