We report a patient who, at age 38, presented with temporal lobe seizu
res and an atypical memory disorder 3 years before the discovery of a
testicular tumor. Detailed neuropsychological testing revealed a relat
ively isolated amnestic syndrome. The amnesia was atypical as the pati
ent could retain information for hour-long periods, only to lose it la
ter. Serologic studies revealed the presence of a circulating autoanti
body that demonstrated an unusual affinity for the nucleolus of cerebr
al cortical neurons. Western blot analysis of cortical neurons reveale
d that this antibody reacted with proteins different from other previo
usly identified paraneoplastic neurologic antigens (Hu, Yo, Ri). We be
lieve this to be a case of paraneoplastic limbic encephalitis secondar
y to the testicular tumor. This patient presents unique characteristic
s with respect to the mode of presentation, features of the amnesia, a
nd the presence of a circulating antibody with an unusual reactivity p
attern.