Klüver-Bucy Syndrome with Isolated Bilateral Hippocampal Atrophy Following Status Epilepticus
A 31-year-old man who had no significant medical history presented with status epilepticus. He had headache, fever of 39° and generalized tonic clonic seizure without recovery of consciousness. His vital sign was stable except fever. On neurological examination, there was no abnormality except stuporous mental status between frequent generalized tonic clonic seizures. Initial blood tests showed no leukocytosis and normal level of inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Cerebrospinal fluid (CSF) was clean and contained 108 leukocytes/mm3 (83% polymor-phonuclear leukocytes and 17% lymphocytes) with normal glucose (78 mg/dL) and protein (42 mg/dL). Serologic testing of blood and CSF was normal. Initial brain MRI showed T2 hyperintensity and swelling of isolated bilateral hippocampus, especially CA1 region without any abnormal lesion in other areas ().\nOn the day of admission, we assumed that the patient had herpes encephalitis, and started maximum dosage of acyclovir (15 mg/kg three times a day) which is the hospital protocol for treatment of herpes encephalitis. Electroencephalography (EEG) showed frequent spike and waves on bilateral fronto-temporal areas dominantly on temporal areas, so we started antiepileptic drugs (initial phenytoin loading with subsequent add-on of levetiracetam, oxcarbazepine and topiramate) and midazolam-coma therapy. Further serologic tests including polymerase chain reaction (PCR) for herpes species were negative and no bacteria and viruses were grown. He recovered from status epilepticus three weeks later, but afterwards he developed KBS which is characterized by hyperphagia (he ate a large amount of food six to seven times a day), hypersexuality (he wanted to have a sex with the nurses, his ex-girlfriend and his mother), hypermetamorphosis, global amnesia (memory registration was normal, but recall was impaired) and dysosmia. On the 46th day of hospitalization, the score of his Korean-version of mini-mental status examination was 20/30 (orientation to time- 3/5, orientation to place- 2/5, immediate recall- 3/3, delayed recall- 0/3, attention and calculation- 3/5). Since we considered that his symptoms occurred due to secondary temporal lobe epilepsy, we performed an EEG, but his EEG showed no definite epileptiform discharges. Forty-five days later, follow-up brain MRI showed isolated bilateral hippocampal atrophy (). Finally, we concluded that he had a KBS, and the patient was discharged on the 58th day of hospitalization and his cognitive dysfunction is persistent with minimal improvement for six months follow-up.