title: A case of adult-onset type II citrullinemia with comorbid epilepsy even after liver transplantation.
abstract: The current study using single case voxel-based morphometry (VBM) of magnetic resonance imaging (MRI) and ¹H-MR-spectroscopy (¹H-MRS) explores the neural background of unexplained seizure attacks and electroencephalography (EEG) abnormalities persisting even after liver transplantation in a patient with adult-onset type II citrullinemia (CTLN2). Although the MRI had shown no gross abnormality, the VBM revealed significantly smaller-than-normal regional volume in the left hippocampus of the patient as compared with 111 age-matched controls. ¹H-MRS further indicated reduction of all metabolite concentrations in the left hippocampus compared with those in the right homolog region, with the single exception of elevated glutamate concentration. These results are similar to those of patients with mesial temporal lobe epilepsy (TLE), although CTLN2-complicated mesial TLE has rarely been reported. In contrast to TLE, periictal asterixis and interictal slow activities on EEG support another possibility that the patient might have slight metabolic encephalopathy even after the liver transplantation.
authors.affiliation: Department of Neuropsychiatry, Graduate School of Medicine, University of Tokyo, Bunkyo-ku, Tokyo, Japan.
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Eriguchi
authors.forename: Hidenori
authors.initials: H
authors.lastname: Yamasue
authors.forename: Nagafumi
authors.initials: N
authors.lastname: Doi
authors.forename: Takuji
authors.initials: T
authors.lastname: Nishida
authors.forename: Osamu
authors.initials: O
authors.lastname: Abe
authors.forename: Haruyasu
authors.initials: H
authors.lastname: Yamada
authors.forename: Shigeki
authors.initials: S
authors.lastname: Aoki
authors.forename: Motomu
authors.initials: M
authors.lastname: Suga
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Inoue
authors.forename: Hiroi
authors.initials: H
authors.lastname: Nonaka
authors.forename: Takayuki
authors.initials: T
authors.lastname: Obata
authors.forename: Hiroo
authors.initials: H
authors.lastname: Ikehira
authors.forename: Keiko
authors.initials: K
authors.lastname: Kobayashi
authors.forename: Kiyoto
authors.initials: K
authors.lastname: Kasai
mesh_terms: D000328:Adult; D001921:Brain / Q000378:metabolism; D001928:Brain Diseases, Metabolic / Q000175:diagnosis / Q000378:metabolism; D001931:Brain Mapping; D020159:Citrullinemia / Q000453:epidemiology / Q000378:metabolism / Q000601:surgery; D015897:Comorbidity; D004569:Electroencephalography / Q000706:statistics & numerical data; D004833:Epilepsy, Temporal Lobe / Q000175:diagnosis / Q000453:epidemiology* / Q000378:metabolism; D007839:Functional Laterality; D006624:Hippocampus / Q000378:metabolism; D006801:Humans; D007091:Image Processing, Computer-Assisted; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging / Q000706:statistics & numerical data; D009682:Magnetic Resonance Spectroscopy / Q000706:statistics & numerical data; D008297:Male; D012598:Sclerosis / Q000175:diagnosis / Q000378:metabolism