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: Yosuke Eriguchi, Hidenori Yamasue, Nagafumi Doi, Takuji Nishida, Osamu Abe, Haruyasu Yamada, Shigeki Aoki, Motomu Suga, Hideyuki Inoue, Hiroi Nonaka, Takayuki Obata, Hiroo Ikehira, Keiko Kobayashi, Kiyoto Kasai
• Journal: Epilepsia (Epilepsia) Vol. 51 Issue 12 Pg. 2484-7 (Dec 2010) ISSN: 1528-1167 [Electronic] United States
• PMID: 21204812 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Wiley Periodicals, Inc. © 2010 International League Against Epilepsy.

Topics

• Adult
• Brain (metabolism)
• Brain Diseases, Metabolic (diagnosis, metabolism)
• Brain Mapping
• Citrullinemia (epidemiology, metabolism, surgery)
• Comorbidity
• Electroencephalography (statistics & numerical data)
• Epilepsy, Temporal Lobe (diagnosis, epidemiology, metabolism)
• Functional Laterality
• Hippocampus (metabolism)
• Humans
• Image Processing, Computer-Assisted
• Liver Transplantation
• Magnetic Resonance Imaging (statistics & numerical data)
• Magnetic Resonance Spectroscopy (statistics & numerical data)
• Male
• Sclerosis (diagnosis, metabolism)