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A case of adult‐onset type II citrullinemia with comorbid epilepsy even after liver transplantation
Author(s) -
Eriguchi Yosuke,
Yamasue Hidenori,
Doi Nagafumi,
Nishida Takuji,
Abe Osamu,
Yamada Haruyasu,
Aoki Shigeki,
Suga Motomu,
Inoue Hideyuki,
aka Hiroi,
Obata Takayuki,
Ikehira Hiroo,
Kobayashi Keiko,
Kasai Kiyoto
Publication year - 2010
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/j.1528-1167.2010.02760.x
Subject(s) - citrullinemia , ictal , liver transplantation , epilepsy , abnormality , hippocampus , transplantation , magnetic resonance imaging , encephalopathy , temporal lobe , electroencephalography , medicine , hepatic encephalopathy , psychology , endocrinology , neuroscience , radiology , chemistry , cirrhosis , psychiatry , urea cycle , biochemistry , amino acid , arginine
Summary The current study using single case voxel‐based morphometry (VBM) of magnetic resonance imaging (MRI) and 1 H‐MR‐spectroscopy ( 1 H‐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. 1 H‐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.