Tohoku J. Exp. Med., 229: 171, 2013.

Erratum

Magnetoencephalography Reveals a Unique Neurophysiological Profile of Focal-Onset Epileptic Spasms

YOSUKE KAKISAKA,^1,2 AJAY GUPTA,¹ REI ENATSU,¹ ZHONG I. WANG,¹ ANDREAS V. ALEXOPOULOS,¹ JOHN C. MOSHER,¹ ANNE-SOPHIE DUBARRY,¹ NAOMI HINO-FUKUYO² and RICHARD C. BURGESS¹

¹Epilepsy Center, Neurological Institute Cleveland Clinic Foundation, Cleveland, OH, USA
²Department of Pediatrics, Tohoku University School of Medicine, Sendai, Miyagi, Japan

Tohoku J. Exp. Med., 2013, 229, 147-151
The patient presented in this article was diagnosed with Aicardi syndrome, not Aicardi-Goutieres syndrome (AGS). “Aicardi-Goutieres syndrome” in the text should be replaced with “Aicardi syndrome” throughout. Thus, the third paragraph of the Introduction, on AGS gene information, is not suitable for this article and should be removed.

Authors' remarks on the two distinct syndromes
Aicardi syndrome is characterized by a triad of callosal agenesis, infantile spasms and chorioretinal “lacunae”. The syndrome has been reported in girls with rare exception. The brain malformation is complex with cortical migraine abnormalities, often cystic formation and sometimes choroid plexus papillomas. The outcome of AS is severe, with a high early mortality, considerable morbidity and a generally poor development outcome.
Aicardi-Goutières syndrome is a familial progressive early onset encephalopathy with basal ganglia calcifications, chronic lymphocytosis and high level of interferon-alpha in cerebrospinal fluid. The syndrome is caused by one of the genes encoding the DNA exonuclease TREX1 (AGS1), the three non-allelic components of the RNase H2 endonuclease complex (RNASEH2B, AGS2; RNASEH2C, AGS3; and RNASEH2A, AGS4), and the deoxynucleoside triphosphate triphosphohydrolase SAMHD1 (AGS5). Signs of severe and progressed brain atrophy with enlarged ventricles are a constant finding.

© 2013 Tohoku University Medical Press

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Tohoku J. Exp. Med., 2013, 229, 171