-Case Reports-

Vigabatrin Therapy for Infantile Spasms in a Case of Cardiofaciocutaneous Syndrome with Cardiac Hypertrophy Developing during Adrenocorticotropic Hormone Treatment

Takayuki Hatori, Yohei Sugiyama, Shinichiro Yamashita, Yuka Hirakubo, Kazuhito Nonaka and Ko Ichihashi

Department of Pediatrics, Jichi Medical University Saitama Medical Center, Saitama, Japan

In a patient with cardiofaciocutaneous syndrome complicated by intractable infantile spasms (West syndrome), cardiac hypertrophy developed during adrenocorticotropic hormone treatment. Various types of antiepileptic drugs, intravenous immunoglobulin, thyrotropin releasing hormone, and a ketogenic diet were ineffective in this case. However, vigabatrin both decreased clinical seizures and improved electroencephalogram findings. Although vigabatrin has not been approved for use in Japan, the results in the present case suggest that this drug should be considered as an alternative therapy for cases of infantile spasms associated with syndromes involving cardiomyopathy or its potential risk factors, such as cardiofaciocutaneous syndrome.

J Nippon Med Sch 2016; 83: 167-171

Keywords
cardiofaciocutaneous syndrome, infantile spasms, hypertrophic cardiomyopathy, vigabatrin

Correspondence to
Takayuki Hatori, MD, PhD, Department of Pediatrics, Jichi Medical University Saitama Medical Center, 1-847 Amanuma-cho, Saitama 330-8503, Japan
t-hatori@nms.ac.jp

Received, January 7, 2016
Accepted, February 4, 2016