Vigabatrin Therapy for Infantile Spasms in a Case of Cardiofaciocutaneous Syndrome with Cardiac Hypertrophy Developing during Adrenocorticotropic Hormone Treatment.
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.
Department of Pediatrics, Jichi Medical University Saitama Medical Center.
Takayuki
T
Hatori
Yohei
Y
Sugiyama
Shinichiro
S
Yamashita
Yuka
Y
Hirakubo
Kazuhito
K
Nonaka
Ko
K
Ichihashi
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use*; D006332:Cardiomegaly / Q000150:complications*; D002675:Child, Preschool; D004476:Ectodermal Dysplasia / Q000150:complications* / Q000000981:diagnostic imaging; D004562:Electrocardiography; D019066:Facies; D005183:Failure to Thrive / Q000150:complications* / Q000000981:diagnostic imaging; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D013036:Spasms, Infantile / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy*; D020888:Vigabatrin / Q000627:therapeutic use*
D000324:Adrenocorticotropic Hormone; D020888:Vigabatrin