title: Effectiveness of Corticosteroid Therapy for Acute Neurological Symptoms in Incontinentia Pigmenti.
abstract: BACKGROUND
  Incontinentia pigmenti is a rare neurocutaneous disorder that may result in neurological symptoms in addition to its characteristic skin rashes. The pathogenesis of central nervous system disorders in incontinentia pigmenti remains unclear, but it has been suggested that vascular abnormalities and inflammatory processes may play important roles. Notably, there is no established treatment for central nervous system disorders in incontinentia pigmenti. We report a neonate with acute neurological symptoms of incontinentia pigmenti who was effectively treated with corticosteroid therapy. We review the literature and discuss the pathophysiology, diagnosis, and treatment of acute central nervous system disorders in incontinentia pigmenti.
  
  
  METHODS
  A 15-day-old girl with incontinentia pigmenti experienced neurological symptoms such as decreased level of consciousness and a weak sucking reflex. Magnetic resonance imaging revealed multiple cerebral infarctions. We administered corticosteroid therapy, and the symptoms improved immediately and significantly.
  
  
  CONCLUSIONS
  We suggest that corticosteroid therapy may be an effective treatment during the acute phase of central nervous system dysfunction due to incontinentia pigmenti. It is important to determine the existence of acute phase lesions on magnetic resonance imaging when neurological symptoms occur or worsen.
authors.affiliation: Department of Neonatology, Kanagawa Children's Medical Center, Yokohama city, Kanagawa Prefecture, Japan. Electronic address: seasons12colors@yahoo.co.jp.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Tomotaki
authors.affiliation: Department of Neonatology, Kanagawa Children's Medical Center, Yokohama city, Kanagawa Prefecture, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Shibasaki
authors.affiliation: Department of Neonatology, Kanagawa Children's Medical Center, Yokohama city, Kanagawa Prefecture, Japan.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Yunoki
authors.affiliation: Department of Neonatology, Kanagawa Children's Medical Center, Yokohama city, Kanagawa Prefecture, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Kishigami
authors.affiliation: Department of Infectious Disease and Immunology, Kanagawa Children's Medical Center, Yokohama city, Kanagawa Prefecture, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Imagawa
authors.affiliation: Department of Radiology, Kanagawa Children's Medical Center, Yokohama city, Kanagawa Prefecture, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Aida
authors.affiliation: Department of Neonatology, Kanagawa Children's Medical Center, Yokohama city, Kanagawa Prefecture, Japan.
authors.forename: Katsuaki
authors.initials: K
authors.lastname: Toyoshima
authors.affiliation: Department of Neonatology, Kanagawa Children's Medical Center, Yokohama city, Kanagawa Prefecture, Japan.
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Itani
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications*; D007231:Infant, Newborn; D009422:Nervous System Diseases / Q000188:drug therapy* / Q000209:etiology*
chemical_list: D000305:Adrenal Cortex Hormones
keywords: central nervous system disorder; corticosteroid therapy; encephalopathy; incontinentia pigmenti; neonate; neurological symptoms