Effectiveness of Corticosteroid Therapy for Acute Neurological Symptoms in Incontinentia Pigmenti

Pediatric Neurology
Seiichi TomotakiYasufumi Itani

Abstract

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. 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. 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 th...Continue Reading

References

Oct 29, 2003·Pediatric Neurology·Sabine J HennelTerrie E Inder
Oct 24, 2007·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Femke Maingay-de GroofPaul P Govaert
Dec 22, 2007·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·J H LeeJ S Chun
Sep 24, 2008·Brain & Development·Naoya MatsumotoKenji Fujieda
Jan 20, 2009·Journal of Child Neurology·Michael S CartwrightE Steve Roach
Feb 15, 2013·Orphanet Journal of Rare Diseases·Snežana MinićMiljana Obradović
Mar 15, 2014·Anais Brasileiros De Dermatologia·Cláudia Schermann PoziomczykAna Elisa Kiszewski Bau

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Citations

Jul 18, 2020·Journal of the European Academy of Dermatology and Venereology : JEADV·C BodemerJ Steffann
Jul 21, 2017·Chemical Reviews·Mingying YangChuanbin Mao

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