Congenital adrenal hyperplasia and brain magnetic resonance imaging abnormalities.

Content Provider	Europe PMC
Author	Samia, Younes-Mhenni Mahdi, Kamoun Baha, Zantour Saida, Jerbi-Ommezine Tahar, Sfar Mohamed Habib, Sfar Mohamed
Copyright Year	2010
Abstract	A 15-yr-old male patient with congenital adrenal hyperplasia (CAH) was referred to ourdepartment with a one year history of gradual worsening of tremors. He was diagnosed withsalt-wasting 21-hydroxylase deficiency CAH at 40 d old and was started on hydrocortisone,fludrocortisone and salt. He was found to have hypertension at 8 yr of age. Detailedinvestigations failed to detect any cause for secondary hypertension. Physical findings onthe current hospitalization objectified obesity, blood pressure of 150/80 mmHg, posturaland action tremor, left cerebellar syndrome, reflex tetra pyramidal syndrome and mentaldecline. Brain magnetic resonance imaging (MRI) showed bilateral periventricular whitematter hyperintensity that was more pronounced in the posterior regions and associatedwith cortico-subcortical atrophy and complete agenesis of the corpus callosum. Allinvestigations for leukoencephalopathy were negative. A diagnosis of brain MRIabnormalities related to CAH was made, and the patient received symptomatic treatment oftremors. Our case report provides evidence of an increased frequency of brain MRIabnormalities in CAH. The literature suggests hormonal imbalance and exposure to excessexogenous glucocorticoids as main probable mechanisms. Thus, in clinical practice, CAHshould be considered as one of the possible causes of brain white matter involvementassociated with or without cerebral atrophy.
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ISSN	09185739
Journal	Clinical Pediatric Endocrinology [Clin Pediatr Endocrinol]
Volume Number	19
DOI	10.1297/cpe.19.109
PubMed Central reference number	PMC3687627
Issue Number	4
PubMed reference number	23926386
e-ISSN	13477358
Language	English
Publisher	The Japanese Society for Pediatric Endocrinology
Publisher Date	2010-10-01
Access Restriction	Open
Rights License	This is an open-access article distributed under the terms of the CreativeCommons Attribution Non-Commercial No Derivatives (by-nc-nd) License. 2010©The Japanese Society for Pediatric Endocrinology
Subject Keyword	congenital adrenal hyperplasia 21-Hydroxylase deficiency MRI leukoencephalopathy corticosteroid
Content Type	Text
Resource Type	Article
Subject	Endocrinology, Diabetes and Metabolism Endocrinology Pediatrics, Perinatology and Child Health