Abstract
Acute encephalopathy in childhood is frequently associated with common infections,
especially in East Asia. Various types have been identified although many cases remain
unclassified. Congenital adrenal hyperplasia (CAH) is an autosomal recessive disease
presenting impairment of cortisol biosynthesis. We report three CAH children with
acute infection-related encephalopathy. They exhibited disturbed consciousness or
seizures, which did not improve after glucocorticoid administration, accompanied by
clinical and laboratory findings of adrenal insufficiency. Brain MRI disclosed various
patterns of white matter lesions, suggesting different types of acute encephalopathy
such as clinically mild encephalitis/encephalopathy with a reversible splenial lesion
or hemiconvulsion-hemiplegia syndrome. Acute encephalopathy should be considered and
brain MRI immediately performed when impairment of consciousness does not improve
after intravenous glucocorticoid administration in CAH patients. Further research
is required to elucidate the epidemiology and pathogenic mechanisms of acute encephalopathy
in CAH.
Keywords
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References
- Diffusion MRI abnormalities after prolonged febrile seizures with encephalopathy.Neurology. 2006; 66: 1304-1309
- Acute encephalopathy associated with influenza and other viral infections.Acta Neurol Scand Suppl. 2007; 186: 45-56
- Reye syndrome and reye-like syndrome.Pediatr Neurol. 2008; 39: 198-200
- Haemorrhagic shock and encephalopathy: a new syndrome with a high mortality in young children.Lancet. 1983; 2: 64-67
- Acute necrotizing encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions.J Neurol Neurosurg Psychiatry. 1995; 58: 555-561
- Clinically mild encephalitis/encephalopathy with a reversible splenial lesion.Neurology. 2004; 63: 1854-1858
- Differences in the time course of splenial and white matter lesions in clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS).J Neurol Sci. 2010; 292: 24-27
- A nation wide survey for the incidence of the central nervous system complications in patients treated for congenital adrenal hyperplasia (in Japanese).Horumon To Rinsho. 2002; 50: 1165-1169
- The spectrum of acute encephalopathy with reduced diffusion in the unilateral hemisphere.Eur J Paediatr Neurol. 2009; 13: 154-159
- Serial changes on diffusion-weighted magnetic resonance imaging in encephalitis or encephalopathy.Pediatr Neurol. 2006; 34: 308-311
- Brain white matter impairment in congenital adrenal hyperplasia.Arch Neurol. 2006; 63: 413-416
- Cognitive and neuroradiological findings in congenital adrenal hyperplasia.Psychoneuroendocrinology. 1994; 19: 55-64
- Magnetic resonance imaging in the congenital adrenal hyperplasia population: increased frequency of white-matter abnormalities and temporal lobe atrophy.J Child Neurol. 1997; 12: 181-186
- Hydrocortisone stimulates the development of oligodendrocytes in primary glial cultures and affects glucose metabolism and lipid synthesis in these cultures.Brain Res. 1987; 431: 79-86
- Prolonged corticosterone treatment of adult rats inhibits the proliferation of oligodendrocyte progenitors present throughout white and gray matter regions of the brain.Glia. 2000; 31: 219-231
- Repeated prenatal corticosteroid administration delays myelination of the corpus callosum in fetal sheep.Int J Dev Neurosci. 2001; 19: 415-425
- Bioavailability of oral hydrocortisone in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.J Endocrinol. 2001; 169: 65-70
- Progesterone and the oligodendroglial lineage: stage-dependent biosynthesis and metabolism.Glia. 2001; 36: 295-308
- Laminar cortical necrosis in adrenal crisis: sequential changes on MRI.Brain Dev. 2008; 30: 77-81
- Serum and CSF levels of cytokines in acute encephalopathy following prolonged febrile seizures.Brain Dev. 2008; 30: 47-52
- Steroid hormone regulation of antiviral immunity.Ann NY Acad Sci. 2000; 917: 935-943
- Encephalitis and encephalopathy associated with an influenza epidemic in Japan.Clin Infect Dis. 2002; 35: 512-517
Article info
Publication history
Published online: April 15, 2011
Accepted:
March 24,
2011
Received in revised form:
March 18,
2011
Received:
January 25,
2011
Identification
Copyright
© 2011 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
