Magnetic Resonance Imaging in the Congenital Adrenal Hyperplasia Population: Increased Frequency of White-Matter Abnormalities and Temporal Lobe Atrophy
Restricted accessOtherFirst published online April, 1997
Magnetic Resonance Imaging in the Congenital Adrenal Hyperplasia Population: Increased Frequency of White-Matter Abnormalities and Temporal Lobe Atrophy
Congenital adrenal hyperplasia results from an adrenal enzyme deficiency, that causes an underproduction of glucocorticoids and sometimes mineralocorticoids and a resultant overproduction of androgens, until treatment with replacement glucocorticoids is instituted. The goal of this study was to determine the frequency and etiology of white-matter changes and temporal lobe atrophy demonstrable on magnetic resonance imaging (MRI) in a group of children and young adults with congenital adrenal hyperplasia. About one third of the patients evidenced white-matter abnormalities or temporal lobe atrophy. All patients, except one with a known stroke, had normal neurologic examinations. Exposure to excess exogenous glucocorticoids in the process of being treated for congenital adrenal hyperplasia is the most theoretically appealing explanation for these MRI findings. However, the relationship of MRI findings to treatment status (over- versus under-suppressed) does not run in clear parallel. (J Child Neurol 1997;12:181-186).
Get full access to this article
View all access options for this article.
References
1.
Tupler L., Coffey C., Logue P., et al: Neuropsychological importance of subcortical white matter hyperintensity . Arch Neurol1992;49:1248-1252.
2.
Lopez O., Becker J., Rezek D., et al: Neuropsychiatric correlates of cerebral white matter radiolucencies in probable Alzheimer's disease. Arch Neurol1992;49:828-834.
3.
Schmidt R., Fazekas F., Kleinert G., et al: MRI signal hyperintensities in the deep and subcortical white matter. Arch Neurol1992; 49:825-827.
4.
Almkvist O., Wahlund L., Andersson-Lumdman G., et al: White matter hyperintensity and neuropsychological functions in dementia and healthy aging. Arch Neurol1990;49:626-632.
5.
Boone K., Miller B., Lesser I., et al: Neuropsychological correlates of white matter lesions in healthy elderly. Arch Neurol1992;49: 549-554.
6.
Awad IA, Spetzler RF, Hodak JA, et al: Incidental subcortical lesions identified on MRI in the elderly: I. Correlation with age and cerebrovascular risk factors. Stroke1986; 17:1084-1089.
7.
Herhotz K., Heindel W., Rackl A.: Regional cerebral blood flow in patients with leukoariosis and atherosclerosis . Arch Neurol1990;47:392-396.
8.
Hunt AL, Orrison WW, Yeo RA, et al: Clinical significance of MRI white matter lesions in the elderly . Neurology1989;39:1470-1474.
9.
Kobari M., Meyer I., Ichijo M.: Leukoariosis, cerebral atrophy, and cerebral perfusion in normal aging . Arch Neurol1990;47:161-165.
10.
Kirkpatrick JB, Hayman LA: White matter lesions in MR imaging of clinically healthy brains of elderly subjects: Possible pathologic basis . Neuroradiology1987;162:509-511.
11.
Fazekas F., Niederkorn K., Schmidt R.: White matter signal abnormalities in normal individuals: Correlation with carotid ultrasound, cerebral blood flow measurements, and cerebrovascular factors. Stroke1988;19:1285-1288.
12.
Coffey C., Fegiel G.: Subcortical hyperintensity on MRI: A comparison of normal and depressed elderly . Am J Psychiatry1990;147:187-189.
13.
de Leon M., McRae T., Tsai J.: Hypercortisolemia is associated with hippocampal atrophy in Alzheimer's patients . Lancet1988;ii: 391-392.
14.
Brant-Zawadski M., Fein G., Van Dyke C., et al: MR imaging of the aging brain: Patchy white-matter lesions and dementia. Am J Neuroradiol1985;6:675-682.
15.
George AE, de Leon MJ, Kalnin A., et al: Leukoencephalopathy in normal and pathologic aging: 2. MRI of brain lucencies. Am J Neuroradiol1986; 7:567-570.
16.
Awad IA, Spetzler RF, Hodak JA, et al: Incidental subcortical lesions identified on MRI: Prevalence and clinical significance in various age groups. Neurosurgery1987;20:222-227.
17.
Soges L.: Migraine: Evaluation by MRI. Am J Neuroradiol1988; 9: 425-429.
18.
Hendric H.: Foci of increased density on T2 image in healthy elderly. Am J Neuroradiol1989; 10: 703-707.
19.
Gerard G., Weisberg LA: MRI periventricular lesions in adults. Neurology1986;36:998-1001.
20.
Fazekas F.: MR signal abnormalities in asymptomatic individuals: Their incidence and functional correlates. Eur Neurol1989 ;29: 164-168.
21.
Nowell M.: MR imaging of white matter in children. Am J Radiol1988;151:359-365.
22.
Sinforiani E. , Livieri C., Mauri M., et al: Cognitive and neuroradiological findings in CAH. Psychoneuroendocrinology1994;19:55-64.
23.
Speiser P., New M.: An update of congenital adrenal hyperplasia, in Lifshitz F (ed): Pediatric Endocrinology. New York, Dekker, 1990 , pp 307-332.
24.
Williams RH (ed): Textbook of Endocrinology . Philadelphia, WB Saunders, 1981.
25.
Dallman M.: Corticosterone replacement in adrenalectomized rats: Insights into the regulation of ACTH secretion, in Rose F (ed): The Control of the Hypothalamic-Pituitary-Adrenocortical Axis . Madison, CT: International Universities Press, 1990, pp 399-426.
26.
Linder B., Esteban NV, Yergey AL, et al: Cortisol production rate in childhood and adolescence. J Pediatr1990; 117:892-896.
27.
Linder B., Feuillan F., Chrousos GP: Alternate day prednisone therapy in congenital adrenal hyperplasia: Adrenal androgen suppression and normal growth. J Clin Endocrinol Metab1989;69: 191-195.
28.
New MI, Gertner JM, Speiser PW, del Balzo P.: Growth and final height in classical and nonclassical 21-hydroxylase deficiency . J Endocrinol Invest1989;12(Suppl 3):91-95.
29.
New MI: Replacement doses of glucocorticoids. JPediatr1991;119: 161.
30.
New M.: Cortisol secretion in children. J Clin Endocrinol1970;30: 361.
31.
Akana S.: Corticosterone: Narrow range required for normal body and thymus weight and ACTH. Am J Physiol1985;249:527-532.
32.
Mulaikal RM , Migeon CJ, Rock JA: Fertility rates in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. N Engl J Med1987;316:178-182.
33.
Hamwi GJ, Serbin RA, Kruger FA: Does adrenal cortical hyperplasia result in adrenocortical carcinoma?N Engl J Med1957;257: 1153-1157.
34.
Heier LA, Bauer CJ, Schwartz L., et al: Large Virchow-Robin spaces: MR clinical correlation. Am J Neuroradiol1989;10:929-936.
35.
George AE, de Leon M., Stylopoulos L., et al:CT diagnostic features of Alzheimer's disease: Importance of the choroidal/hippocampal fissure complex. Am J NeuroradioL1990;11:101-107.
36.
Speiser P., Nass R., Heier L., et al: Failure of steroid replacement to control pituitary function in congenital adrenal hyperplasia (CAH): Hormonal and MRI data. Horm Res1995;44:241-246.
37.
Chimowitz M. , Estes M., Furlan A., Awad I.: Further observations on the pathology of subcortical lesions identified on MRI. Arch Neurol1992;49:748-752.
38.
Munoz D., Hastak S., Harper B., et al: Pathologic correlates of increased signals of the centrum ovale on MRI. Arch Neurol1993;50:492-497.
39.
Van Swieten J., Van den Hout J., Van Ketel B., et al: Periventricular lesions in the white matter on MRI in the elderly. Brain1991;114: 761-774.
40.
Braffman BH , Zimmerman RA, Trojanowski JQ, et al: Brain MR: Pathologic correlation with gross and histopathology: 1. Lacunar infarction and Virchow-Robin spaces. AJR Am J Roentgenol1988;151:551-558.
41.
Fazekas F., Kleinart R., Offenbacher H., et al: Pathological correlates of incidental MRI white matter signal intensities. Neurology1993;43:1683-1689.
42.
Scheltens PHBarkhof F., Leys D., et al: Histopathological correlates of white matter changes on MRI in Alzheimer's disease and normal aging. Neurology1995;45:883-889.
43.
Damasio H.: A CT guide to the identification of the cerebrovascular territories . Arch Neurol1983;40:138-142.
44.
Barnett H, Mohr J, Stein B, Yatsu F (eds): Stroke. New York, Churchill Livingstone, 1986.
45.
Ayus J., Krothapalli R., Arieff A.: Treatment of symptomatic hyponatremia and its relation to brain damage . N Engl J Med1987;317: 1190-1195.
46.
David S., Kalyankar G.: Effect of adrenalectomy on GABA concentrations in the central nervous system . J Neurochem1986; 46:161-165.
47.
Faunt J., Crocker A.: Effects of adrenalectomy on responses mediated by dopamine D-1 and D-2 receptors . Eur J Pharm1989;162: 237-244.
48.
Howard E., Benjamin J.: DNA, ganglioside and sulfatide in brains of rats given corticosterone in infancy, with an estimate of cell loss during development. Brain Res1975;92:73-87.
49.
McEwen B., Angulo J., Cameron H., et al: Paradoxical effects of adrenal steroids on the brain: Protection versus degeneration. Biol Psychiatry1992;31:177-199.
50.
Scheff S., Hoff S., Anderson K.: Altered regulation of lesion induced synaptogenesis by adrenalectomy and corticosterone in young adult rats. Exp Neurol1986;93:456-470.
51.
Swann A.: Dexamethasone and adrenalectomy alter Brain (Na+, K+)-atpase response in noradrenergic stimulation or depletion. Eur J Pharmacol1988;158:43-52.
52.
Sapolsky R. , Krey C., McEwen B.: Glucocorticoids and neuronal function . J Neurosci1985;5:1222-1228.
53.
Cotterrell M., Balazs R., Johnson AL: Effects of corticosteroids on the biochemical maturation of rat brain: Postnatal cell formation. J Neurochem1972;19:2151-2167.
54.
Sapolsky R. , Pulsinelli W.: Glucocorticoids potentiate ischemic injury to neurons: Therapeutic implications. Science1985;229:1397-1400.
55.
Raley-Susman K., Sapolsky R.: Glucocorticoid effects on neuronal development and vitality, in Lou H, Greisen G, Falck Larsen J (eds): Brain Lesions in the Newborn. Munksgaard, Copenhagen, 1994, pp 257-268.
56.
Meyer J., Fairman M.: Early adrenalectomy increases myelin in rat brain. Dev Brain Res1985;17:1-9.
57.
Bentson J.: Steroids and apparent cerebral atrophy on computer tomography scans . J Comput Assist Tomogr1978;2:16-23.
58.
Okuna T.: Cerebral atrophy following ACTH. J Comput Assist Tomogr1980;4:20-23.
59.
McEwen B. : Glucocorticoids and the hippocampus, in Garten D (ed): Current Topics in Neuroendocrinology. New York, Springer-Verlag, 1982, pp 1-22.
60.
Woolley C., Gould E., McEwen B.: Exposure to excess glucocorticoids alters dendritic morphology of adult hippocampal pyramidal neurons. Brain Res1990;531:225-231.
61.
Starkman M. , Gebarski S., Berent S., Schteingart D.: Hippocampal formation volume, memory dysfunction and cortisol level in patients with Cushing syndrome. Biol Psychiatry1992;32:756-765.
62.
Oberfield S. , Cowan L., Levine L., et al: Altered cortisol response and hippocampal atrophy in pediatric HIV disease. J Acquir Immune Defic Syndr1994;7:57-62.
63.
Nass R., Baker S.: A review of the neuropsychological profile in congenital adrenal hyperplasia , in Tallal P, McEwen B (eds): Psychonezcroendocrinology1991 ;16:189-201.
