Case Reports in Clinical Practice 2017. 2(2):55-59.

Neurological Symptom in a Case of Adrenal Insufficiency
mahboobe hemmatabadi, Tina Deihim, Neda Meftah, Sahar Karimpour


Around 50% of patients with Addison disease (AD) have other autoimmune disorders. Neuropsychiatric symptoms may be presenting features of an Addisonian crisis or may be presented in patients with adrenoleukodystrophy (ALD) or Hashimoto encephalopathy; that these disorders are associated with primary adrenal insufficiency (AI). Nearly 5% of individuals with autoimmune AD develop pernicious anemia. X-linked ALD (X-ALD) is an inherited neurodegenerative disorder; a frequent but under-recognized cause of primary adrenocortical insufficiency. The classic picture of Vitamin B12 deficiency is subacute combined degeneration of the dorsal (posterior) and lateral spinal columns. The neuropathy is symmetrical, affects the legs more than the arms. It begins with paresthesia and ataxia and can progress to severe weakness, spasticity, paraplegia, even fecal, and urinary incontinence. We report a case of autoimmune AI who had presented with some neurologic symptoms. The challenging point was differentiating between X-ALD and other neurological syndromes that have an association with AI.
  We report a case of autoimmune adrenal insufficiency who had presented with some neurologic symptoms. The challenging point was differentiating between X-ALD and other neurological syndromes associating with adrenal insufficiency.


Addison; Adrenoleukodystrophy; Vitamin B12 deficiency

Full Text:



Charmandari E, Nicolaides NC, Chrousos GP. Adrenal insufficiency. Lancet 2014; 383(9935): 2152-67.

Arlt W, Allolio B. Adrenal insufficiency. Lancet 2003; 361(9372): 1881-93.

Betterle C, Dal Pra C, Mantero F, Zanchetta R. Autoimmune adrenal insufficiency and autoimmune polyendocrine syndromes: autoantibodies, autoantigens, and their applicability in diagnosis and disease prediction. Endocr Rev 2002; 23(3): 327-64.

Vrkljan AM, Pasalic A, Strinovic M, Peric B, Kruljac I, Mirosevc G. Coexistence of Addison's disease and pernicious anemia: Is The New Classification Of Autoimmune Polyglandular Syndrome Appropriate? Acta Clin Croat 2015; 54(2): 232-5.

Husebye ES, Perheentupa J, Rautemaa R, Kampe O. Clinical manifestations and management of patients with autoimmune

polyendocrine syndrome type I. J Intern Med 2009; 265(5): 514-29.

Steinberg SJ, Moser AB, Raymond GV. XLinked Adrenoleukodystrophy. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, editors. GeneReviews(®). Seattle, WA: University of Washington; 2015.

Ronghe MD, Barton J, Jardine PE, Crowne EC, Webster MH, Armitage M, et al. The importance of testing for adrenoleucodystrophy in males with idiopathic Addison's disease. Arch Dis Child 2002; 86(3): 185-9.

Laureti S, Casucci G, Santeusanio F, Angeletti G, Aubourg P, Brunetti P. X-linked adrenoleukodystrophy is a frequent cause of idiopathic Addison's disease in young adult male patients. J Clin Endocrinol Metab 1996; 81(2): 470-4.

Neufeld M, Maclaren NK, Blizzard RM. Two types of autoimmune Addison's disease associated with different polyglandular

autoimmune (PGA) syndromes. Medicine (Baltimore) 1981; 60(5): 355 62.

Riggs JE. Neurologic manifestations of electrolyte disturbances. Neurol Clin 2002; 20(1): 227-39, vii.

Moser HW, Raymond GV, Dubey P. Adrenoleukodystrophy: New approaches to a neurodegenerative disease. JAMA 2005; 294(24): 3131-4.

Myers KA, Rho JM, Wei XC. Teaching neuroimages: MRI findings in X-linked adrenoleukodystrophy. Neurology 2015; 85(17): e132-e133.

Moser HW, Bergin A, Naidu S, Ladenson PW. Adrenoleukodystrophy. Endocrinol Metab Clin North Am 1991; 20(2): 297-318.

Wakerley BR, Uncini A, Yuki N. GuillainBarre and Miller Fisher syndromes--new diagnostic classification. Nat Rev Neurol 2014; 10(9): 537-44.

Bandmann O, Weiss KH, Kaler SG. Wilson's disease and other neurological copper disorders. Lancet Neurol 2015; 14(1): 103-13.

Patel R, Sabat S, Kanekar S. Imaging Manifestations of neurologic complications in anemia. Hematol Oncol Clin North Am 2016; 30(4): 733-56.

Ravina B, Loevner LA, Bank W. MR findings in subacute combined degeneration of the spinal cord: a case of reversible cervical

myelopathy. AJR Am J Roentgenol 2000; 174(3): 863-5.

Bizzaro N, Antico A. Diagnosis and classification of pernicious anemia. Autoimmun Rev 2014; 13(4-5): 565-8.

HY, Lee JW, Park KS, Wi JY, Kang HS. Spine MR imaging features of subacute combined degeneration patients. Eur Spine J 2014; 23(5): 1052-8.


  • There are currently no refbacks.

Creative Commons Attribution-NonCommercial 3.0

This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly.