Severe Cognitive Dysfunction in a Patient with Polyendocrinopathy

Abstract

Background: Polyglandular Autoimmune syndromes (PGAs) or polyendocrinopathies are immune mediated multiple endocrine gland failure sometimes accompanied by nonendocrine autoimmune disorders with varieties of presentations.

Case report: We describe a case of a middle aged man with severe cognitive dysfunction, brain atrophy, adrenal insufficiency, hypothyroidism, renal failure, thrombocytopenia, and positive antiphospholipid antibodies, with significant renal and cognitive improvement after hormone replacement.

Conclusions: PGAs may present with a broad spectrum of manifestations related to different organs like nervous,renal,cardiac and hematopoietic systems, sometimes challenging both to physician and the patient.

polyglandular autoimmune syndrome - adrenal insufficiency - dementia - antiphospholipid antibody syndrome

References

• 1 Akcay MN, Tekin SB, Akcay G. Addisonian crisis due to adrenal gland metastasis in Hodgkin's disease.  Int J Clin Pract. 2003;  57 840-841
• 2 Alarcon-Segovia D, Sanchez-Guerrero J. Correction of thrombocytopenia with small dose aspirin in the primary antiphospholipid syndrome.  J Rheumatol. 1989;  16 1359-1361
• 3 Amoroso A, Del Porto F, Garzia P, Mariotti A, Addessi MA, Afeltra A. Primary antiphospholipid syndrome and cerebral atrophy: a rare association?.  Am J Med Sci. 1999a;  317 425-428
• 4 Amoroso A, Mitterhofer AP, Ferri GM, Galluzzo S, Vadacca M, Afeltra A. Neurological involvement in antiphospholipid antibodies syndrome (APS).  Eur Rev Med Pharmacol Sci. 1999b;  3 205-209
• 5 Aumaitre O, Pouplard-Barthelaix A, Thieblot P, Dordain G, Moinade S. [Association of autoimmune dementia, Basedow's disease, and Gougerot-Sjogren syndrome].  Ann Med Interne (Paris). 1985;  136 470-473
• 6 Berneis K, Buitrago-Tellez C, Muller B, Keller U, Tsakiris DA. Antiphospholipid syndrome and endocrine damage: why bilateral adrenal thrombosis?.  Eur J Haematol. 2003;  71 299-302
• 7 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;  23 327-364
• 8 Bober E, Kovanlikaya A, Buyukgebiz A. Primary antiphospholipid syndrome: an unusual cause of adrenal insufficiency.  Horm Res. 2001;  56 140-144
• 9 Bono G, Fancellu R, Blandini F, Santoro G, Mauri M. Cognitive and affective status in mild hypothyroidism and interactions with L-thyroxine treatment.  Acta Neurol Scand. 2004;  110 59-66
• 10 Challa S, Jebakumar A, George M, William RR. Haemorrhagic pseudocyst of the adrenal in an adult (a case report).  Australas Radiol. 1989;  33 402-403
• 11 Dubovik V, Faigon M, Feldon J, Michaelson DM. Decreased density of forebrain cholinergic neurons in experimental autoimmune dementia.  Neuroscience. 1993;  56 75-82
• 12 Eilam D, Szechtman H, Faigon M, Dubovik V, Feldon J, Michaelson DM. Disintegration of the spatial organization of behavior in experimental autoimmune dementia.  Neuroscience. 1993;  56 83-91
• 13 Espinosa G, Cervera R, Font J, Asherson RA. Adrenal involvement in the antiphospholipid syndrome.  Lupus. 2003a;  12 569-572
• 14 Espinosa G, Santos E, Cervera R. et al . Adrenal involvement in the antiphospholipid syndrome: clinical and immunologic characteristics of 86 patients.  Medicine (Baltimore). 2003b;  82 106-118
• 15 Folstein MF, Folstein SE, McHugh PR. “Mini-mental state”. A practical method for grading the cognitive state of patients for the clinican.  J Psychiatr Res. 1975;  12 189-198
• 16 Folstein MF, Whitehouse PJ. Cognitive impairment of Alzheimer disease.  Neurobehav Toxicol Teratol. 1983;  5 631-634
• 17 Gomez-Puerta JA, Cervera R, Calvo LM. et al . Dementia associated with the antiphospholipid syndrome: clinical and radiological characteristics of 30 patients.  Rheumatology (Oxford). 2005;  44 95-99
• 18 Graner JL. Addison, pernicious anemia and adrenal insufficiency.  CMAJ. 1985;  133 855-857 , 880
• 19 Harper MA, Earnshaw BA. Combined adrenal and thyroid deficiency (Schmidt's syndrome) presenting as an acute psychosis.  Med J Aust. 1970;  1 546-545 , 48
• 20 Heinrich TW, Grahm G. Hypothyroidism Presenting as Psychosis: Myxedema Madness Revisited.  Prim Care Companion J Clin Psychiatry. 2003;  5 260-266
• 21 Heller T, Bergholtz M, Martin F, Borck V. [Bilateral adrenal hemorrhage occurring two times in primary antiphospholipid syndrome (APS). Anticoagulation as treatment of hemorrhage].  Dtsch Med Wochenschr. 2002;  127 1695-1698
• 22 Kalashnikova LA. [Primary antiphospholipid syndrome and cerebrovascular disturbances].  Zh Nevrol Psikhiatr Im SS Korsakova. 2005;  105 11-16
• 23 Kannan CR. Diseases of the adrenal cortex.  Dis Mon. 1988;  34 601-674
• 24 Klose M, Lange M, Kosteljanetz M, Poulsgaard L, Feldt-Rasmussen U. Adrenocortical insufficiency after pituitary surgery: an audit of the reliability of the conventional short synacthen test.  Clin Endocrinol (Oxf). 2005;  63 499-505
• 25 Lam KY, Lo CY. Metastatic tumours of the adrenal glands: a 30-year experience in a teaching hospital.  Clin Endocrinol (Oxf). 2002;  56 95-101
• 26 Lee IT, Lin SY, Sheul WH. Mood disorder and secondary adrenal insufficiency after pituitary tumor operation and radiotherapy.  J Chin Med Assoc. 2003;  66 375-378
• 27 Lee SI, Jeon HS, Yoo WH. Reversible dementia in systemic lupus erythematosus without antiphospholipid antibodies or cerebral infarction.  Rheumatol Int. 2004;  24 305-308
• 28 Lindesay J. The concept of delirium.  Dement Geriatr Cogn Disord. 1999;  10 310-314
• 29 Lopponen MK, Isoaho RE, Raiha IJ. et al . Undiagnosed diseases in patients with dementia-a potential target group for intervention.  Dement Geriatr Cogn Disord. 2004;  18 321-329
• 30 Mackworth-Young C. Primary antiphospholipid syndrome: A distinct entity?.  Autoimmun Rev. 2006;  5 70-75
• 31 Michaelson DM, Alroy G, Goldstein D, Chapman J, Feldon J. Characterization of an experimental autoimmune dementia model in the rat.  Ann N.Y. Acad Sci. 1991;  640 290-294
• 32 Michaelson DM, Dubovik V, Faigon M, Eilam D, Feldon J. Decreased density of forebrain cholinergic neurons and disintegration of the spatial organization of behavior in experimental autoimmune dementia (EAD).  Ann N.Y. Acad Sci. 1993;  695 244-248
• 33 Moudouni SM, En-Nia I, Rioux-Leclercq N, Guille F, Lobel B. Solitary contralateral adrenal metastasis after nephrectomy for renal cell carcinoma.  Urol Int. 2002;  68 295-298
• 34 Nabriski D, Ellis M, Ness-Abramof R, Shapiro M, Shenkman L. Autoimmune thyroid disease and antiphospholipid antibodies.  Am J Hematol. 2000;  64 7.75-3
• 35 Neufeld M, Maclaren N, Blizzard R. Autoimmune polyglandular syndromes.  Pediatr Ann. 1980;  9 154-162
• 36 Neufeld M, Maclaren NK, Blizzard RM. Two types of autoimmune Addison's disease associated with different polyglandular autoimmune (PGA) syndromes.  Medicine (Baltimore). 1981;  60 355-362
• 37 Opocher G, Mantero F. Adrenal complications of HIV infection.  Baillieres Clin Endocrinol Metab. 1994;  8 769-776
• 38 Oron L, Dubovik V, Novitsky L, Eilam D, Michaelson DM. Animal model and in vitro studies of anti neurofilament antibodies mediated neurodegeneration in Alzheimer's disease.  J Neural Transm Suppl. 1997;  49 77-84
• 39 Presotto F, Fornasini F, Betterle C, Federspil G, Rossato M. Acute adrenal failure as the heralding symptom of primary antiphospholipid syndrome: report of a case and review of the literature.  Eur J Endocrinol. 2005;  153 507-514
• 40 Ringkananon U, Khovidhunkit W, Vongthavaravat V, Sridama V, Lalitanantpong S, Snabboon T. Adrenal crisis due to bilateral adrenal hemorrhage in primary antiphospholipid syndrome.  J Med Assoc Thai. 2005;  88 534-537
• 41 Sanna G, Bertolaccini ML, Cuadrado MJ. et al . Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies.  J Rheumatol. 2003;  30 985-992
• 42 Shenker Y, Skatrud JB. Adrenal insufficiency in critically ill patients.  Am J Respir Crit Care Med. 2001;  163 1520-1523
• 43 Tanne D, Hassin-Baer S. Neurologic manifestations of the antiphospholipid syndrome.  Curr Rheumatol Rep. 2001;  3 286-293
• 44 Tomei E, Francone A, Salabe GB, Diacinti D, Gentile F, De Martinis C. Magnetic resonance imaging (MRI) studies of brain atrophy in hypothyroidism.  Rays. 1988;  13 43-47
• 45 Topliss DJ, White EL, Stockigt JR. Significance of thyrotropin excess in untreated primary adrenal insufficiency.  J Clin Endocrinol Metab. 1980;  50 56-52
• 46 Werber EA, Chapman J, Arlazoroff A. Brainstem auditory evoked potentials in experimental autoimmune dementia.  Neurosci Lett. 1993;  151 234-236

Correspondence