1. bookVolumen 60 (2022): Heft 2 (June 2022)
Zeitschriftendaten
License
Format
Zeitschrift
eISSN
2501-062X
Erstveröffentlichung
30 Mar 2015
Erscheinungsweise
4 Hefte pro Jahr
Sprachen
Englisch
access type Uneingeschränkter Zugang

Autoimmune polyglandular syndrome type II presenting as subacute combined degeneration of spinal cord: a neuroendocrinology crossroad

Online veröffentlicht: 14 Jun 2022
Volumen & Heft: Volumen 60 (2022) - Heft 2 (June 2022)
Seitenbereich: 123 - 126
Eingereicht: 27 Jul 2021
Zeitschriftendaten
License
Format
Zeitschrift
eISSN
2501-062X
Erstveröffentlichung
30 Mar 2015
Erscheinungsweise
4 Hefte pro Jahr
Sprachen
Englisch
Abstract

Introduction: Autoimmune polyglandular syndrome (APS) is a condition having multiple endocrine abnormalities. It is divided into three types depending on the involvement of various endocrinopathies. It is also associated with other systemic involvement. The basic pathophysiology of this syndrome revolves around autoimmunity.

Case presentation: We discuss the case of a 50-year-old gentleman who presented to us in emergency with subacute onset progressive weakness of both lower limbs followed by upper limbs. On examination, patient was confused and disoriented. General examination findings include hypotension, pallor, facial puffiness and vitiligo. Neurological examination revealed spasticity and motor weakness in all four limbs with extensor planter response. Sensory examination during hospital course revealed posterior column involvement. Laboratory and radiological investigations confirmed subacute combined degeneration of spinal cord secondary to pernicious anaemia, Addison’s disease and autoimmune thyroid disease. The final diagnosis of autoimmune polyglandular syndrome type II was made after fulfilment of the required criteria.

Conclusion: Autoimmune polyglandular syndrome type II can rarely present to neurologist as subacute combined degeneration of spinal cord. This syndrome and its systemic association should be kept in mind in order to reach the final diagnosis.

1. HUSEBYE ES, ANDERSON MS, KÄMPE O. Autoimmune Polyendocrine Syndromes. N Engl J Med. 2018 Mar 22; 378(12):1132–1141.10.1056/NEJMra1713301Search in Google Scholar

2. CHEN QY, KUKREJA A, MACLAREN NK. The Autoimmune Polyglandular Syndromes. In: Endocrinology. W.B. Saunders, Philadelphia, 2001:587–99.Search in Google Scholar

3. SCHATZ DA, WINTER WE. Autoimmune polyglandular syndrome. II: Clinical syndrome and treatment. Endocrinol Metab Clin North Am. 2002 Jun; 31(2):339–52.10.1016/S0889-8529(01)00012-3Search in Google Scholar

4. SCHMIDT MB. Eine biglandulare Erkrankung (Nebennieren und Schilddruse) bei Morbus Addisonii. Verh Dtsch Ges Pathol. 1926; 21:212–221.Search in Google Scholar

5. ROBLES DT, FAIN PR, GOTTLIEB PA, EISENBARTH GS. The genetics of autoimmune polyendocrine syndrome type II. Endocrinol Metab Clin North Am. 2002 Jun; 31(2):353–68, vi-vii.10.1016/S0889-8529(01)00015-9Search in Google Scholar

6. 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 Jun;23(3):327–64. Erratum in: Endocr Rev. 2002 Aug; 23(4):579.10.1210/edrv.23.3.046612050123Search in Google Scholar

7. PETER A. GOTTLIEB, AARON W. MICHELS. Autoimmune Polyendocrine Syndromes. In: Harrison’s Principles of Internal Medicine, McGraw-Hill Education, New York, 2018:382:2756.Search in Google Scholar

Empfohlene Artikel von Trend MD

Planen Sie Ihre Fernkonferenz mit Scienceendo