表现为亚急性脊髓合并变性的自身免疫性多腺综合征II型:神经内分泌学的十字路口

IF 1.6 Q2 MEDICINE, GENERAL & INTERNAL Romanian Journal of Internal Medicine Pub Date : 2022-06-14 Print Date: 2022-06-01 DOI:10.2478/rjim-2021-0038
Prateek Bapat, Suman Kushwaha, Chirag Gupta, R Kirangowda, Vaibhav Seth
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引用次数: 2

摘要

自身免疫性多腺综合征(APS)是一种多发性内分泌异常的疾病。根据各种内分泌疾病的参与情况分为三种类型。它还与其他系统性参与有关。这种综合征的基本病理生理学与自身免疫有关。病例介绍:我们讨论的情况下,一位50岁的绅士谁向我们提出了紧急亚急性发作进行性无力的双下肢随后上肢。检查时,病人神志不清,没有方向感。一般检查结果包括低血压、脸色苍白、面部浮肿和白癜风。神经学检查显示四肢痉挛和运动无力,并伴有伸肌种植反应。住院期间感觉检查发现后柱受累。实验室和放射检查证实继发于恶性贫血、阿狄森氏病和自身免疫性甲状腺疾病的脊髓亚急性合并变性。自身免疫性多腺综合征II型的最终诊断是在满足要求标准后做出的。结论:自身免疫性多腺综合征II型很少以亚急性脊髓合并变性的形式出现在神经科医生面前。为了达到最终诊断,应牢记这种综合征及其系统性关联。
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Autoimmune polyglandular syndrome type II presenting as subacute combined degeneration of spinal cord: a neuroendocrinology crossroad.

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.

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来源期刊
Romanian Journal of Internal Medicine
Romanian Journal of Internal Medicine MEDICINE, GENERAL & INTERNAL-
CiteScore
3.20
自引率
5.30%
发文量
35
审稿时长
15 weeks
期刊介绍: Romanian Journal of Physics is a journal publishing physics contributions on the following themes: •Theoretical Physics & Applied Mathematics •Nuclear Physics •Solid State Physics & Materials Science •Statistical Physics & Quantum Mechanics •Optics •Spectroscopy •Plasma & Lasers •Nuclear & Elementary Particles Physics •Atomic and Molecular Physics •Astrophysics •Atmosphere and Earth Science •Environment Protection
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