Brzozowska Malgorzata Monika, Banthia Shraddha, Thompson Simon, Narasimhan Manisha, Lee James
Department of Endocrinology, Sutherland Hospital, Sydney, NSW, Australia.
The University of New South Wales Sydney, Faculty of Medicine, Sydney, NSW, Australia.
Case Rep Endocrinol. 2021 May 19;2021:5525156. doi: 10.1155/2021/5525156. eCollection 2021.
Autoimmune hypothyroidism may result in a wide range of neuromuscular disorders. The frequently observed neurological manifestations of acquired hypothyroidism include mild to moderate myopathy and sensorimotor neuropathy, which usually resolve by clinical and electrophysiological criteria, in adults treated with thyroid hormone replacement. We report a case of a 30-year-old male with severe hypothyroidism secondary to chronic autoimmune thyroiditis who presented with a 2-year history of progressive fatigue, upper and lower limb weakness, myalgia, and intermittent paraesthesia. His neurological exam demonstrated proximal and distal muscle weakness, lower limb areflexia, and relatively intact sensory modalities. The patient's biochemistry revealed unusually and profoundly raised the thyroid stimulating hormone (TSH) level of 405.5 mIU/L (reference range (RR): 0.27-4.2 mIU/L) and creatine kinase (CK) level of 20,804 U/L (RR: 45-250 U/L), while his nerve conduction studies (NCS) demonstrated severe sensorimotor polyneuropathy with both axonal and demyelinating features. Thyroid hormone replacement therapy over the first 3 months resulted in biochemical normalization of his extremely deranged thyroid function tests (TFTs) and CK levels. At 12 months, despite maintaining euthyroidism and noticeable improvement in strength, his nerve conduction studies (NCS) demonstrated the continued absence of distal motor and sensory responses in his lower limbs with only partial improvement in sensory amplitudes and conduction velocities in his upper limbs. This report highlights the potential for severe neuromuscular consequences from advanced and chronic autoimmune hypothyroidism. The patient's myopathy has resolved over a period of three months with prompt normalization of CK levels. Concerningly, the patient achieved significant but incomplete recovery from his mixed axonal and demyelinating neuropathy with residual mild distal weakness and areflexia in his lower limbs and persistent motor and sensory impairments on his NCS. The severity and incomplete resolution of our patient's neurological manifestations emphasize the importance of early diagnosis and the need for prompt therapeutic intervention for hypothyroidism.
自身免疫性甲状腺功能减退可能导致多种神经肌肉疾病。获得性甲状腺功能减退常见的神经学表现包括轻度至中度肌病和感觉运动神经病,在接受甲状腺激素替代治疗的成年人中,这些症状通常根据临床和电生理标准得以缓解。我们报告一例30岁男性患者,继发于慢性自身免疫性甲状腺炎的严重甲状腺功能减退,有2年进行性疲劳、上下肢无力、肌痛和间歇性感觉异常病史。他的神经学检查显示近端和远端肌肉无力、下肢腱反射消失以及感觉功能相对完好。患者的生化检查显示甲状腺刺激激素(TSH)水平异常大幅升高至405.5 mIU/L(参考范围(RR):0.27 - 4.2 mIU/L),肌酸激酶(CK)水平为20,804 U/L(RR:45 - 250 U/L),而他的神经传导研究(NCS)显示严重的感觉运动性多发性神经病,具有轴索性和脱髓鞘性特征。最初3个月的甲状腺激素替代治疗使他极度紊乱的甲状腺功能检查(TFTs)和CK水平恢复生化正常。在12个月时,尽管维持了甲状腺功能正常且力量有明显改善,但他的神经传导研究(NCS)显示下肢仍持续无远端运动和感觉反应,上肢感觉波幅和传导速度仅部分改善。本报告强调了晚期慢性自身免疫性甲状腺功能减退导致严重神经肌肉后果的可能性。患者的肌病在3个月内随着CK水平迅速恢复正常而得到缓解。令人担忧的是,患者从混合性轴索性和脱髓鞘性神经病中取得了显著但不完全的恢复,下肢仍残留轻度远端无力和腱反射消失,神经传导研究仍存在持续的运动和感觉障碍。患者神经学表现的严重性和未完全缓解强调了早期诊断的重要性以及对甲状腺功能减退进行及时治疗干预的必要性。