MBBS Student, Department of Neurology, Stanley Medical College, Chennai, Tamil Nadu, India, Orcid Id: https://orcid.org/0009-0004-4214-6793.
MBBS Student, Department of Neurology, Stanley Medical College, Chennai, Tamil Nadu, India, Orcid Id: https://orcid.org/0000-0003-3245-3816.
J Assoc Physicians India. 2024 Oct;72(10):e23-e24. doi: 10.59556/japi.72.0695.
Guillain-Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy with various subtypes, including the acute motor axonal neuropathy (AMAN) variant. Distal muscle weakness is typically rare in AMAN. Myositis, an inflammatory muscle condition, is infrequently documented in GBS. This case report presents an unusual presentation of GBS with unilateral claw hand and myositis.
A 55-year-old male presented with bilateral limb pain and weakness, progressing to significant motor impairment over 5 days. Symptoms began after a brief febrile illness with gastrointestinal distress. Upon examination, the patient exhibited decreased muscle strength in all limbs, dysphagia, and partial clawing of the left hand. Neurological assessment showed cranial nerve involvement and dysautonomia. Blood tests revealed elevated creatine phosphokinase (CPK) levels, and cerebrospinal fluid (CSF) analysis showed high protein without cellular abnormalities. Diagnosed with the AMAN variant of GBS, the patient was treated with intravenous immunoglobulin (IVIG) and antibiotics. Physiotherapy for speech, limbs, chest, and swallowing was initiated. Gradual improvement was observed, with increased limb power by the third week, although swallowing difficulties persisted longer.
This case highlights a rare presentation of the AMAN variant of GBS with unilateral claw hand and myositis. The findings suggest that elevated CPK levels in GBS may not directly indicate myositis but could be secondary to the syndrome. Prompt diagnosis and treatment of the patient for recovery have been emphasized. This report underlines the need to consider GBS in patients presenting with atypical motor impairments and elevated CPK levels.
吉兰-巴雷综合征（GBS）是一种急性自身免疫性多神经根神经病，具有多种亚型，包括急性运动轴索性神经病（AMAN）变异型。AMAN 变体通常很少出现远端肌肉无力。肌炎是一种炎症性肌肉疾病，在 GBS 中很少有记录。本病例报告介绍了一种不典型的 GBS 表现，伴有单侧爪状手和肌炎。
一名 55 岁男性因双侧肢体疼痛和无力就诊，5 天内进展为严重运动功能障碍。症状在短暂发热性疾病伴胃肠道不适后开始。检查时，患者四肢肌力下降，吞咽困难，左手部分爪状。神经系统评估显示颅神经受累和自主神经功能障碍。血液检查显示肌酸磷酸激酶（CPK）水平升高，脑脊液（CSF）分析显示蛋白升高但无细胞异常。诊断为 AMAN 变异型 GBS，给予静脉免疫球蛋白（IVIG）和抗生素治疗。开始进行言语、四肢、胸部和吞咽的物理治疗。观察到逐渐改善，第三周时四肢力量增加，但吞咽困难持续时间更长。
本病例突出了 AMAN 变异型 GBS 伴单侧爪状手和肌炎的罕见表现。研究结果表明，GBS 中 CPK 水平升高并不直接提示肌炎，而可能是该综合征的继发表现。强调了对患者进行及时诊断和治疗以促进康复。本报告强调了在出现不典型运动障碍和 CPK 水平升高的患者中考虑 GBS 的必要性。
