M Chozhan, P Vishaal, Gurunathan Srinivasan, Lakshmanan Sankaranarayanan, Mugundhan Krishnan
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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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":22693,"journal":{"name":"The Journal of the Association of Physicians of India","volume":"72 10","pages":"e23-e24"},"PeriodicalIF":0.0000,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Claw Hand in Acute Motor Axonal Neuropathy Variant.\",\"authors\":\"M Chozhan, P Vishaal, Gurunathan Srinivasan, Lakshmanan Sankaranarayanan, Mugundhan Krishnan\",\"doi\":\"10.59556/japi.72.0695\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Case description: </strong>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.</p><p><strong>Conclusion: </strong>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. 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引用次数: 0
摘要
背景:吉兰-巴雷综合征(GBS)是一种急性自身免疫性多发性神经病,有多种亚型,包括急性运动性轴索神经病(AMAN)变异型。在 AMAN 中,远端肌无力通常比较少见。肌炎是一种肌肉炎症,在 GBS 中很少见。本病例报告了一种表现不寻常的 GBS,伴有单侧爪手和肌炎:病例描述:一名 55 岁的男性患者出现双侧肢体疼痛和无力,并在 5 天内发展为明显的运动障碍。症状始于短暂发热并伴有胃肠不适。经检查,患者四肢肌力下降,吞咽困难,左手部分呈爪状。神经系统评估显示,患者有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)分析显示高蛋白,无细胞异常。患者被诊断为 AMAN 变异型 GBS,接受了静脉注射免疫球蛋白(IVIG)和抗生素治疗。患者开始接受语言、四肢、胸部和吞咽方面的物理治疗。虽然吞咽困难持续了较长时间,但患者的病情逐渐好转,第三周时肢体力量有所增强:本病例强调了一种罕见的伴有单侧爪手和肌炎的 AMAN 变异型 GBS。研究结果表明,GBS 患者 CPK 水平升高可能并不直接表明患有肌炎,但可能继发于该综合征。我们强调要对患者进行及时诊断和治疗,以促进康复。本报告强调,对于出现不典型运动障碍和 CPK 水平升高的患者,有必要考虑 GBS。
Claw Hand in Acute Motor Axonal Neuropathy Variant.
Background: 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.
Case description: 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.
Conclusion: 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.