Claw Hand in Acute Motor Axonal Neuropathy Variant.

J Assoc Physicians India

Professor and Head of Department, Department of Neurology, Stanley Medical College, Chennai, Tamil Nadu, India.

Published: October 2024

AI Article Synopsis

  • Guillain-Barré syndrome (GBS) is an autoimmune condition that can cause muscle weakness and has various forms, including acute motor axonal neuropathy (AMAN), which usually doesn't present with distal muscle weakness or myositis.
  • A 55-year-old male exhibited symptoms like limb pain, weakness, and unilateral claw hand after a mild illness, and tests showed high CPK levels and protein in cerebrospinal fluid, leading to a diagnosis of AMAN.
  • The case emphasizes the importance of recognizing GBS as a possible cause for unusual motor symptoms and elevated CPK, highlighting how prompt diagnosis and treatment can improve recovery outcomes.

Article Abstract

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.

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Source
http://dx.doi.org/10.59556/japi.72.0695DOI Listing

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