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Cervicobrachial polymyositis.

Satish V Khadilkar1, Namit Gupta, Rakhil S Yadav

  • 1*Department of Neurology, Grant Medical College and Sir JJ Group of Hospitals, Mumbai, India; †Bombay Hospitals, Mumbai, India; and ‡Artemis Health Institute, Gudgeon, India.

Journal of Clinical Neuromuscular Disease
|November 22, 2014
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Summary
This summary is machine-generated.

This study characterizes cervicobrachial polymyositis, a rare inflammatory myopathy causing upper limb and neck weakness. Early diagnosis is crucial as it mimics neurological conditions but is treatable with corticosteroids.

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Area of Science:

  • Neurology
  • Immunology
  • Rheumatology

Background:

  • Polymyositis is an inflammatory myopathy characterized by muscle weakness.
  • A specific subgroup presents with predominant or isolated proximal upper limb and neck weakness, often misdiagnosed.

Purpose of the Study:

  • To characterize and analyze a subgroup of patients with polymyositis presenting with predominant or isolated proximal upper limb and neck weakness.
  • To highlight diagnostic challenges and treatment responses in this specific polymyositis subtype.

Main Methods:

  • Retrospective analysis of 14 polymyositis patients with upper limb/neck weakness over 14 years (1999-2013).
  • Inclusion based on Bohan and Peter and Targoff criteria.
  • Evaluation of clinical, laboratory, histopathological, and radiological profiles.

Main Results:

  • Predominant or isolated proximal upper limb and neck weakness observed in all patients (86% female, mean age 37).
  • Commonly misdiagnosed as motor neuron disease, radiculopathy, or myasthenia gravis.
  • Elevated creatine kinase (CK), associated autoimmune antibodies (42.8%), and interstitial lung disease (36%) noted.
  • Muscle biopsy confirmed inflammatory myopathy; MRI showed muscle abnormalities.

Conclusions:

  • Cervicobrachial polymyositis presents with distinct upper limb and neck weakness patterns, often confused with neurological disorders.
  • Diagnosis requires a combination of clinical findings, elevated CK, EMG, muscle biopsy, and MRI.
  • Prompt corticosteroid treatment is effective, with 86% showing response.