J Electrodiagn Neuromuscul Dis.  2021 Aug;23(2):55-61. 10.18214/jend.2021.00010.

Juvenile-Onset Relapsing Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Case Report

Affiliations
  • 1Department of Physical Medicine and Rehabilitation, Korea University Anam Hospital, Seoul, Korea
  • 2Department of Pediatrics, Korea University Anam Hospital, Seoul, Korea
  • 3Department of Biomedical Sciences, Korea University College of Medicine, Seoul, Korea
  • 4Brain Convergence Research Center, Korea University College of Medicine, Seoul, Korea

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, immune-mediated condition. It is a symmetric, motor-predominant neuropathy that results in both proximal and distal limb muscle weakness and is characterized by electrodiagnostic or pathologic features of demyelination. CIDP is a treatable disease that is known to be rare in the pediatric population. This case report describes a 12-year-old female who presented with gait disturbance and progressive upper and lower limb weakness. The electrophysiologic findings were compatible with demyelinating polyneuropathy. Combined with the clinical features, the diagnosis of CIDP was made and treatment was administered. The first-line immunomodulatory treatment seemed to be effective, as shown by improvements in electrophysiologic and clinical parameters, but the relapsing-remitting clinical course required additional immunomodulatory treatment. Herein, we describe the patient’s clinical and electrophysiologic course according to the treatment.

Keyword

Child; Polyradiculoneuropathy, chronic inflammatory demyelinating; Electrodiagnosis
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