Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses

  • Chang, Soo Jin (Division of Pediatric Neurology, Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Epilepsy Research Institute, Yonsei University College of Medicine) ;
  • Lee, Ji Hyun (Division of Pediatric Neurology, Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Epilepsy Research Institute, Yonsei University College of Medicine) ;
  • Kim, Shin Hye (Department of Pediatrics, Myongji Hospital, Kwandong University College of Medicine) ;
  • Lee, Joon Soo (Division of Pediatric Neurology, Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Epilepsy Research Institute, Yonsei University College of Medicine) ;
  • Kim, Heung Dong (Division of Pediatric Neurology, Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Epilepsy Research Institute, Yonsei University College of Medicine) ;
  • Kang, Joon Won (Department of Pediatrics, Chungnam National University Hospital, Chungnam National University College of Medicine) ;
  • Lee, Young Mock (Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Kang, Hoon-Chul (Division of Pediatric Neurology, Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Epilepsy Research Institute, Yonsei University College of Medicine)
  • Received : 2013.06.14
  • Accepted : 2014.11.21
  • Published : 2015.05.10
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Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-$Barr{\acute{e}}$ syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

Keywords

References

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