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[Chronic inflammatory demyelinating polyneuropathy in childhood]
1Department of Neurology, Hirosaki University School of Medicine, Japan.
No to Shinkei = Brain and Nerve
|March 1, 1993
Summary
Pediatric acquired demyelinating neuropathy shares features with adult CIDP but may present with pes cavus and nerve thickening. Steroid therapy is effective even with significant nerve degeneration, suggesting therapeutic trials are warranted.
Area of Science:
- Neurology
- Pediatric Neurology
- Clinical Electrophysiology
Background:
- Acquired demyelinating neuropathies in children can mimic adult Chronic Inflammatory Demyelinating Polyneuropathy (CIDP).
- Understanding unique pediatric presentations and diagnostic challenges is crucial for timely intervention.
Observation:
- Six pediatric cases of steroid-responsive acquired demyelinating neuropathy were analyzed.
- Clinical and electrophysiological findings resembled adult CIDP, with notable exceptions like pes cavus and nerve thickening in early-onset cases.
- Histopathology revealed thinly-myelinated fibers, edema, and cellular infiltrates, with varied fascicular involvement.
Findings:
- Adult CIDP diagnostic criteria are largely applicable to pediatric cases.
- Repeated electrophysiological tests may be necessary to confirm multifocal nerve lesions, especially when conduction block is subtle.
- Significant nerve degeneration on sural nerve biopsy did not preclude a good response to steroid therapy.
Implications:
- Therapeutic trials of steroids are recommended for suspected demyelinating neuropathies in children, even with clinical features suggesting genetic causes.
- Prognosis and treatment response cannot be reliably predicted by the degree of nerve degeneration in sural nerve biopsies.
- Early diagnosis and treatment of pediatric acquired demyelinating neuropathy are essential for favorable outcomes.