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[Chronic peripheral neuropathies with corticosensitive heredodegenerative aspects: 2 cases].

J C Antoine1, D Michel, N Kopp

  • 1Service de Neurologie, Hôpital de Bellevue, CHU Saint-Etienne.

Revue Neurologique
|January 1, 1989
PubMed
Summary
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Two childhood neuropathy cases suggest chronic inflammatory polyneuropathy (CIP) may mimic hereditary neuropathy with liability to myelin (HMSN). Prednisone treatment showed improvement, indicating potential therapeutic options for atypical presentations.

Area of Science:

  • Neurology
  • Genetics
  • Immunology

Background:

  • Hereditary neuropathy with liability to myelin (HMSN) typically presents in childhood with progressive motor and sensory deficits.
  • Differentiating HMSN from other neuropathies, such as chronic inflammatory polyneuropathy (CIP), can be challenging, especially in atypical cases.

Observation:

  • Two pediatric patients presented with symptoms suggestive of HMSN, including pes cavus and kyphoscoliosis.
  • One patient experienced relapsing sensory-motor deficits with conduction blocks, responding to prednisone, indicative of superimposed CIP.
  • The second patient had a progressive, severe neuropathy with high CSF protein and demyelination on nerve biopsy, also consistent with childhood CIP.

Findings:

  • Case 1 demonstrated improvement with prednisone, suggesting a treatable inflammatory component.

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  • Case 2's findings, including nerve biopsy results, supported a diagnosis of childhood CIP despite initial HMSN-like features.
  • Both cases highlight the heterogeneity in childhood neuropathies and the importance of considering inflammatory causes.

    • Implications:

    • These findings suggest that prednisone may be a beneficial treatment for some children with atypical HMSN presentations.
    • The study underscores the need for a broad differential diagnosis in pediatric neuropathies, including inflammatory conditions.
    • Further research into the heterogeneous physiopathology of childhood neuropathies is warranted.