ArticleInternal medicine (Tokyo, Japan)2026
Hereditary Neuropathy with Liability to Pressure Palsies Mimicking a Chronic Inflammatory Demyelinating Polyneuropathy Variant: A Case Report Highlighting Diagnostic Challenges.
Article in Internal medicine (Tokyo, Japan), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
We herein report a case of chronic inflammatory demyelinating polyneuropathy (CIDP) that was ultimately identified as hereditary neuropathy with liability to pressure palsies (HNPP) based on PMP22 gene testing. The patient exhibited gradually progressive relapsing-remitting sensorimotor symptoms without obvious nerve compression episodes and received immunotherapy for CIDP for one year without improvement. This case highlights the clinical and electrophysiological challenges of differentiating long-standing HNPP from CIDP. In refractory CIDP cases with bilateral foot drop and immunotherapy resistance, HNPP should be considered and genetic testing performed, even in the absence of a family history.
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