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Hereditary Neuropathy with Liability to Pressure Palsies: Case Report and Discussion

  • Electrodiagnostic Corner
  • Published:
HSS Journal

Abstract

Hereditary neuropathy with liability to pressure palsies (HNPP) is an uncommon diagnosis that should be considered in patients with multiple compressive neuropathies. We present the case of a woman who presented with bilateral hand numbness and weakness. Electrodiagnostic testing revealed bilateral carpal tunnel syndrome, bilateral ulnar neuropathy at the elbow, left peroneal neuropathy at the fibular head, and a primarily demyelinating generalized sensorimotor neuropathy. Subsequent genetic testing identified a deletion at chromosome 17p11.2 to confirm the diagnosis of HNPP. Treatment of this largely self-limiting disease is controversial, and this patient suffered minimal disability with treatment including splinting and surgical releases.

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Authors and Affiliations

  1. Department of Physiatry, Hospital for Special Surgery, 535 East 70th Street, New York, NY, 10021, USA

    Marc J. Grossman MD & Joseph Feinberg MD, MS

  2. Department of Laboratory Medicine, Hospital for Special Surgery, 535 East 70th Street, New York, NY, 10021, USA

    Edward F. DiCarlo MD

  3. Department of Radiology and Imaging, Hospital for Special Surgery, 535 East 70th Street, New York, NY, 10021, USA

    Sherri B. Birchansky MD

  4. Department of Orthopedic Surgery, Hospital for Special Surgery, 535 East 70th Street, New York, NY, 10021, USA

    Scott W. Wolfe MD

Authors
  1. Marc J. Grossman MD
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  2. Joseph Feinberg MD, MS
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  3. Edward F. DiCarlo MD
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  4. Sherri B. Birchansky MD
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  5. Scott W. Wolfe MD
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Corresponding author

Correspondence to Marc J. Grossman MD.

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Grossman, M.J., Feinberg, J., DiCarlo, E.F. et al. Hereditary Neuropathy with Liability to Pressure Palsies: Case Report and Discussion. HSS Jrnl 3, 208–212 (2007). https://doi.org/10.1007/s11420-007-9056-1

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  • DOI: https://doi.org/10.1007/s11420-007-9056-1

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