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Published on: November 3, 2016
When childbirth triggers a hidden neuropathy: hereditary neuropathy with liability to pressure palsies revealed by
Paula Peydro-Lavoie1, Gwendal Le Masson1,2, Stéphane Mathis1,2
1Nerve-Muscle Unit, University Hospital (CHU) of Bordeaux, Bordeaux, France.
Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder caused by a PMP22 gene deletion (or mutations), characterized by recurrent, compression-induced mononeuropathies. Postpartum neurological complications are relatively common, but HNPP is rarely considered in their differential diagnosis. We report a case of a 38-year-old woman who developed acute painless bilateral lower limb paralysis following vaginal delivery complicated by prolonged lithotomy positioning and vacuum extraction. Electroneuromyography revealed a demyelinating sensorimotor polyneuropathy with conduction block at the fibular neck, and genetic testing confirmed HNPP. This case underscores the importance of recognizing HNPP as a cause of postpartum paralysis, particularly in patients with a family history of recurrent nerve palsies. Multidisciplinary management, including genetic counseling and tailored obstetric planning for future pregnancies, is essential to optimize outcomes.
Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder caused by a PMP22 gene deletion (or mutations), characterized by recurrent, compression-induced mononeuropathies. Postpartum neurological complications are relatively common, but HNPP is rarely considered in their differential diagnosis. We report a case of a 38-year-old woman who developed acute painless bilateral lower limb paralysis following vaginal delivery complicated by prolonged lithotomy positioning and vacuum extraction. Electroneuromyography revealed a demyelinating sensorimotor polyneuropathy with conduction block at the fibular neck, and genetic testing confirmed HNPP. This case underscores the importance of recognizing HNPP as a cause of postpartum paralysis, particularly in patients with a family history of recurrent nerve palsies. Multidisciplinary management, including genetic counseling and tailored obstetric planning for future pregnancies, is essential to optimize outcomes.
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