Hereditary neuropathy with predisposition to pressure palsy

Introduction
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By Florian P Thomas MD PhD MA and Francisco de Assis Aquino Gondim MD MSc PhD

Hereditary neuropathy with predisposition to pressure palsy (HNPP) is also known as or subsumes Familial brachial plexus neuropathy. -ed.

Hereditary neuropathy with predisposition to pressure palsy may be considered the genetic opposite of CMT1A, the single most common subtype of Charcot-Marie-Tooth disease, as it is most often caused by a heterozygous deletion of the same gene region containing the PMP22 gene that is duplicated in CMT1A. HNPP may be the inherited neuropathy with the widest phenotypic range.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
Pregnancy
Anesthesia
References cited
Contributors