Hereditary Brachial Plexus Neuropathy

Hereditary Brachial Plexus Neuropathy
HBPN-presents with attacks of multifocal nerve injury affecting the upper limbs.
Attacks are acute or sub acute in onset, often associated with severe pain, significant morbidity and disability.
Pathophysiologic localization in unilateral or asymmetrical in the brachial plexus or nerves arising from the plexus or roots, without a more generalized peripheral neuropathy (distinguishing from HNPP-more generalized with focal changes).
Historical overview

1886 Dreschfeld described 43 year old female with 3 discrete episodes; termed “rheumatic peripheral neuritis” mentioned pt’s sister had similar attacks.
1961-Jacob et al. described in 7 pts in two unrelated families attacks.
Features of HBPN

Autosomal dominant with varied penetrance and expression.
Recurrent attacks.
Associated with stressors-pregnancy and parturition, mild trauma, infection, immunization.
Multifocal or patchy axonal degeneration of upper limb nerves, plexus, possibly roots, with inflammation in areas of degenerating fibers.
The most important feature between HBPN and sporadic form is family history.

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