Cranial Nerve Involvement in Neuralgic Amyotrophy

Pietro E Doneddu,Elia Sechi

doi:10.4172/2155-9562.1000399

Abstract

Neuralgic Amyotrophy (NA) is generally conceived as a multifocal neuropathy of the brachial plexus. However, different atypical forms with the involvement of other peripheral nerves, such as the lumbosacral plexus, the intercostal and the phrenic nerve and the cranial nerves (CNs), have been reported in the literature. In an attempt to shed more light on the atypical presentation of NA with CN involvement, we summarized the most relevant features of all cases of idiopathic NA with CN palsy, published up to January 2016. CN involvement in NA usually occurs with concomitant classic brachial plexopathy. Less frequently, CN palsy is the unique manifestation of the syndrome. In these cases, clinical suspicion and imaging studies are essential for a correct diagnosis, which may have therapeutic and prognostic relevance.

Highlights

Neuralgic Amyotrophy (NA) is a multifocal neuropathy that comprises an idiopathic (INA) and a hereditary form (HNA)
Focal sensory loss occurs but motor manifestations are typically much more pronounced on examination. Apart from this typical form, NA can manifest with involvement of other peripheral nerves, such as the cranial nerves (CNs), the lumbosacral plexus, the intercostal nerves and the phrenic nerve (“atypical forms”)
The most frequent associations were the bilateral involvement of the X nerve (3 cases) and of IX-X-XIXII nerves in combination (3 cases)

Summary

Introduction

Neuralgic Amyotrophy (NA) is a multifocal neuropathy that comprises an idiopathic (INA) and a hereditary form (HNA). It generally presents with a classic phenotype (“typical form”) characterized by acute-onset of severe shoulder pain followed, hoursdays later, by weakness and wasting of the shoulder girdle and arm muscles with multifocal distribution. Focal sensory loss occurs but motor manifestations are typically much more pronounced on examination. Apart from this typical form, NA can manifest with involvement of other peripheral nerves, such as the cranial nerves (CNs), the lumbosacral plexus, the intercostal nerves and the phrenic nerve (“atypical forms”). Several case reports and small case series have shown that CN involvement in NA can be highly heterogeneous, further complicating the diagnosis

Methods

Results

Discussion and Conclusion