Hereditary Neuropathy with Liability to Pressure Palsies Presenting with Bilateral Brachial Plexopathy after Liposuction - Case Report

Hereditary neuropathy with liability to pressure palsies (HNPP) is a rare hereditary disorder characterized by recurrent episodes of nerve compression. The first attack usually occurs in the second or third decade of life. In the majority of cases, HNPP is associated with a mutation at chromosome 17 p11.2-12 comprising the gene encoding peripheral myelin protein 22 (PMP22). Here we present a case report of a 43-year-old male with HNPP confirmed by DNA testing. The patient complained of recurrent episodes of bilateral foot extensor muscles weakness and/or hyperesthesia on the outer surface of the hands and forearms, which started after a prolonged posture maintaining and without evident precipitating factors. We also describe typical clinical, electrophysiological, and nerve ultrasound characteristics of the disease.

Download Full-text

Elderly-onset case of hereditary neuropathy with liability to pressure palsies: A case report

Neurology and Clinical Neuroscience ◽

10.1111/ncn3.12214 ◽

2018 ◽

Vol 6 (5) ◽

pp. 141-142

Author(s):

Tatsuya Sato ◽

Risa Maekawa ◽

Akihiko Mitsutake ◽

Junko Katsumata ◽

Tomonari Seki ◽

...

Keyword(s):

Case Report ◽

Hereditary Neuropathy ◽

Elderly Onset ◽

Pressure Palsies

Download Full-text

P28-11 Brachial plexopathy as the initial presentation in patient with hereditary neuropathy with liability to pressure palsies (HNPP)

Clinical Neurophysiology ◽

10.1016/s1388-2457(10)61106-6 ◽

2010 ◽

Vol 121 ◽

pp. S271 ◽

Cited By ~ 1

Author(s):

L.B. Tay ◽

K.K. Verma ◽

E. Pica

Keyword(s):

Initial Presentation ◽

Hereditary Neuropathy ◽

Brachial Plexopathy ◽

Pressure Palsies

Download Full-text

Clinical and neurophysiological features of the hereditary neuropathy with liability to pressure palsy due to the 17p11.2 deletion

Arquivos de Neuro-Psiquiatria ◽

10.1590/0004-282x20160010 ◽

2016 ◽

Vol 74 (2) ◽

pp. 99-105 ◽

Cited By ~ 4

Author(s):

Aline Pinheiro Martins de Oliveira ◽

Raquel Campos Pereira ◽

Patrícia Toscano Onofre ◽

Vanessa Daccach Marques ◽

Gilberto Brown de Andrade ◽

...

Keyword(s):

Nerve Conduction Studies ◽

Frequent Pattern ◽

Hereditary Neuropathy ◽

Brachial Plexopathy ◽

Motor Neuropathy ◽

Sensorimotor Polyneuropathy ◽

Pressure Palsy ◽

Pressure Palsies ◽

Sensory Polyneuropathy ◽

17P11.2 Deletion

ABSTRACT The hereditary neuropathy with liability to pressure palsies (HNPP) is an autossomal dominant disorder manifesting recurrent mononeuropathies. Objective Evaluate its clinical and nerve conduction studies (NCS) characteristics, searching for diagnostic particularities. Method We reviewed the neurological manifestations of 39 and the NCS of 33 patients. Results Family history was absent in 16/39 (41%). The onset complaints were weakness in 24, pain in 6, sensory deficit in 5 and paresthesias in 4. Pain was seen in 3 other patients. The following neuropathy patterns were found: multiple mononeuropathy (26), mononeuropathy (7), chronic sensorimotor polyneuropathy (4), chronic sensory polyneuropathy (1) and unilateral brachial plexopathy (1). NCS showed a sensorimotor neuropathy with focal conduction slowing in 31, two had mononeuropathy and another brachial plexopathy. Conclusion HNPP presentation is variable and may include pain. The most frequent pattern is of an asymmetrical sensory and motor neuropathy with focal slowing at specific topographies on NCS.

Download Full-text