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Neurology International
Volume 8
Issue 3
10.4081/ni.2016.6677
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Neurology International is published by MDPI from Volume 12 Issue 3 (2020). Previous articles were published by another publisher in Open Access under a CC-BY licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Case Report

Hereditary Neuropathy with Liability to Pressure Palsies: A Single-Center Experience in Southern Brazil

by
Paulo José Lorenzoni
*,
Cláudia Suemi Kamoi Kay
,
Cristiane Cavalet
,
Raquel C. Arndt
,
Lineu Cesar Werneck
and
Rosana Herminia Scola
Service of Neuromuscular Disorders, Division of Neurology, Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná (UFPR), Curitiba, Brazil
*
Author to whom correspondence should be addressed.
Neurol. Int. 2016, 8(3), 6677; https://doi.org/10.4081/ni.2016.6677
Submission received: 4 July 2016 / Revised: 5 August 2016 / Accepted: 8 August 2016 / Published: 30 September 2016

Abstract

The spectrum of clinical and electrophysiological features in hereditary neuropathy with liability to pressure palsies (HNPP) is broad. We analyze a series of Brazilian patients with HNPP. Correlations between clinical manifestations, laboratory features, electrophysiological analyze, histological and molecular findings were done. In five cases, more than one episode occurred before diagnosis. Median nerve in the carpal tunnel at the wrist, ulnar nerve in its groove at the elbow, fibular nerve in the head of the fibula at the knee, radial nerve in its groove of the humerus and suprascapular nerve in its notch at the supraspinous fossa were found as focal neuropathies. One patient presented with persistent writer’s cramp after ulnar nerve palsy. Nerve conduction studies showed focal neuropathy in all patients and concomitant generalized symmetrical neuropathy in eight patients. Molecular analysis of the PMP22 gene detected deletion of the 1.5-Mb fragment in all patients.
Keywords: Hereditary neuropathy with liability to pressure palsies; hereditary neuropathy; dystonia; writer’s cramp; electrophysiological study Hereditary neuropathy with liability to pressure palsies; hereditary neuropathy; dystonia; writer’s cramp; electrophysiological study

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MDPI and ACS Style

Lorenzoni, P.J.; Kay, C.S.K.; Cavalet, C.; Arndt, R.C.; Werneck, L.C.; Scola, R.H. Hereditary Neuropathy with Liability to Pressure Palsies: A Single-Center Experience in Southern Brazil. Neurol. Int. 2016, 8, 6677. https://doi.org/10.4081/ni.2016.6677

AMA Style

Lorenzoni PJ, Kay CSK, Cavalet C, Arndt RC, Werneck LC, Scola RH. Hereditary Neuropathy with Liability to Pressure Palsies: A Single-Center Experience in Southern Brazil. Neurology International. 2016; 8(3):6677. https://doi.org/10.4081/ni.2016.6677

Chicago/Turabian Style

Lorenzoni, Paulo José, Cláudia Suemi Kamoi Kay, Cristiane Cavalet, Raquel C. Arndt, Lineu Cesar Werneck, and Rosana Herminia Scola. 2016. "Hereditary Neuropathy with Liability to Pressure Palsies: A Single-Center Experience in Southern Brazil" Neurology International 8, no. 3: 6677. https://doi.org/10.4081/ni.2016.6677

APA Style

Lorenzoni, P. J., Kay, C. S. K., Cavalet, C., Arndt, R. C., Werneck, L. C., & Scola, R. H. (2016). Hereditary Neuropathy with Liability to Pressure Palsies: A Single-Center Experience in Southern Brazil. Neurology International, 8(3), 6677. https://doi.org/10.4081/ni.2016.6677

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