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Hereditary neuropathy with liability to pressure palsies: a case report

https://doi.org/10.14412/2074-2711-2021-4-116-122

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Abstract

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.

About the Authors

A. K. Polynnikova
Department of Nervous System Diseases and Neurosurgery, N.V. Sklifosovsky Institute of Clinical Medicine
Russian Federation

Anastasia Konstantinovna Polynnikova.

11, Rossolimo St., Build. 1, Moscow 119021.


Competing Interests:

There are no conflicts of interest.



O. E. Zinovyeva
Department of Nervous System Diseases and Neurosurgery, N.V. Sklifosovsky Institute of Clinical Medicine
Russian Federation

11, Rossolimo St., Build. 1, Moscow 119021.


Competing Interests:

There are no conflicts of interest.



O. A. Solokha
Department of Nervous System Diseases and Neurosurgery, N.V. Sklifosovsky Institute of Clinical Medicine; A.Ya. Kozhevnikov Clinic of Nervous System Diseases, I.M.Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation

11, Rossolimo St., Build. 1, Moscow 119021.


Competing Interests:

There are no conflicts of interest.



E. V. Misyuryaeva
A.Ya. Kozhevnikov Clinic of Nervous System Diseases, I.M.Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation

11, Rossolimo St., Build. 1, Moscow 119021.


Competing Interests:

There are no conflicts of interest.



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For citation:


Polynnikova A.K., Zinovyeva O.E., Solokha O.A., Misyuryaeva E.V. Hereditary neuropathy with liability to pressure palsies: a case report. Neurology, Neuropsychiatry, Psychosomatics. 2021;13(4):116-122. (In Russ.) https://doi.org/10.14412/2074-2711-2021-4-116-122

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ISSN 2074-2711 (Print)
ISSN 2310-1342 (Online)