Ann Rehabil Med.  2014 Feb;38(1):109-115. 10.5535/arm.2014.38.1.109.

Sonographic Evaluation of the Peripheral Nerves in Hereditary Neuropathy With Liability to Pressure Palsies: A Case Report

Affiliations
  • 1Department of Physical Medicine and Rehabilitation, Korea University Guro Hospital, Seoul, Korea. snyang@dreamwiz.com

Abstract

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominantly inherited disorder that affects peripheral nerves by repeated focal pressure. HNPP can be diagnosed by clinical findings, electrodiagnostic studies, histopathological features, and genetic analysis. Ultrasonography is increasingly used for the diagnosis of neuromuscular diseases; however, sonographic features of HNPP have not been clearly defined. We report the sonographic findings and comparative electrodiagnostic data in a 73-year-old woman with HNPP, confirmed by genetic analysis. The cross-sectional areas of peripheral nerves were enlarged at typical nerve entrapment sites, but enlargement at non-entrapment sites was uncommon. These sonographic features may be helpful for diagnosis of HNPP when electrodiagnostic studies are suspicious of HNPP and/or gene study is not compatible.

Keyword

Hereditary neuropathy with liability to pressure palsies; Ultrasonography; Electrophysiology

MeSH Terms

Aged
Diagnosis
Electrophysiology
Female
Humans
Nerve Compression Syndromes
Neuromuscular Diseases
Paralysis*
Peripheral Nerves*
Ultrasonography*

Figure

  • Fig. 1 Transverse sonogram of right median nerve (dotted line) at the carpal tunnel inlet (A) and the forearm (B). The median nerve is hypoechoic and enlarged (cross-sectional area 10.8 mm2 and 12.7 mm2 at the carpal tunnel inlet and in the forearm, respectively).

  • Fig. 2 Transverse sonogram of right common peroneal nerve (dotted line) at the fibular head (FH) level. The common peroneal nerve is swollen (cross-sectional area 16.2 mm2).


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