Nemaline Myopathy Associated with Monoclonal Gammopathy –  a Case Report


Authors: M. Forgáč 1;  T. Uher 1;  J. Zámečník 2
Authors‘ workplace: Neurologická klinika 1. LF UK a VFN v Praze 1;  Ústav patologie a molekulární medicíny 2. LF UK a FN v Motole, Praha 2
Published in: Cesk Slov Neurol N 2014; 77/110(2): 247-250
Category: Case Report

Práce byla podpořena granty VZ MŠM 0021620849, PRVOUK‑ P26/ LF/ 4 a projektem (Ministerstva zdravotnictví ČR) koncepčního rozvoje výzkumné organizace 00064203 (FN Motol).

Overview

Nemaline (rod) myopathy is an uncommon muscle disease with a wide spectrum of phenotypes. Since 1966 until 2008, 71 patients with sporadic late‑ onset nemaline myopathy have been described. Among them, 12 had monoclonal gammopathy. This case report describes a 64‑year‑ old man with IgG kappa monoclonal gammopathy of undetermined significance, who developed progressive muscle weakness and symmetric hypotrophy, mainly of the proximal limb muscles a year after dia­gnosis. Muscle bio­psy and electron microscopy revealed the presence of numerous typical rod‑ shaped inclusions in muscle fibers and the dia­gnosis of nemaline myopathy was established. The patient did not respond to therapy with corticosteroids, application of IVIG had also been considered. The case report is supplemented with basic information related to sporadic late‑ onset nemaline myopathy.

Key words:
nemaline myopathy – monoclonal gammopathy – muscle biopsy

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manu­script met the ICMJE “uniform requirements” for biomedical papers.


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Labels
Paediatric neurology Neurosurgery Neurology

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Czech and Slovak Neurology and Neurosurgery

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