Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy

Margherita Milone, Amiram Katz, Anthony A. Amato, Carl A. Soderland, Miruna Segarceanu, Nathan P. Young, H. Royden Jones

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Sporadic late onset nemaline myopathy (SLONM) is a progressive myopathy of indeterminate etiology and poor outcome. If associated with a monoclonal gammopathy, SLONM carries a more unfavorable prognosis. Immunotherapy was unsuccessful. We report two HIV-negative SLONM/ monoclonal gammopathy patients who improved following intravenous immunoglobulin (IVIg) treatment alone or in combination with immunosuppressant agents. This favorable response to treatment suggests that a dysimmune mechanism is operative in some SLONM individuals. We suggest that IVIg deserves initial consideration for SLONM therapy.

Original languageEnglish (US)
Pages (from-to)272-276
Number of pages5
JournalMuscle and Nerve
Volume41
Issue number2
DOIs
StatePublished - Feb 2010

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Nemaline Myopathies
Intravenous Immunoglobulins
Immunotherapy
Paraproteinemias
Muscular Diseases
Immunosuppressive Agents
Therapeutics
HIV

Keywords

  • IVIg
  • Monoclonal gammopathy
  • Nemaline myopathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)
  • Physiology

Cite this

Milone, M., Katz, A., Amato, A. A., Soderland, C. A., Segarceanu, M., Young, N. P., & Jones, H. R. (2010). Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy. Muscle and Nerve, 41(2), 272-276. https://doi.org/10.1002/mus.21504

Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy. / Milone, Margherita; Katz, Amiram; Amato, Anthony A.; Soderland, Carl A.; Segarceanu, Miruna; Young, Nathan P.; Jones, H. Royden.

In: Muscle and Nerve, Vol. 41, No. 2, 02.2010, p. 272-276.

Research output: Contribution to journalArticle

Milone, M, Katz, A, Amato, AA, Soderland, CA, Segarceanu, M, Young, NP & Jones, HR 2010, 'Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy', Muscle and Nerve, vol. 41, no. 2, pp. 272-276. https://doi.org/10.1002/mus.21504
Milone M, Katz A, Amato AA, Soderland CA, Segarceanu M, Young NP et al. Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy. Muscle and Nerve. 2010 Feb;41(2):272-276. https://doi.org/10.1002/mus.21504
Milone, Margherita ; Katz, Amiram ; Amato, Anthony A. ; Soderland, Carl A. ; Segarceanu, Miruna ; Young, Nathan P. ; Jones, H. Royden. / Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy. In: Muscle and Nerve. 2010 ; Vol. 41, No. 2. pp. 272-276.
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