Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients

Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients Publisher Pubmed

Okhovat AA^{1, 2} ; Nilipour Y^{3, 4} ; Boostani R⁵ ; Vahabizad F² ; Najmi S⁶ ; Nafissi S¹ ; Fatehi F¹

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1. Neurology Department, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
2. Neurology Department, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran
3. Pediatric Pathology Research Center, Research Institute for Children's health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
4. Mofid Children Hospital, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran
5. Neurology Department, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran
6. Department of Neurology, Tabriz University of Medical Science, Tabriz, Iran

Source: Neuromuscular Disorders Published:2021

Abstract

Sporadic late-onset nemaline myopathy (SLONM) is a rare, acquired muscle disease presenting with subacute progression in adulthood. It can be accompanied by a monoclonal gammopathy of undetermined significance (MGUS). We describe clinical and histopathological findings of four SLONM patients with MGUS. In all patients, nemaline rod, inter-myofibrillary network disruption, atrophic changes, peripheral basophilic discoloration, vacuole without rim, and cytoplasmic body without inflammation were seen. Three out of four patients were treated with prednisolone in combination with IVIG monthly and had an appropriate response to the treatment. The optimal first-line treatment remains unclear in SLONM-MGUS, although corticosteroids plus IVIg is associated with favorable clinical response. These treatment modalities might be used as an optional treatment before autologous stem cell transplantation; however, further studies with a higher number of patients are required. © 2020 Elsevier B.V.

Style	Citing Format
MLA	Okhovat AA, et al.. "Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients." Neuromuscular Disorders, vol. 31, no. 1, 2021, pp. 29-34.
APA	Okhovat AA, Nilipour Y, Boostani R, Vahabizad F, Najmi S, Nafissi S, Fatehi F (2021). Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients. Neuromuscular Disorders, 31(1), 29-34.
Chicago	Okhovat AA, Nilipour Y, Boostani R, Vahabizad F, Najmi S, Nafissi S, Fatehi F. "Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients." Neuromuscular Disorders 31, no. 1 (2021): 29-34.
Harvard	Okhovat AA et al. (2021) 'Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients', Neuromuscular Disorders, 31(1), pp. 29-34.
Vancouver	Okhovat AA, Nilipour Y, Boostani R, Vahabizad F, Najmi S, Nafissi S, et al.. Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients. Neuromuscular Disorders. 2021;31(1):29-34.
BibTex	@article{ author = {Okhovat AA and Nilipour Y and Boostani R and Vahabizad F and Najmi S and Nafissi S and Fatehi F}, title = {Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients}, journal = {Neuromuscular Disorders}, volume = {31}, number = {1}, pages = {29-34}, year = {2021} }
RIS	TY - JOUR AU - Okhovat AA AU - Nilipour Y AU - Boostani R AU - Vahabizad F AU - Najmi S AU - Nafissi S AU - Fatehi F TI - Sporadic Late-Onset Nemaline Myopathy With Monoclonal Gammopathy of Undetermined Significance: Report of Four Patients JO - Neuromuscular Disorders VL - 31 IS - 1 SP - 29 EP - 34 PY - 2021 ER -

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