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Isolated neck extensor myopathy: is it responsive to immunotherapy?

Srikanth Muppidi¹, David S Saperstein, Aziz Shaibani

¹Department of Neurology, University of Texas Southwestern Medical Center, Dallas, TX 75390-9129, USA. srikanth.muppidi@utsouthwestern.edu

Journal of Clinical Neuromuscular Disease

|September 3, 2010

Summary

Isolated neck extensor myopathy (INEM) patients showed significant neck strength improvement with immunosuppressive therapy. This suggests a trial of these agents is beneficial for INEM, even if it appears noninflammatory.

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Area of Science:

Neurology
Immunology

Background:

Isolated neck extensor myopathy (INEM) is a rare condition affecting neck extensor muscles.
The underlying cause and optimal treatment for INEM remain unclear.

Observation:

This study retrospectively reviewed four INEM patients diagnosed between 2002 and 2008.
Patients presented with neck extensor weakness, normal creatine kinase, and myopathic changes on electrodiagnostic studies of cervical paraspinal muscles.
Muscle biopsies in three patients showed no inflammation, suggesting a noninflammatory myopathy.

Findings:

All four INEM patients received immunosuppressive agents.
Neck strength improved by at least one point on the Medical Research Council scale in all patients within 3-6 months of treatment initiation.
These findings indicate a positive response to immunomodulatory therapy.

Implications:

A trial of immunosuppressive agents should be considered for patients diagnosed with INEM.
While INEM is often noninflammatory, a focal myositis might be missed on biopsy and could explain treatment response.
Further research is warranted to elucidate the pathophysiology and refine treatment strategies for INEM.