TY - JOUR
T1 - Isolated neck extensor myopathy
T2 - Is it responsive to immunotherapy?
AU - Muppidi, Srikanth
AU - Saperstein, David S.
AU - Shaibani, Aziz
AU - Nations, Sharon P.
AU - Vernino, Steven
AU - Wolfe, Gil I.
PY - 2010/9/1
Y1 - 2010/9/1
N2 - Objective: To determine if isolated neck extensor myopathy (INEM) is responsive to immunosuppressive treatment. Methods: We retrospectively reviewed charts of patients with INEM from 2002 to 2008 to identify patients and determine the response to immunomodulatory therapy. Clinical, electrodiagnostic, histologic, and radiographic data were reviewed. Results: Four patients were identified during the study period. Three were women. The age of onset of neck extensor weakness ranged from 58 to 78 years. Serum creatine kinase levels were within normal limits in all patients. None had clinical, laboratory, or electrophysiological findings to suggest a generalized neuromuscular disorder. On electrodiagnostic studies, all patients had myopathic changes with or without irritative features in cervical paraspinal muscles. No inflammation was present on muscle biopsy from three of the patients. All patients received one or more immunosuppressive agents. Neck strength improved by 1 point or greater on the Medical Research Council scale in all subjects with a peak response observed between 3 and 6 months after treatment initiation. Conclusions: A trial of immunosuppressive agents should be offered to patients with INEM because a subset will improve. Rigorously defined, INEM is a noninflammatory myopathy. However, a focal myositis could be missed on muscle biopsy and may explain the favorable response to treatment.
AB - Objective: To determine if isolated neck extensor myopathy (INEM) is responsive to immunosuppressive treatment. Methods: We retrospectively reviewed charts of patients with INEM from 2002 to 2008 to identify patients and determine the response to immunomodulatory therapy. Clinical, electrodiagnostic, histologic, and radiographic data were reviewed. Results: Four patients were identified during the study period. Three were women. The age of onset of neck extensor weakness ranged from 58 to 78 years. Serum creatine kinase levels were within normal limits in all patients. None had clinical, laboratory, or electrophysiological findings to suggest a generalized neuromuscular disorder. On electrodiagnostic studies, all patients had myopathic changes with or without irritative features in cervical paraspinal muscles. No inflammation was present on muscle biopsy from three of the patients. All patients received one or more immunosuppressive agents. Neck strength improved by 1 point or greater on the Medical Research Council scale in all subjects with a peak response observed between 3 and 6 months after treatment initiation. Conclusions: A trial of immunosuppressive agents should be offered to patients with INEM because a subset will improve. Rigorously defined, INEM is a noninflammatory myopathy. However, a focal myositis could be missed on muscle biopsy and may explain the favorable response to treatment.
KW - head drop
KW - immunosuppression
KW - myopathy
KW - neck extensors
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U2 - 10.1097/CND.0b013e3181d4a515
DO - 10.1097/CND.0b013e3181d4a515
M3 - Review article
C2 - 20808161
AN - SCOPUS:77956441856
SN - 1522-0443
VL - 12
SP - 26
EP - 29
JO - Journal of Clinical Neuromuscular Disease
JF - Journal of Clinical Neuromuscular Disease
IS - 1
ER -