Cervicobrachial Polymyositis

Abstract

Cervicobrachial polymyositis presents with predominant or isolated proximal upper limb and neck weakness. It has marked female preponderance and affects neck flexors and extensors and trapezius and deltoid muscles severely. Wrist and finger extensors are also weakened. In a proportion of patients, it is associated with serological markers of autoimmune disorders and interstitial lung disease. In addition to serum creatine kinase, electromyography, and muscle biopsy, magnetic resonance imaging forms a noninvasive adjunct test in the diagnostic process. Because of the pattern of weakness, cervicobrachial polymyositis tends to be confused with other common neurological conditions having upper girdle weakness, leading to delay in the diagnosis of this potentially treatable condition.

Brachio-cervical inflammatory myopathy with associated scleroderma phenotype and lupus serology

“…A series of 14 patients with “cervicobrachial polymyositis” described clinical features consistent with BCIM, but muscle biopsy only described interstitial mononuclear inflammatory infiltrates without elaboration. 4 Consistent with our case, several reports of scleroderma have been associated with brachio-cervical weakness. One described B-cell inflammatory infiltrates on muscle biopsy but did not describe MAC deposition, 5 while a series of 5 patients showed inflammatory infiltrates without elaboration.…”

“…In the larger series, 1 , 4 the referring diagnoses were rarely myopathy (with motor neuron disease and myasthenia gravis being the most common), highlighting that the brachio-cervical pattern of weakness is an under-recognized presentation of inflammatory myopathy. This has important clinical implications, given the treatable nature of this condition.…”

Long-term Follow-up and Muscle Imaging Findings in Brachio-Cervical Inflammatory Myopathy

“…After the first description of this entity, only few other reports have been published, mostly highlighting the prevalence of the disease among the female patients and the response to immunosuppressive agents. [8][9][10][11] MRI, that has been lately used in genetic and inflammatory myopathies for diagnostic purposes, sometimes providing specific patterns of involvement, and in follow-up, for the evaluation of disease progression and treatment response, has not been systematically investigated in BCIM. 12,13 Here, we report clinical and instrumental findings of patients followed at the Fondazione Policlinico Universitario A. Gemelli IRCCS affected by BCIM focusing on radiologic, histopathologic, and serologic assessments at baseline and after long-term follow-up.…”

“…Weakness of distal upper limb muscles, especially in the posterior forearm, was also always present in our cohort although not frequently reported in BCIM so far. 10 Lower limb involvement was rare, even if it might be present in very advanced disease.…”

“…The slowly progressive course and this misleading clinical picture led to a mean diagnostic delay of approximately 1.5 years, which was comparable to previous studies, yet undesirable in a potentially treatable condition. 10,11 Muscle MRI examination could narrow this delay and is instrumental in differentiating this entity from other IIMs, motor neuron diseases, or hereditary myopathies.…”