PMID- 25415516
OWN - NLM
STAT- MEDLINE
DCOM- 20151104
LR  - 20141122
IS  - 1537-1611 (Electronic)
IS  - 1522-0443 (Linking)
VI  - 16
IP  - 2
DP  - 2014 Dec
TI  - Cervicobrachial polymyositis.
PG  - 59-68
LID - 10.1097/CND.0000000000000059 [doi]
AB  - OBJECTIVES: To characterize and analyze a subgroup of patients with polymyositis 
      presented with predominant or isolated proximal upper limb and neck weakness. 
      METHODS: Patients with polymyositis, presenting with predominant or isolated 
      weakness of upper limbs and the neck, were included. Bohan and Peter and Targoff 
      criteria were used for the diagnosis of polymyositis. The study period was of 14 
      years (from 1999 to 2013). Detailed clinical, laboratory, histopathological, 
      and/or radiological profiles were evaluated and analyzed in all patients. 
      RESULTS: The mean age of presentation was 37 years (23-58 years), and 86% (12/14) 
      patients were female. All patients developed progressive symmetrical weakness 
      predominantly affecting upper limbs, and 50% (7/14) had weakness only in upper 
      limbs. The referring diagnoses in all 14 patients were other than polymyositis, 
      for example, motor neuron disease, cervical radiculopathy, brachial neuritis, 
      chronic inflammatory demyelinating polyradiculoneuropathy, anti-MuSK myasthenia 
      gravis, and facioscapulohumeral muscular dystrophy. Weakness in the neck and 
      shoulder region was the most common presenting complaints, presenting in 71.4% 
      (10/14) and 64.3% (9/14) of patients, respectively, followed by neck and shoulder 
      pain in 57.14% (8/14) patients. The most severely affected muscles were neck 
      flexors, neck extensors, the trapezius, and the deltoid. Less severe but 
      preferential involvement of wrist and finger extensors was present. Serum 
      creatine kinase was elevated (median CK, 1275 IU/L). Of note, 42.8% (6/14) had 
      associated systemic autoimmune antibodies such as ANA, RA, and anti-Ro 
      antibodies, whereas 36% (5/14) patients had associated interstitial lung disease 
      (ILD). Active irritative myopathy was found in 93% (13/14) patients on needle 
      electromyography studies, and all patients had evidence of inflammatory myopathy 
      (polymyositis) on muscle biopsy. MRI of the cervicobrachial region was performed 
      in the first 6 patients showing T2 hyper intensities (6/6) and contrast 
      enhancement (4/4) of clinically affected muscles. Eighty-six percent (12/14) of 
      patients responded to corticosteroids with an average response initiation time of 
      7.4 weeks (1-12 weeks). Subsequently used steroid sparing agents were 
      azathioprine (6/14) and methotrexate (6/14). Intravenous immunoglobulin (IVIG) 
      was used in 2 patients and plasmapharesis in 1 patient. CONCLUSIONS: 
      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.
FAU - Khadilkar, Satish V
AU  - Khadilkar SV
AD  - *Department of Neurology, Grant Medical College and Sir JJ Group of Hospitals, 
      Mumbai, India; daggerBombay Hospitals, Mumbai, India; and double daggerArtemis Health Institute, 
      Gudgeon, India.
FAU - Gupta, Namit
AU  - Gupta N
FAU - Yadav, Rakhil S
AU  - Yadav RS
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Neuromuscul Dis
JT  - Journal of clinical neuromuscular disease
JID - 100887391
SB  - IM
MH  - Adult
MH  - Female
MH  - Humans
MH  - Longitudinal Studies
MH  - Male
MH  - Middle Aged
MH  - Neck
MH  - Polymyositis/*diagnosis/physiopathology
MH  - Retrospective Studies
MH  - Upper Extremity
MH  - Young Adult
EDAT- 2014/11/22 06:00
MHDA- 2015/11/05 06:00
CRDT- 2014/11/22 06:00
PHST- 2014/11/22 06:00 [entrez]
PHST- 2014/11/22 06:00 [pubmed]
PHST- 2015/11/05 06:00 [medline]
AID - 00131402-201412000-00003 [pii]
AID - 10.1097/CND.0000000000000059 [doi]
PST - ppublish
SO  - J Clin Neuromuscul Dis. 2014 Dec;16(2):59-68. doi: 10.1097/CND.0000000000000059.