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LETTER TO EDITOR
Year : 2011  |  Volume : 59  |  Issue : 5  |  Page : 771-772

Charcot's shoulder in syringomyelia


1 Department of Clinical Neurophysiology, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Neurosurgery, Sir Ganga Ram Hospital, New Delhi, India
3 Department of Radiology, Sir Ganga Ram Hospital, New Delhi, India

Date of Submission02-Aug-2011
Date of Decision03-Aug-2011
Date of Acceptance11-Aug-2011
Date of Web Publication22-Oct-2011

Correspondence Address:
Samhita Panda
Department of Clinical Neurophysiology, Sir Ganga Ram Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.86563

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How to cite this article:
Panda S, Madan V S, Sud S. Charcot's shoulder in syringomyelia. Neurol India 2011;59:771-2

How to cite this URL:
Panda S, Madan V S, Sud S. Charcot's shoulder in syringomyelia. Neurol India [serial online] 2011 [cited 2019 Nov 13];59:771-2. Available from: http://www.neurologyindia.com/text.asp?2011/59/5/771/86563


Sir,

A 56-year-old male presented with difficulty in lifting left upper limb of 3 years duration. At the onset, there was neck pain radiating to left upper limb associated with numbness and paraesthesias over shoulder. Weakness progressively increased with inability to lift left shoulder over 3 months. He had history of blunt injury to neck and chest, 20 and 4 years back. No other relevant history could be elicited. On examination, he had a limp proximal left upper limb with shoulder droop and left shoulder joint was not palpable. Power at left shoulder was 2/5, elbow flexors 4/5 and wrist 5/5. Deep tendon jerks were absent in left upper limb. Loss of touch and pain sensation was observed over left C2-C5 dermatomes. Lower limb and spine examination was unremarkable. Plain X-ray of left shoulder showed resorption of left humeral head [Figure 1]a. Electrodiagnostic evaluation showed preganglionic involvement of left C5-C7 myotomes. Computed tomography (CT) scan of left shoulder revealed fracture and osteolytic changes of scapula with near-complete resorption of humeral head [Figure 1]b. Magnetic resonance imaging (MRI) cervicodorsal spine demonstrated a small, asymmetrical syrinx in left hemichord with ipsilateral cord atrophy from C2 to T1 segments [Figure 1]c-d. Based on these findings, diagnosis of neuropathic left shoulder joint secondary to syringomyelia was considered. Other causes of rapid joint destruction were excluded. The patient was managed conservatively with mobilization and prevention of joint trauma.
Figure 1: (a) Antero-posterior X-ray of left shoulder shows osteolysis and erosion of head of humerus and parts of scapula and glenoid with pathological fracture. (b) Axial CT scan of the shoulder shows abnormal soft tissue around the shoulder joint with dystrophic calcification. (c) T2W sagittal image of MRI cervical spine reveals cord atrophy and increase in signal within the cord extending from the cervico-medullary junction to T1 disc level. (d) T2W axial image shows left hemiatrophy of the cord with increase in signal within it

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Charcot's joint is a progressive destructive joint disease associated with decreased joint sensation. Various etiologies include: syringomyelia (25%), diabetes mellitus (0.16-2.5%) and tabes dorsalis (5-10%). [1] Shoulders and elbows are commonly involved in syringomyelia, hips and knees in tabes dorsalis, and ankles and feet in diabetes mellitus. [2] Rarely, it is associated with spinal cord and peripheral nerve injury, leprosy, myelomeningocele, amyloidosis, congenital insensitivity to pain and familial dysautonomia. [2] Charcot's shoulder is a rare disorder described in fewer than 60 patients in world literature. Syringomyelia associated Charcot's joint predominantly involves upper limbs (80%), shoulder joint followed by elbow. [3]

Syringomyelia leads to interruption of lateral spinothalamic tract, dorsal column fibers, anterior horn cells and sympathetic pathways sequentially leading to loss of joint sensation. [4] Symptoms related to shoulder frequently precede or overshadow neurological deficits. Onset of symptoms may be acute with neck and shoulder pain. The atrophic form of neuropathic arthropathy usually affects non-weight bearing joints such as shoulder, elbow and wrist with severe destruction rapidly, sometimes in less than 6 weeks. [5] This case highlights neuropathic shoulder as a rare complication of syringomyelia. The cornerstone of therapy is maintenance of function and arthrodesis is contraindicated.

 
  References Top

1.Jones J, Wolf S. Neuropathic shoulder arthropathy (Charcot joint) associated with syringomyelia. Neurology 1998;50:825-7.  Back to cited text no. 1
    
2.Shapiro G, Bostrom M. Heterotopic ossification and Charcot neuroarthropathy. In: Chapman MW, editor. Chapman's Orthopaedic Surgery. Philadelphia: Lippincott Williams and Wilkins; 2001. p. 3245-62.  Back to cited text no. 2
    
3.Hatzis N, Kaar TK, Wirth MA, Toro F, Rockwood CA Jr. Neuropathic arthropathy of the shoulder. J Bone Joint Surg Am 1998;80:1314-9.  Back to cited text no. 3
    
4.Mancall E: Syringomyelia. In: Rowland LP, editor. Merritt's textbook of neurology. Philadelphia: Lea and Febiger; 1984. p. 552-6.  Back to cited text no. 4
    
5.Brower AC, Allman RM. Pathogenesis of the neurotrophic joint: Neurotraumatic vs. neurovascular. Radiology 1981;139:349-54.  Back to cited text no. 5
    


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