British Journal of Neurosurgery, December 2014; 28(6): 811–812 © 2014 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2014.944482
SHORT REPORT
Charcot arthropathy of the elbow joint as a presenting feature of Chiari malformation with syringomyelia Sushanta K. Sahoo & Pravin Salunke Neurosurgery, PGIMER, Chandigarh, India
sensory loss to pain and temp in left C4–D4 dermatomes. The left elbow was non-tender and its movements were not restricted. Radiology showed Chiari malformation with cervico-dorsal syrinx. X-ray of the left elbow showed destruction, heterotopic calcification and some debris in the joint (Fig. 1). She underwent foramen magnum decompression and duraplasty. Six months later all her symptoms improved except for the elbow swelling. One year later this particular symptom persisted though static. MRI at this time showed adequate foramen magnum decompression and reduction of the size of syrinx (Fig. 1).
Abstract Charcot arthropathy of the elbow joint is occasionally seen with Chiari malformation with syringomyelia, but rarely as a presenting feature as in the reported case. The treatment is directed toward its underlying cause to halt its progression. Thus, it is important to diagnose the cause as early as possible. Keywords: Charcot elbow; Chiari malformation; syringomyelia
Introduction Charcot joint is an uncommon progressive degenerative arthropathy associated with underlying neurological disorders like diabetes, leprosy, syphilis, or traumatic denervation of a limb. Charcot joint is also seen in approximately 5% of cases of syringomyelia.1 Elbow joint is the commonest joint to be involved secondary to syringomyelia.2 However, Charcot arthropathy of elbow joint as the presenting symptom of syringomyelia is extremely rare. Furthermore, the treatment of such joints at present is directed toward treatment of syringomyelia that only halts the progression. This makes early detection of such pathology essential. We report a case of Chiari malformation with syringomyelia, presenting mainly with Charcot elbow.
Discussion Neuropathic arthropathy (Charcot joint) caused by syringomyelia is seen.1 Syrinx may be associated with Chiari malformation and tethered cord. Elbow is the most common joint affected followed by shoulder and wrist.2 The pathophysiology of this is not clearly known. The side of syrinx appears to determine the side of the neuropathic arthropathy.2 It is possible that the pain carrying fibers which, prevents movements of joint beyond physiological limits, are destroyed by syrinx at the cord. This leads to excessive stress and strain at the joints giving rise to fractures and healing process. The treatment of such arthropathy is directed toward the underlying neurological disorder. High index of suspicion is necessary so that underlying cause of Charcot elbow is detected and treated early. This halts the progression of arthropathy though it does not regress. Surgical intervention of the joints does not seem to have a good outcome and is indicated only in gross instability and nerve compression syndromes.3 In conclusion, neuropathic arthropathy may be a presenting feature of syringomyelia. Early detection and treatment
Case report A 25-year-old non-diabetic female presented with complains of insidious onset of painless progressive swelling in the left elbow for 2 years. The diagnosis could not be made until she started complaining of sub-occipital pain with burning and dysesthetic pain of left upper half of trunk and left upper limb for 1 year. Examination revealed, subtle spasticity in lower limbs with lower motor features in the upper limbs (hyporeflexia). She had suspended
Correspondence: Dr Pravin Salunke, Assist Professor, PGIMER, Sector 12, Chandigarh 160012, India. Tel: ⫹ 919915504994. Fax: ⫹ 0172-2744401. E-mail: [email protected] Received for publication 11 April 2014; accepted 6 July 2014
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S. K. Sahoo & P. Salunke
Fig. 1. Upper left quadrant shows the swollen left elbow as compared to the right. The X-ray shows destruction, heterotopic calcification and some debris in the joint. The lower left quadrant shows preoperative MRI showing tonsillar herniation with CSF block at foramen magnum (arrow) and cervico-dorsal syrinx (arrow). The right lower quadrant shows MRI an year after foramen magnum decompression showing good flow across foramen magnum (arrow) and decrease in size of syrinx (arrow).
directed toward the underlying cause of neuropathic joint is important to halt the progression. Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.
References 1. Schlesinger EB, Antunes JL, Michelsen WJ, Louis KM. Hydromyelia: clinical presentation and comparison of modalities of treatment. Neurosurgery 1981;9:356–65. 2. Deng X, Wu L, Yang C, Xu Y. Neuropathic arthropathy caused by syringomyelia. J Neurosurg Spine 2013;18:303–9. 3. Kwon YW, Morrey BF. Neuropathic elbow arthropathy: a review of six cases. J Shoulder Elbow Surg 2006;15:378–82.
Copyright of British Journal of Neurosurgery is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
SHORT REPORT
Charcot arthropathy of the elbow joint as a presenting feature of Chiari malformation with syringomyelia Sushanta K. Sahoo & Pravin Salunke Neurosurgery, PGIMER, Chandigarh, India
sensory loss to pain and temp in left C4–D4 dermatomes. The left elbow was non-tender and its movements were not restricted. Radiology showed Chiari malformation with cervico-dorsal syrinx. X-ray of the left elbow showed destruction, heterotopic calcification and some debris in the joint (Fig. 1). She underwent foramen magnum decompression and duraplasty. Six months later all her symptoms improved except for the elbow swelling. One year later this particular symptom persisted though static. MRI at this time showed adequate foramen magnum decompression and reduction of the size of syrinx (Fig. 1).
Abstract Charcot arthropathy of the elbow joint is occasionally seen with Chiari malformation with syringomyelia, but rarely as a presenting feature as in the reported case. The treatment is directed toward its underlying cause to halt its progression. Thus, it is important to diagnose the cause as early as possible. Keywords: Charcot elbow; Chiari malformation; syringomyelia
Introduction Charcot joint is an uncommon progressive degenerative arthropathy associated with underlying neurological disorders like diabetes, leprosy, syphilis, or traumatic denervation of a limb. Charcot joint is also seen in approximately 5% of cases of syringomyelia.1 Elbow joint is the commonest joint to be involved secondary to syringomyelia.2 However, Charcot arthropathy of elbow joint as the presenting symptom of syringomyelia is extremely rare. Furthermore, the treatment of such joints at present is directed toward treatment of syringomyelia that only halts the progression. This makes early detection of such pathology essential. We report a case of Chiari malformation with syringomyelia, presenting mainly with Charcot elbow.
Discussion Neuropathic arthropathy (Charcot joint) caused by syringomyelia is seen.1 Syrinx may be associated with Chiari malformation and tethered cord. Elbow is the most common joint affected followed by shoulder and wrist.2 The pathophysiology of this is not clearly known. The side of syrinx appears to determine the side of the neuropathic arthropathy.2 It is possible that the pain carrying fibers which, prevents movements of joint beyond physiological limits, are destroyed by syrinx at the cord. This leads to excessive stress and strain at the joints giving rise to fractures and healing process. The treatment of such arthropathy is directed toward the underlying neurological disorder. High index of suspicion is necessary so that underlying cause of Charcot elbow is detected and treated early. This halts the progression of arthropathy though it does not regress. Surgical intervention of the joints does not seem to have a good outcome and is indicated only in gross instability and nerve compression syndromes.3 In conclusion, neuropathic arthropathy may be a presenting feature of syringomyelia. Early detection and treatment
Case report A 25-year-old non-diabetic female presented with complains of insidious onset of painless progressive swelling in the left elbow for 2 years. The diagnosis could not be made until she started complaining of sub-occipital pain with burning and dysesthetic pain of left upper half of trunk and left upper limb for 1 year. Examination revealed, subtle spasticity in lower limbs with lower motor features in the upper limbs (hyporeflexia). She had suspended
Correspondence: Dr Pravin Salunke, Assist Professor, PGIMER, Sector 12, Chandigarh 160012, India. Tel: ⫹ 919915504994. Fax: ⫹ 0172-2744401. E-mail: [email protected] Received for publication 11 April 2014; accepted 6 July 2014
811
812
S. K. Sahoo & P. Salunke
Fig. 1. Upper left quadrant shows the swollen left elbow as compared to the right. The X-ray shows destruction, heterotopic calcification and some debris in the joint. The lower left quadrant shows preoperative MRI showing tonsillar herniation with CSF block at foramen magnum (arrow) and cervico-dorsal syrinx (arrow). The right lower quadrant shows MRI an year after foramen magnum decompression showing good flow across foramen magnum (arrow) and decrease in size of syrinx (arrow).
directed toward the underlying cause of neuropathic joint is important to halt the progression. Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.
References 1. Schlesinger EB, Antunes JL, Michelsen WJ, Louis KM. Hydromyelia: clinical presentation and comparison of modalities of treatment. Neurosurgery 1981;9:356–65. 2. Deng X, Wu L, Yang C, Xu Y. Neuropathic arthropathy caused by syringomyelia. J Neurosurg Spine 2013;18:303–9. 3. Kwon YW, Morrey BF. Neuropathic elbow arthropathy: a review of six cases. J Shoulder Elbow Surg 2006;15:378–82.
Copyright of British Journal of Neurosurgery is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
