case report

Charcot shoulder caused by Chiari type I malformation Springer-Verlag 1516/2011 wkw512

Wien Klin Wochenschr (2011) 123: 512514DOI 10.1007/s00508-011-0010-3 Springer-Verlag 2011Printed in Austria

Wiener klinische WochenschriftThe Central European Journal of Medicine

Charcot shoulder caused by Chiari type I malformation with syringomyelia with six-year follow-upGordan Grahovac1, Milorad Vilendecic

1, Dubravka Srdoc2

1Department of Neurosurgery, University Hospital Dubrava, Zagreb, Croatia2Department of Radiology, University Hospital Dubrava, Zagreb, Croatia

Received January 20, 2011, accepted after revision May, 13, 2011, published online July 12, 2011

Durch Chiari Typ 1 Malformation ausgelste Charcot Schulter mit Syringomyelie: 6 Jahre Follow-up.

Zusammenfassung. Wir berichten radiologische und klinische Details einer Patientin mit einer durch Syringo-myelie und Chiari Typ 1 Malformation ausgelsten neuropathischen Gelenkserkrankung der Schulter. Neuro-pathische Gelenke sind 1868 erstmals von Charcot be-schrieben worden. In der Folge wurden sie bei vielen Arten von peripheren und zentralen Nervenerkrankungen beob-achtet. Es gibt viele Grnde fr eine Syringomyelie einer davon ist die Chiari Typ 1 Malformation. Schulter und Ell-bogen sind am hufi gsten von der Syringomyelie-induzier-ten Neuropathie befallen. Unsere Patientin wurde 6 Jahre seit der Diagnosestellung einer neuropathischen Arthropa-thie verbunden mit einer durch Chiari Typ 1 Malformation induzierten Syringomyelie kontrolliert. Nach der Opera-tion zeigte sie keinerlei Progression der Erkrankung.

Summary. We are presenting a case report of neuropathic arthropathy of the shoulder secondary to a syringomyelia and Chiari type I malformation, with detailed clinical and radiological fi ndings. Neuropathic joints were fi rst de-scribed in 1868 by Charcot, and subsequently were de-scribed in many types of peripheral diseases and central neuron diseases. Th ere are many causes of syringomyelia, and one of the causes of this disease is a Chiari type I mal-formation. Subsequently, shoulders and elbows are most commonly aff ected by syringomyelia-induced neuropa-thy. Our patient was observed for six years after being di-agnosed with neuropathic arthropathy and syringomyelia caused by Chiari and did not show any disease progres-sion after their surgery.

Key words: Arnold-Chiari malformation, type 1, arthrop-athy, neurogenic, syringomyelia, shoulder.

Introduction

Neuropathic joint disease, also called Charcot neuroar-thropathy, is a progressive and chronic degenerative dis-ease which causes loss of sensation and pain or destruction of the aff ected joint. Patients with neuropathy are particu-larly prone to developing neuropathic arthropathy (NA). Th e joints most frequently aff ected by syringomyelia are shoulders and elbows, weight bearing joints such as knees and hips in tabes dorsalis, and ankle and foot in diabetes mellitus [1]. We will present a case of neuropathic arthrop-athy of the shoulder with secondary to syringomyelia and Chiari type I malformation and a six-year follow-up with the patient in question.

Case report

A 62-year-old woman was admitted to our department six years ago because of a swelling of the left shoulder that had started six months earlier. Th e patient stated that during the last ten years she had experienced numbness and a sensation of decreased temperature in her left arm, and she was burnt on several occasions. Various specialists in-cluding neurologists and orthopedic surgeons had exam-ined her. Six months before we admitted this patient, she had noticed swelling and redness of the skin of the left shoulder. During our neurological examination, there was a loss of superfi cial feeling, pain and increased tempera-ture in the C3-Th 1 dermatomes of the left arm. Deep ten-don refl exes were normal on the upper and lower extremities. Other neurological examinations were within normal ranges. During the examination, her left shoulder was swollen with limited abduction, and with crepitation in all directions of shoulder movement. Old burn scars were evident on the left forearm.

An anteroposterior plain radiograph of the left shoulder revealed the absence of the humeral head, bone fragmen-tation and subluxation of the articular surfaces (Fig. 1). A magnetic resonance (MR) of the cervical and thoracic spine revealed a Chiari type I malformation and syringo-myelia from C1 to Th 5 level (Fig. 2). An MR of the left shoul-

Correspondence: Gordan Grahovac, MD, Department of Neurosurgery, University Hospital Dubrava, 10000 Zagreb, Croatia, E-mail: ggrahov@mef.hr

case report

Charcot shoulder caused by Chiari type I malformationwkw 1516/2011 Springer-Verlag 513

der showed destruction and dislocation of the humeral head and atrophy of the muscles in the left shoulder (Fig. 1).

Th e patient underwent surgery; a sub-occipital cran-iotomy was performed with a laminectomy of the CI to CIII vertebrae and reconstruction of dura mater with her peri-cranium graft. Our patient recovered successfully after surgery, and we recommended arthrodesis for her left shoulder. She did not show any progression of syringomy-elia in follow-up MRs within the last fi ve years, nor had other joints of the upper extremities shown any sign of neuropathic arthropathy.

Discussion

Lesions of the upper and lower motor neurons can lead to arthropathy. Diseases like multiple sclerosis, syringomy-

elia, tabes dorsalis, and leprosy can cause central sensory lesions, and diseases such as diabetes mellitus, alcohol-ism, infections, pernicious anemia, and amyloidosis can cause peripheral sensory lesions. All these diseases can cause neuropathic arthropathy by causing sensory impair-ment [1, 2]. Neuropathic arthropathy commonly aff ects weight-bearing joints such as the ankle, knee, hip, and in patients with Chiari type 1 and syringomyelia, the shoul-der and elbow are the most commonly aff ected joints [13].

Syringomyelia is a chronic and progressive disease of the spinal cord, characterized by longitudinal cavitations of the spinal cord containing cerebrospinal fl uid which in-volves the cervical and cervicothoracic regions [3]. It de-velops in approximately 7585% of patients with a Chiari type I malformation [4]. Th e clinical presentation of syrin-gomyelia depends on the extent and localization of the

A B

Fig. 1. (A) Anterior-posterior radiograph of the left shoulder. Plain X-ray of the left shoulder is showing destruction of the left humeral head with fragmentation. (B) A coronal T2 weighted MR image of the left shoulder showing erosion, destruction and dislocation of the humeral head

A B

Fig. 2. Sagittal, T2-weighted images (A) of cervical and (B) of cervico-thoracic spine presenting syrinx from C1 to Th5 level with Arnold-Chiari type I malformation. Ethics statement: The patient gave oral consent for publication of his data in medial literature. The patient was fully informed that his medical data would be published only in scientifi c medical journal

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Charcot shoulder caused by Chiari type I malformation Springer-Verlag 1516/2011 wkw514

syrinx. Th e syrinx cavity disrupts the adjacent gray and white matter, which consequently causes destruction of pain and temperature sensory fi bers because they cross the midline. Th is is the reason that loss of pain and tem-perature sensation in upper extremities is the fi rst sign of disease in such patients [5]. Th e loss of pain and tempera-ture sensation in her left arm was also the fi rst sign of dis-ease in our patient.

Th ere are two theories that try to explain the pathogen-esis of neuropathic arthropathy in extremities. According to the French theory, the destruction of joints is due to damage of the central nervous system that controls joint and bone nutrition, and according to the German theory, the destruction of joints is due to the accumulation of sub-clinical joint trauma that is unnoticed because of a lack of sensation in such joints [6]. Bower and Allmand showed that even a bed-ridden patient can develop neuropathic arthropathy which excludes a mechanical basis of this dis-ease alone. Now it is believed that both theories play a role in the pathophysiology of this rare disease. It is presumed that a neurovascular mechanism has an initial role in the pathogenesis of neuropathic arthropathy, and the neu-rotraumatic theory has a compounding eff ect on the dis-eases progression [6].

Th e radiological appearance of a diseased joint in the advanced stages of disease is characterized by debris, de-struction, dislocation, sclerosis, and disorganization of the joint [7]. Radiographic fi ndings of hypertrophy or atrophy of the joint are believed to be two natural stages of this dis-ease [4, 8]. Our patient experienced destruction and dislo-cation of the shoulder, but other joints in the left upper extremities were unaff ected.

In most cases, the treatment is conservative. It is neces-sary to treat the underlying disease and decrease the progression of joint deformity [9]. Immobilizing the joint is indispensable in the treatment of NA, combined with splinting and joint aspiration for reducing the joints ligamentous laxity [9]. Nonsteroidal anti-infl ammatory (NSAID) drugs may control the infl ammation of the swol-len synovium [1, 10]. Our patient also was treated with NSAID drugs, but refused immobilization or splinting of the shoulder.

More aggressive modalities of treatment such as arthr-odesis have been tried, but the results were confounding. Arthroplasty is strictly contraindicated in such patients be-cause of bone weakness and because the disease has a po-tential to be progressive [2, 7].

Neuropathic arthropathy due to syringomyelia caused by Chiari type I has complex fi ndings, and slow disease progression usually is diagnosed in late stages such as in our case.

Our case is interesting because patient did not show disease progression six years after surgery in other joints of the left upper extremities and right upper extremities. In a case of NA, it is necessary to make image examinations of the spinal cord due to lesions of cervical spinal cord which

can cause NA. Th ere are several cases in current literature which describe NA of the shoulder and other joints of the upper extremities combined with Chiari type I and syrin-gomyelia, but this remains a rare disease which in many cases is still diagnosed late, but properly treated [2, 5, 7, 10].

Conclusion

Nevertheless, neuropathic arthropathy is a slow progress-ing disease, and it still remains undetected until it reaches late phases of progression. It is necessary to keep in mind that many diseases can cause neuropathic arthropathy, and that prompt diagnosis and treatment of the primary disease can prevent neuropathic arthropathy. Neuropathic arthropathy should be considered in cases where extreme destruction of the aff ected joint is detected without com-plaints of pain from the patient, and neurological underly-ing problem should be investigated to avoid disease progression.

Disclaimer

None.

Confl ict of interestNone of authors, their immediate family, and any research foundation with which they are affi liated did not receive any fi nancial payments or other benefi ts from any com-mercial entity related to the subject of this article.

References

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5. Nacir B, Arslan Cebeci S, Cetinkaya E, Karagoz A, Erdem HR. Neuropathic arthropathy progressing with multiple joint involvement in the upper extremity due to syringomy-elia and type I Arnold-Chiari malformation. Rheumatol Int 2010;30(7):97983.

6. Brower AC, Allman RM. Pathogenesis of the neurotrophic joint: neurotraumatic vs. neurovascular. Radiology 1981;139(2):34954.

7. Jones J, Wolf S. Neuropathic shoulder arthropathy (Char-cot joint) associated with syringomyelia. Neurology 1998;50(3):8257.

8. Deirmengian CA, Lee SG, Jupiter JB. Neuropathic arthropa-thy of the elbow. A report of fi ve cases. J Bone Joint Surg Am 2001;83-A(6):83944.

9. Ruette P, Stuyck J, Debeer P. Neuropathic arthropathy of the shoulder and elbow associated with syringomyelia: a report of 3 cases. Acta Orthop Belg 2007;73(4):5259.

10. Sequeira W. Th e neuropathic joint. Clin Exp Rheumatol 1994;12(3):32537.

Charcot shoulder caused by Chiari type I malformation with syringomyelia with six-year follow-upDurch Chiari Typ 1 Malformation ausgelste Charcot Schulter mit Syringomyelie: 6 Jahre Follow-up.IntroductionCase reportDiscussionConclusionDisclaimerConflict of interestReferences