Neuropathic osteoarthropathy secondary to syringomielia

Clinical Cases 18.01.2007
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 63 years, female
Authors: Bruno Graça, João Filipe Costa, Andrea Canelas, Cristina Marques, Marta Ferreira
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Details
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AI Report

Clinical History

Patient with personal history of syringomielia, is referred to the clinician with swollen and warmth shoulders. AP radiographs of the left shoulder showed destruction of the humeral head and of the glenoid, with dislocation, marked productive changes and detachment of bone fragments.

Imaging Findings

A 63 year old female patient, tetraparetic, with past history of cervical laminectomy and drainage of syringomielic cavity, presented with a long history of edema, warmth and moderate pain in the shoulders. There where mild painful symptoms in the elbows, wrists and hands. AP radiographs of the shoulders revealed marked destruction of the left glenohumeral joint, with debris, joint distention and dislocation (fig. 1). The right shoulder showed intense osteoarthritis-like alterations, with exuberant osteophytes in the humeral head and bone debris (fig. 2). One can appreciate similar alterations in the left elbow (fig. 3). In the hands, bilateral degenerative alterations in the 3rd, 4th and 5th IFP articulations, sparing the 1st carpometacarpal and the IFD articulations, is amenable with neuropathic arthropathy (fig. 4). The articulations of the lower limb showed no significant alterations (fig. 5). There was paralytic kiphoscoliosis in the dorsal spine.

Discussion

The loss of sensory nervous function on musculoskeletal system results in severe joint damage. The joints, without sensitive feedback, are subjected to recurrent injury, leading to specific radiographic abnormalities wich are termed Neuropathic Osteoarthropathy or Charcot´s joint, since was French scientist Jean-Martin Charcot who first described this disease, in 1868 (1). The site of involvement in neuropathic osteoarthropathy depends of the etiology. In tabes dorsalis, the spine and the major articulations of the lower limb are affected. In syringomyelia the alterations are present in the glenohumeral joint, elbow, wrist and spine. Meningomyelocele involves ankle and intertarsal joints. In amyloidosis the knee and ankle are commonly affected. Diabetes mellitus and alcoholism show typical alterations in metatarsophalangeal, tarsometatarsal and intertarsal joints (2). The classic resulting radiographic picture of Charcot's joints is that of the "5 Ds": normal density, joint distention, bony debris, joint disorganization and dislocation. But almost 40% of neuropathic joint disease are atrophic, with severe bone resorption and little or no debris. The atrophic manifestation is more common in the upper extremities (3). Early manifestations of may simulate osteoarthritis and calcium pyrophosphate dihydrate crystal deposition disease. As the alterations evolve, Charcot's joints tend to exhibit large effusions, which may decompress along soft tissue planes and carry bony debris far from the joint. Bone resorption and formation occurs simultaneously. Bone eburnation, ligamentous laxity and subluxation are also evident. Ankylosis is rare. Neuropathic joint's may suffer infectious complications, particularly in diabetes mellitus (1). Syringomielia is the major cause of neuropathic shoulder. There may be also alterations in the elbow, wrist and fingers joints. Changes in the spine are most characteristic in the cervical region (1,3).

Differential Diagnosis List

Neuropathic osteoarthropathy

Final Diagnosis

Neuropathic osteoarthropathy

Liscense

Figures

AP radiograph of the left shoulder

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AP radiograph of the left shoulder

AP radiograph of the right shoulder

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AP radiograph of the right shoulder

AP and lateral radiographs of the left elbow

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AP and lateral radiographs of the left elbow

AP radiograph of the hands

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AP radiograph of the hands

Articulations of the lower limb

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Articulations of the lower limb