Imaging Findings in Chiari I Malformation with Syringomyelia in a Case of Charcot Shoulder

Abstract

Neuropathic arthropathy of the shoulder is reported in only 5% of cases. Here, we report a rare case of neuropathic arthropathy of the shoulder, secondary to Chiari malformation Type I with associated syringomyelia, that remained undetected for four years. A 38-year-old female presented to our Department with a swelling over the right shoulder that had persisted for four years. X-ray of the joint showed destruction of the head of the right humerus, with typical blunt amputated appearance of the bone and increased joint space. Magnetic resonance imaging showed destruction and lateral dislocation of the head of the humerus. Large amount of fluid collection was seen in and around the right shoulder joint. Neuropathic osteoarthropathy can be defined as bone and joint changes that occurs secondary to loss of sensation. In our case, neuropathic shoulder joint was secondary to syringomyelia associated with Chiari I malformation.

Charcot Elbow Joint as the Initial Symptom in Chiari Malformation with Syringomyelia

“…According to the X-ray manifestations, CJ is divided into the hardening type, the absorption type and the mixed type. [ 2 ] The X-ray film in our case showed narrow elbow space, dislocation of the joint, bone resorption and ossification in the surrounding soft tissue. Thus, our case belonged to the absorption type, which was consistent with the diagnostic criteria of CJ.…”

Charcot arthropathy of the shoulder associated with typical and atypical findings

“…Neuropathic arthropathy of the shoulder is a relatively rare situation, typically due to syringomyelia (Crowther and Bell, 2007;Atalar et al, 2010;Nacir et al, 2010;Grahovac et al, 2011;Kumar et al, 2011). The joints involved depend on the underlying disease: typically the shoulders and elbows in syringomyelia (Atalar et al, 2010;Nacir et al, 2010;Grahovac et al, 2011;Kumar et al, 2011) hips and knees in tabes dorsalis and the ankles and feet in diabetes mellitus (van der Ven et al, 2009). Clinically, the joint is swollen with effusion, deformed and unstable.…”

Shoulder arthropathy secondary to syringomyelia: systematic review

“…It typically coexists with type 1 Chiari malformations (6). At the same time, SM is reported to be the main cause of the Charcot joint in the upper limbs (3,7,8). This may bring overlapping neurological symptoms to the clinical picture.…”

“…Shoulder neuroarthropathy (NA) is a rarely described, devastating disease that causes progressive degeneration of joint structures, persistent inflammation and severe limitation of shoulder function ( 1 , 2 ). The joints of the upper limb are much less often affected by the process of NA ( 3 ). The shoulder is the most frequent which accounts for only 5% of all Charcot joint locations ( 4 ).…”