Pseudogout of the Transverse Atlantal Ligament: An Unusual Cause of Cervical Myelopathy

Abstract

Calcium pyrophosphate dihydrate (CPPD) deposition disease is an inflammatory arthropathy characterized by the deposition of weakly positively birefringent crystals in articular and periarticular structures. It has also been referred to as pseudogout (owing to its clinical similarity to gout) and articular chondrocalcinosis. T h e CPPD crystals are deposited exclusively in joints and bursae, with occasional extension beyond the anatomical confines of the joint. The cervical spine is uncommonly affected, and symptomatic cervical spine involvement is rarer still. There have been only 12 reported cases of symptomatic CPPD deposition disease involving the C1-2 articulation. 1 -6 We describe a case of an elderly woman who developed a progressive cervical myelopathy from C1-2 instability associated with CPPD deposition disease involving the transverse atlantal ligament. ABSTRACT:Background: Calcium pyrophosphate dihydrate deposition in the cervical spine is infrequently symptomatic. This is especially true at the craniocervical junction and upper cervical spine. Case Report: A 7 0 -y e a r-old previously healthy woman presented with a progressive cervical myelopathy of four months duration. Results: Examination revealed sensorimotor findings consistent with an upper cervical myelopathy. Radiological studies (plain radiographs, computed tomography, and magnetic resonance imaging) revealed C1-2 instability, and a well-defined extradural 3cm x 1cm retroodontoid mass causing spinal cord compression. Transoral resection of the mass was performed followed by posterior C1-2 stabilization. Histological examination of the mass confirmed calcium pyrophosphate dihydrate deposition. Follow-up examination showed marked clinical and radiological improvement. Conclusion: Although uncommon, calcium pyrophosphate dihydrate deposition disease should be considered in the differential diagnosis of extradural mass lesions in the region of the odontoid.RÉSUMÉ: Pseudo-goutte du ligament transverse de l'atlas: une cause rare de myélopathie cervicale. Introduction: Il est rare que des dépôts de pyrophosphate dihydrate de calcium provoquent des symptômes, particulièrement quand ils sont situés à la jonction crânio-cervicale et à la région supérieure du rachis cervical. Observation: Une femme de 70 ans, sans antécédents médicaux, a consulté pour une myélopathie cervicale évoluant depuis 4 mois. Résultats: L'examen physique a montré des anomalies sensitivo-motrices compatibles avec une myélopathie cervicale haute. L'imagerie (radiographie simple, tomodensitométrie et résonance magnétique) ont montré une instabilité C1-C2 et une masse épidurale, rétro-odontoïde, bien définie de 3cm par 1cm comprimant la moelle épinière. La patiente a subi une résection transorale de la masse avec stabilisation postérieure C1-C2. L'examen histologique de la masse a confirmé qu'il s'agissait d'un dépôt de pyrophosphate dihydrate de calcium. L'état clinique de la patiente, ainsi que l'imagerie, se sont améliorés de façon importante. Conclusion: Bien que rare, le dé...

Calcium pyrophosphate dihydrate crystal deposition in the craniovertebral junction

“…The diagnosis of fibrocartilage alone at the C1-2 junction is normally not anatomically feasible, given the lack of an intervertebral disc, and as such should raise suspicion of CPPD deposition. These findings were shared by Greisdale and colleagues 10 who observed CPPD crystals deposited in a fibrocartilaginous matrix on histological examination of the surgical mass, and by Ishida et al, 12 who observed chondroid metaplasia in and around the areas of CPPD deposition in 15 patients that could be misdiagnosed as chondrosarcoma or another incorrect pathological entity. Doherty and Dieppe 6 postulated that a transformation of fibroblasts into chondrocytes occurs in the ligamentous tissues followed by production of inorganic pyrophosphate leading to the deposition of CPPD crystal in the collagen or degenerated fibrils.…”

“…Other authors 1,3,10 also choose the transoral-transpharyngeal surgical approach, but without posterior stabilization. One of our patients underwent dorsal occipital-cervical fusion months after his ventral decompression due to return of motor weakness, ataxia, and pain; another patient underwent fusion over 1 year later due to the return of pain with neck flexion.…”

Spinal Epidural Hematoma Caused by Pseudogout: A Case Report and Literature Review

“…There are reports of pseudogout causing myelopathy, mainly in the cervical cord,1 2 3 and invasion of pyrophosphate crystals into the ligamentum flavum has also been reported in the cervical spine in the setting of pseudogout,4 5 but there was no vascular involvement in these cases. Kawano et al performed electron microscopy of operative specimens and suggested that pyrophosphate crystals are preferentially deposited in the ligamentum flavum and areas of degenerate disk, as these are relatively mobile structures within the vertebral column 4.…”

Transdural approach to resection of retro-odontoid cysts in elderly patients: report of 3 cases

“…21,32 Any anterior approach that entails a transnasal or transoral/transpharyngeal approach with resection of the odontoid process to access the lesion in elderly patients has been noted to result in a high rate of morbidity, including infection, spinal fluid leak, longer operative time, and frequent need for posterior instrumented stabilization. 2,11,26 Based on a review of the literature, the incidence of postoperative sepsis, cervical infection, or respiratory or cardiac complications was reported in up to 8.3% of patients. 8,23 The literature discussed here pertains predominantly to patients with rheumatoid arthritis.…”