CPPD crystal deposition disease of the cervical spine: A common cause of acute neck pain encountered in the neurology department

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Abstract

Background

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is one of the most common forms of crystal-associated arthropathy in the elderly. However, CPPD deposition on the cervical spine is less well known, and only a limited number of cases have been reported to date. Here, we report our recent clinical experience with CPPD crystal deposition disease of the cervical spine and describe the clinical features of this disease.

Methods

Fourteen patients with clinically diagnosed CPPD crystal deposition disease of the cervical spine at our department during the period from January 2005 to December 2008 were analyzed retrospectively.

Results

Patients ranged in age from 54 to 92 (mean ± SD, 77.5 ± 8.5). Chief symptoms of patients were acute posterior neck pain and fever. All patients had markedly restricted neck rotation. Serum CRP level was highly elevated in all patients (10.16 ± 5.35 mg/dL). Computed tomography of the cervical spine demonstrated linear calcific deposits in the transverse ligament of atlas (crowned dens syndrome) in all patients. Calcific deposits were also found in other periodontoid structures and the ligamenta flava in some patients. Posterior neck pain, fever, and increased serum inflammatory indicators were relieved within 1 to 3 weeks by nonsteroidal antiinflammatory drugs (NSAIDs) or a combination of NSAIDs and prednisolone. Most of the patients were misdiagnosed as having other diseases before consultation.

Conclusions

CPPD crystal deposition disease of the cervical spine is one of the most common underrecognized causes of acute neck pain in the neurology department, especially in elderly patients.

Introduction

The crystal-associated arthropathies form a group of metabolic diseases in which crystals, such as calcium pyrophosphate dihydrate (CPPD), basic calcium phosphate (e.g., hydroxyapatite and octacalcium phosphate), and monosodium urate, are deposited in and around joints, leading to inflammatory and destructive lesions. Pseudogout is one of the most common forms of crystal-associated arthritis associated with CPPD deposition in the joints and periarticular tissues [1], [2], [3]. The prevalence of CPPD deposition is extremely high and it is reported that knee chondrocalcinosis, which is assumed to be due to CPPD deposition, is found in 9.6% of individuals older than 50 years old, and this incidence increases with age [4], [5]. Attacks of pseudogout can be either monoarticular or oligoarticular, and the most commonly affected joints are the knees, followed by the shoulders, wrists, and ankles [2]. CPPD crystal deposition also induces arthritis mimicking rheumatoid arthritis (pseudorheumatoid arthritis) or osteoarthritis (pseudoosteoarthritis). However, CPPD crystal deposition on the cervical spine is less well known and only a limited number of cases of this condition have been reported to date [6], [7], [8], [9], [10], [11], [12], [13], [14].

Here, we report our recent clinical experience with 14 patients with CPPD crystal deposition disease of the cervical spine manifested by acute severe posterior neck pain. Acute neck pain is a common complaint among patients in the neurology department; however, it is sometimes difficult to make a precise diagnosis. Although a small number of patients have previously been reported, our recent clinical experience with 14 patients suggests that CPPD crystal deposition disease of the cervical spine is one of the most common underrecognized causes of acute neck pain.

Section snippets

Patients and methods

Data from 14 patients with clinically and radiologically diagnosed CPPD crystal deposition disease of the cervical spine at our department during the period from January 2005 to December 2008 were analyzed retrospectively. The following data were collected from the medical records of the patients: gender; age; clinical course; inflammatory indicators, such as white blood-cell count (WBC), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; radiological findings; and outcome.

Clinical findings

The data for the 14 patients are summarized in Table 1. The male-to-female ratio was 1.0 (7:7), and patients ranged in age from 54 to 92 with a mean ± SD of 77.5 ± 8.5. Thirteen patients (93%) were older than 70 years of age. None of the patients was previously diagnosed as having pseudogout or other crystal-associated arthropathies, although two patients had histories of severe neck pain attacks. The patients' chief symptoms were acute severe posterior neck pain (100%) and fever (79%). Nine (64%)

Discussion

Disease caused by CPPD crystal deposition was first described by McCarty et al. in 1962 [1]. CPPD deposition disease is a pathological process of crystal formation classically within articular, hyaline, and fibrocartilaginous structures, and can also be found in periarticular structures such as the joint capsule, tendons, and ligaments. This pathological process is especially common in the elderly [4], [5]; however, there have been relatively few reports regarding CPPD deposition on the

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