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Abstract
An 87-year-old Japanese man presented to our hospital with a 5-day history of fever and neck pain. On physical examination, his stiff neck indicated restricted movement, especially on rotation. CT of the head revealed calcification of the atlantoaxial joint consistent with crowned dens syndrome, and celecoxib was started. Four days later, he returned to our emergency department as his neck pain and fever had not improved. Pneumonia and a urinary tract infection were suspected. The day following admission, blood culture results were positive for methicillin-resistant Staphylococcus aureus. A contrast-enhanced CT revealed an upper cervical epidural abscess at the level of C1–C2. He was discharged following 8 weeks of antibiotic treatment.
- infectious diseases
- medical management
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Background
Differential diagnoses for neck pain range from self-limiting to life-threatening diseases. Crowned dens syndrome (CDS) is characterised through benign yet severe neck pain due to calcium pyrophosphate deposition (also known as pseudo-gout) of the atlantoaxial joint, especially in elderly people.1 However, an upper cervical epidural abscess (UCEA) is a potentially life-threatening infectious disease, requiring prompt diagnosis and appropriate treatment.2 Here, we describe a patient with a delayed UCEA diagnosis, which was masked due to CDS.
Case presentation
An 87-year-old Japanese man presented to our hospital with a 5-day history of fever and neck pain. He reported no history of recent neck trauma or other symptoms, and he had been taken acetaminophen for his fever but no antibiotics. He had no remarkable medical history except for prostate cancer treating by leuprorelin, acute cholangitis and lumbar spinal stenosis. On physical examination, his stiff neck indicated restricted movement, especially on rotation, whereas no other abnormalities or relevant neurological signs were observed. A blood test showed a leucocyte count of 6.4×109/L (normal: 3.9–9.8×109/L), neutrophil percentage of 85.1% (normal: 32%–75%) and a C-reactive protein level of 6.53 mg/dL (normal: <0.3 mg/dL). CT of the head revealed calcification of the atlantoaxial joint consistent with CDS (figure 1), and celecoxib was started.
An axial head CT depicting calcification (arrow) of the transverse ligament.
Four days later, he returned to our emergency department as his neck pain and fever had not improved. On admission, his leucocyte count, neutrophil percentage and C-reactive protein level had markedly increased to 16.3×109/L, 95.3%, and 27.73 mg/dL, respectively. Following a whole-trunk CT, pneumonia and a urinary tract infection were suspected. He was admitted to hospital and intravenous ceftriaxone was administered. The day following admission, blood culture results were positive for methicillin-resistant Staphylococcus aureus (MRSA); therefore, ceftriaxone was switched to intravenous vancomycin. His C-reactive protein levels improved; however, his neck pain and MRSA-related bacteremia persisted, and he experienced bladder and bowel dysfunction. He also had a weakness of extremities with a normal deep tendon reflex, but it was difficult to distinguish whether the weakness came from the compression of the spinal cord or from his frailty. A contrast-enhanced CT revealed a UCEA at the level of C1–C2 (figure 2) and a plain MRI of the head also showed the abscess compressing the spinal canal (figure 3). Oral and nasal drainage was performed to drain the abscess; however, it was unsuccessful and no pus was aspirated. Surgical drainage was considered challenging given the location, and his family declined a surgical approach.
Sagittal CT with contrast showing an epidural abscess (arrow) at the level of C1–C2.
Sagittal plain fat-suppressed T2-weighted MRI showing an epidural abscess compressing the spinal canal and edematous changes in the bone marrow.
Outcome and follow-up
Intravenous vancomycin was administered for 4 weeks until blood cultures showed negative results, and a follow-up CT and MRI confirmed improvement of the epidural abscess. Vancomycin was then switched to a combination of oral sulfamethoxazole and trimethoprim. Our patient was discharged without quadriplegia following 8 weeks of antibiotic treatment and aggressive rehabilitation due to his frailty.
Discussion
Most patients with CDS complain of posterior neck pain and limited neck movement, especially rotation, as observed in our patient, and these symptoms usually resolve within 1–3 weeks after treatment with non-steroidal anti-inflammatory medication.1 One observational study reported that 19% of elderly people (aged from 80 to 90 years) who had undergone a head CT scan were found to have calcification of the atlantoaxial joint.3 In the elderly population, CDS should not be too readily diagnosed even if calcification consistent with CDS is observed, and other differential diagnoses should be considered if treatment does not improve patient symptoms.
A UCEA is a rare surgical emergency, with S. aureus reported to be the leading causative pathogen.2 The most common source of infection is via a haematogenous route; however, approximately 20% of patients with a UCEA have no identifiable source.2 In this patient, the tendency of posterior spread was a key finding to distinguish a UCEA from a retropharyngeal abscess, as the presence of prevertebral fascia strongly prevents the posterior spread of a retropharyngeal abscess.4 UCEA treatment often requires surgical drainage and prolonged antibiotics in contrast to the treatment of CDS; therefore, clinicians need to diagnose a UCEA promptly and appropriately.2
This case study prompts consideration of a differential diagnosis in elderly patients with neck pain and fever.
Learning points
Crowned dens syndrome (CDS) is characterised as calcification of the atlantoaxial joint; however, in elderly people, CDS should not be too readily diagnosed.
An upper cervical epidural abscess (UCEA) is a rare surgical emergency, and clinicians need to be aware that CDS can mask a UCEA.
It is important to consider further examination if treatment does not improve patient symptoms.
Footnotes
Contributors HS drafted the manuscript. TH, SN, and KS revised the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Next of kin consent obtained.
Provenance and peer review Not commissioned; externally peer reviewed.