The Neuroradiology Journal 27: 495-497, 2014 - doi: 10.15274/NRJ-2014-10056
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Crowned Dens Syndrome A Case Report
ANNA KOYFMAN1, DANIEL YAFFE1,2 1 2
Department of Radiology, Meir Medical Center; Kfar Saba, Israel Sackler Faculty of Medicine, Tel Aviv University; Ramat Aviv, Israel
Key words: acute headache, calcium deposits, alar ligament, crowned dens syndrome
SUMMARY – Crystal deposition in the cervical spine around the odontoid process may lead to acute neck pain. This rare condition is called crowned dens syndrome and should be considered in the differential diagnosis of a possible etiology for fever, headache and cervical pain of unknown origin. The syndrome is often overlooked, thus leading to misdiagnosis, invasive and useless investigations (lumbar puncture, biopsy), inappropriate treatment (steroids, antibiotics, antiviral drugs) and prolonged hospitalization. This can be prevented by imaging, based on a cervical CT scan that allows an accurate diagnosis. The disease has a good prognosis and symptoms usually subside within a few weeks. We describe a patient with crowned dens syndrome which manifested with clinical (acute occipital headache) and radiographic (calcium deposits in the alar ligament) features. Our patient recovered in four days with symptomatic therapy.
Introduction Crystal deposition in the cervical spine around the odontoid process may lead to acute neck pain. This rare condition is called crowned dens syndrome and was first described by Bouvet et al. in 1985 1. It is often overlooked, thus leading to misdiagnosis, invasive and useless investigations (lumbar puncture, biopsy), inappropriate treatment (steroids, antibiotics, antiviral drugs) and prolonged hospitalization. This can be prevented by imaging, based on a cervical CT scan that allows an accurate diagnosis 2. We describe the case of 53-year-old woman with acute onset of severe headache associated with calcified deposits in the alar ligament. The syndrome in this particular ligament was first reported in 2001 by Kobayashi et al. 2. Case Report A 53-year-old woman presented to the emergency department with acute severe occipital headache. The patient was otherwise healthy and denied a history of trauma. She did not
have inflammatory indicators, such as increased body temperature, CRP levels or white blood cell count. Because of severe headache without relief from analgesic drugs, she underwent a lumbar puncture that revealed no abnormality. A computed tomography scan of the head was performed in which serious pathology was excluded. A calcification in the alar ligament was assumed to be the cause of pain (Figure 1). The patient was treated symptomatically with analgesic drugs and intravenous fluids. The symptoms improved after four days and the patient was discharged. Discussion Crystal deposition disease comprises a group of metabolic diseases in which crystals are deposited in and around joints, leading to inflammatory and destructive lesions. It can occur within various soft tissues, such as cartilage, joint capsules, synovium, bursae, tendons, and ligaments 3. The idiopathic form is the most common and the prevalence of the condition increases with age 4. However, there are rare familial forms. Association with metabolic dis495
Crowned Dens Syndrome
Anna Koyfman
Figure 1 Axial CT image of the brain at level C1-2 shows calcium deposits around the right side of the odontoid process in the alar ligament.
eases including hyperparathyroidism, hemochromatosis, ochronosis and hypophosphatasia has also been reported 3. The deposition of crystals in and around joints may be due to local or systemic metabolic disturbances that raise solute concentration, a loss of local inhibitors of crystal growth, or the presence of abnormal surfaces that promote crystal nucleation. The appearance of crystals may induce inflammation, as multiple protein molecules can adsorb onto their surface, leading to formation of a crystal-protein complex. Then the crystal is taken into the neutrophil via endocytosis in an attempt at degradation, but this has an opposite effect, which results in a release of proteolytic enzymes and ultimately cell death 4. Crystal deposition disease specifically involves the cervical spine around the odontoid process, including the synovial membrane, articular capsule, transverse ligament, and transverse cruciate and alar ligaments 4,5. Although such calcification often remains asymptomatic, it may be associated with acute neck pain usually reflecting a nonspecific inflammatory reaction around the crystals. This is called crowned dens syndrome. In the study by Salaffi et al., only nine of the 49 cases of crystal deposit (18.4%), presented with neck symptoms 3. This 496
is a rare condition, and there are only a few case reports on the disease 6. Thus, according to Goto et al., by 2007 only 35 cases had been reported in the English language literature 5. Crowned dens syndrome is a clinical (acute neck pain) and radiographic (calcium deposits around dens) entity 3. The crowned dens deposits can be caused either by calcium pyrophosphate dehydrate or by hydroxyapatite. It is not possible to differentiate between them on the basis of imaging findings alone. The definitive diagnosis is based on histological study of the crystals 4. The clinical findings include acute and severe neck pain and marked restriction of neck motion, particularly in rotation. There could be other manifestations, such as meningism, cervico-brachial pain and occipital and temporal headaches 3. Inflammatory indicators, such as increased body temperature, CRP levels and white blood cell count may be present. Computed tomography is the technique of choice for the detection of crystal deposition adjacent to the odontoid process 6. It is important to exclude inflammatory diseases, tumors and trauma. The disease has a good prognosis and symptoms usually subside within a few weeks 5. According to Taniguchi et al., most patients recover without sequelae with symptomatic therapy 7. Modification or resorption of
www.centauro.it
The Neuroradiology Journal 27: 495-497, 2014 - doi: 10.15274/NRJ-2014-10056
the calcifications is common and may be complete in two weeks 4. However, treatment has been reported to lead to quicker resolution of the symptoms 5. NSAIDs are the gold standard treatment. They provide rapid pain relief within a few days. Low doses of oral colchicine may also be prescribed. In severe diseases unresponsive to NSAIDs, moderate doses of steroids may be used 6. Our patient recovered with symptomatic therapy. Rapid diagnosis of crowned dens syndrome can prevent misdiagnosis, invasive and unnecessary investigations (lumbar puncture, biopsy), inappropriate treatment (steroids, antibiotics, antiviral drugs) and prolonged hospitalization. For example, the triad of headaches, fever and cervical morning stiffness evokes either meningitis leading to lumbar puncture, or cervical metastatic spondylitis in the case of a medi-
cal history of cancer. Moreover, when cervical stiffness is associated with pain in the shoulder girdle and jaw claudication, crowned dens syndrome can mimic polymyalgia rheumatic and/ or giant cell arteritis, resulting in temporal artery biopsy and long-term steroid treatment 3. In addition, misdiagnosis of fracture leads to prolonged external immobilization of the neck. Conclusion We described a patient with crowned dens syndrome that manifested with clinical (acute headache) and radiographic (calcium deposits in the alar ligament) features. This rare condition should be considered in the differential diagnosis of a possible etiology for fever, headache and cervical pain of unknown origin 6.
References 1 Bouvet JP, Le Parc JM, Michalski B, et al. Acute neck pain due to calcifications surrounding the odontoid process: the crowned dens syndrome. Arthritis Rheum. 1985; 28: 1417-20. doi: 10.1002/art.1780281215. 2 Kobayashi Y, Mochida J, Saito I, et al. Calcification of the alar ligament of the cervical spine: imaging findings and clinical course. Skeletal Radiol. 2001; 30: 295297. doi: 10.1007/s002560100325. 3 Salaffi F, Carotti M, Guglielmi G, et al. The crowned dens syndrome as a cause of neck pain: clinical and computed tomography study in patients with calcium pyrophosphate dihydrate deposition disease. Clin Exp Rheumatol. 2008; 26: 1040-1046. 4 Feydy A, Lioté F, Carlier R, et al. Cervical spine and crystal-associated diseases: imaging findings. Eur Radiol. 2006; 16: 459-468. doi: 10.1007/s00330-005-2776-z. 5 Goto S, Umehara J, Aizawa T, et al. Crowned dens syndrome. J Bone Joint Surg Am. 2007; 89: 2732-6. doi: 10.2106/JBJS.F.01322. 6 Takahashi T, Minakata Y, Tamura M, et al. A rare case of crowned dens syndrome mimicking aseptic meningitis. Case Rep Neurol. 2013; 5: 40-46. doi: 10.1159/000348745.
7 Taniguchi A, Ogita K, Murata T, et al. Painful neck on rotation: diagnostic significance for crowned dens syndrome. J Neurol. 2010; 257: 132-135. doi: 10.1007/ s00415-009-5304-2.
Dr. Anna Koyfman Dept. of Radiology Meir Medical Center, 59 Tschernichovsky St. Kfar Saba 42281 Israel Tel.: (972-9)747-15-12 Mobile: (972-52) 423-39-43 Fax: (972-9)747-11-29 E-mail: [email protected]
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www.centauro.it
Crowned Dens Syndrome A Case Report
ANNA KOYFMAN1, DANIEL YAFFE1,2 1 2
Department of Radiology, Meir Medical Center; Kfar Saba, Israel Sackler Faculty of Medicine, Tel Aviv University; Ramat Aviv, Israel
Key words: acute headache, calcium deposits, alar ligament, crowned dens syndrome
SUMMARY – Crystal deposition in the cervical spine around the odontoid process may lead to acute neck pain. This rare condition is called crowned dens syndrome and should be considered in the differential diagnosis of a possible etiology for fever, headache and cervical pain of unknown origin. The syndrome is often overlooked, thus leading to misdiagnosis, invasive and useless investigations (lumbar puncture, biopsy), inappropriate treatment (steroids, antibiotics, antiviral drugs) and prolonged hospitalization. This can be prevented by imaging, based on a cervical CT scan that allows an accurate diagnosis. The disease has a good prognosis and symptoms usually subside within a few weeks. We describe a patient with crowned dens syndrome which manifested with clinical (acute occipital headache) and radiographic (calcium deposits in the alar ligament) features. Our patient recovered in four days with symptomatic therapy.
Introduction Crystal deposition in the cervical spine around the odontoid process may lead to acute neck pain. This rare condition is called crowned dens syndrome and was first described by Bouvet et al. in 1985 1. It is often overlooked, thus leading to misdiagnosis, invasive and useless investigations (lumbar puncture, biopsy), inappropriate treatment (steroids, antibiotics, antiviral drugs) and prolonged hospitalization. This can be prevented by imaging, based on a cervical CT scan that allows an accurate diagnosis 2. We describe the case of 53-year-old woman with acute onset of severe headache associated with calcified deposits in the alar ligament. The syndrome in this particular ligament was first reported in 2001 by Kobayashi et al. 2. Case Report A 53-year-old woman presented to the emergency department with acute severe occipital headache. The patient was otherwise healthy and denied a history of trauma. She did not
have inflammatory indicators, such as increased body temperature, CRP levels or white blood cell count. Because of severe headache without relief from analgesic drugs, she underwent a lumbar puncture that revealed no abnormality. A computed tomography scan of the head was performed in which serious pathology was excluded. A calcification in the alar ligament was assumed to be the cause of pain (Figure 1). The patient was treated symptomatically with analgesic drugs and intravenous fluids. The symptoms improved after four days and the patient was discharged. Discussion Crystal deposition disease comprises a group of metabolic diseases in which crystals are deposited in and around joints, leading to inflammatory and destructive lesions. It can occur within various soft tissues, such as cartilage, joint capsules, synovium, bursae, tendons, and ligaments 3. The idiopathic form is the most common and the prevalence of the condition increases with age 4. However, there are rare familial forms. Association with metabolic dis495
Crowned Dens Syndrome
Anna Koyfman
Figure 1 Axial CT image of the brain at level C1-2 shows calcium deposits around the right side of the odontoid process in the alar ligament.
eases including hyperparathyroidism, hemochromatosis, ochronosis and hypophosphatasia has also been reported 3. The deposition of crystals in and around joints may be due to local or systemic metabolic disturbances that raise solute concentration, a loss of local inhibitors of crystal growth, or the presence of abnormal surfaces that promote crystal nucleation. The appearance of crystals may induce inflammation, as multiple protein molecules can adsorb onto their surface, leading to formation of a crystal-protein complex. Then the crystal is taken into the neutrophil via endocytosis in an attempt at degradation, but this has an opposite effect, which results in a release of proteolytic enzymes and ultimately cell death 4. Crystal deposition disease specifically involves the cervical spine around the odontoid process, including the synovial membrane, articular capsule, transverse ligament, and transverse cruciate and alar ligaments 4,5. Although such calcification often remains asymptomatic, it may be associated with acute neck pain usually reflecting a nonspecific inflammatory reaction around the crystals. This is called crowned dens syndrome. In the study by Salaffi et al., only nine of the 49 cases of crystal deposit (18.4%), presented with neck symptoms 3. This 496
is a rare condition, and there are only a few case reports on the disease 6. Thus, according to Goto et al., by 2007 only 35 cases had been reported in the English language literature 5. Crowned dens syndrome is a clinical (acute neck pain) and radiographic (calcium deposits around dens) entity 3. The crowned dens deposits can be caused either by calcium pyrophosphate dehydrate or by hydroxyapatite. It is not possible to differentiate between them on the basis of imaging findings alone. The definitive diagnosis is based on histological study of the crystals 4. The clinical findings include acute and severe neck pain and marked restriction of neck motion, particularly in rotation. There could be other manifestations, such as meningism, cervico-brachial pain and occipital and temporal headaches 3. Inflammatory indicators, such as increased body temperature, CRP levels and white blood cell count may be present. Computed tomography is the technique of choice for the detection of crystal deposition adjacent to the odontoid process 6. It is important to exclude inflammatory diseases, tumors and trauma. The disease has a good prognosis and symptoms usually subside within a few weeks 5. According to Taniguchi et al., most patients recover without sequelae with symptomatic therapy 7. Modification or resorption of
www.centauro.it
The Neuroradiology Journal 27: 495-497, 2014 - doi: 10.15274/NRJ-2014-10056
the calcifications is common and may be complete in two weeks 4. However, treatment has been reported to lead to quicker resolution of the symptoms 5. NSAIDs are the gold standard treatment. They provide rapid pain relief within a few days. Low doses of oral colchicine may also be prescribed. In severe diseases unresponsive to NSAIDs, moderate doses of steroids may be used 6. Our patient recovered with symptomatic therapy. Rapid diagnosis of crowned dens syndrome can prevent misdiagnosis, invasive and unnecessary investigations (lumbar puncture, biopsy), inappropriate treatment (steroids, antibiotics, antiviral drugs) and prolonged hospitalization. For example, the triad of headaches, fever and cervical morning stiffness evokes either meningitis leading to lumbar puncture, or cervical metastatic spondylitis in the case of a medi-
cal history of cancer. Moreover, when cervical stiffness is associated with pain in the shoulder girdle and jaw claudication, crowned dens syndrome can mimic polymyalgia rheumatic and/ or giant cell arteritis, resulting in temporal artery biopsy and long-term steroid treatment 3. In addition, misdiagnosis of fracture leads to prolonged external immobilization of the neck. Conclusion We described a patient with crowned dens syndrome that manifested with clinical (acute headache) and radiographic (calcium deposits in the alar ligament) features. This rare condition should be considered in the differential diagnosis of a possible etiology for fever, headache and cervical pain of unknown origin 6.
References 1 Bouvet JP, Le Parc JM, Michalski B, et al. Acute neck pain due to calcifications surrounding the odontoid process: the crowned dens syndrome. Arthritis Rheum. 1985; 28: 1417-20. doi: 10.1002/art.1780281215. 2 Kobayashi Y, Mochida J, Saito I, et al. Calcification of the alar ligament of the cervical spine: imaging findings and clinical course. Skeletal Radiol. 2001; 30: 295297. doi: 10.1007/s002560100325. 3 Salaffi F, Carotti M, Guglielmi G, et al. The crowned dens syndrome as a cause of neck pain: clinical and computed tomography study in patients with calcium pyrophosphate dihydrate deposition disease. Clin Exp Rheumatol. 2008; 26: 1040-1046. 4 Feydy A, Lioté F, Carlier R, et al. Cervical spine and crystal-associated diseases: imaging findings. Eur Radiol. 2006; 16: 459-468. doi: 10.1007/s00330-005-2776-z. 5 Goto S, Umehara J, Aizawa T, et al. Crowned dens syndrome. J Bone Joint Surg Am. 2007; 89: 2732-6. doi: 10.2106/JBJS.F.01322. 6 Takahashi T, Minakata Y, Tamura M, et al. A rare case of crowned dens syndrome mimicking aseptic meningitis. Case Rep Neurol. 2013; 5: 40-46. doi: 10.1159/000348745.
7 Taniguchi A, Ogita K, Murata T, et al. Painful neck on rotation: diagnostic significance for crowned dens syndrome. J Neurol. 2010; 257: 132-135. doi: 10.1007/ s00415-009-5304-2.
Dr. Anna Koyfman Dept. of Radiology Meir Medical Center, 59 Tschernichovsky St. Kfar Saba 42281 Israel Tel.: (972-9)747-15-12 Mobile: (972-52) 423-39-43 Fax: (972-9)747-11-29 E-mail: [email protected]
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