Case Report

An Idiopathic Case of Calcium Pyrophosphate Dihydrate Crystal Deposition Disease with Crowned Dens Syndrome in a Young Patient

Authors: Zeliha Unlu MD, Serdar Tarhan MD, Eylem Mete Ozmen MD

Abstract

Abstract:Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is rare in patients under the age of 40 in the absence of metabolic or familial predisposition. A high incidence of involvement of the transverse ligament of the atlas in CPPD deposition disease was reported. However, involvement of the craniocervical junction is rarely symptomatic. We report a rare case in a young male with severe idiopathic CPPD crystal deposition disease, including crowned dens syndrome in the cervical spine.

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References

References1.Rosenthal AK. Update in calcium deposition diseases. Curr Opin Rheumatol 2007;19:158–162.RosenthalAKUpdate in calcium deposition diseases.Curr Opin Rheumatol200719158-1622.Ahn JK, Kim HJ, Kim EH, et al. Idiopathic calcium pyrophosphate dihydrate (CPPD) crystal deposition disease in a young male patient: a case report. J Korean Med Sci 2003;18:917–920.AhnJK]]KimHJ]]KimEH&etal;Idiopathic calcium pyrophosphate dihydrate (CPPD) crystal deposition disease in a young male patient: a case report.J Korean Med Sci200318917-9203.Hayashi M, Matsunaga T, Tanikawa H. Idiopathic widespread calcium pyrophosphate dihydrate crystal deposition disease in a young patient. Skeletal Radiol 2002;31:246–250.HayashiM]]MatsunagaT]]TanikawaHIdiopathic widespread calcium pyrophosphate dihydrate crystal deposition disease in a young patient.Skeletal Radiol200231246-2504.Hammoudeh M, Siam AR. Pseudogout in a young patient. Clin Rheumatol 1998;17:242–245.HammoudehM]]SiamARPseudogout in a young patient.Clin Rheumatol199817242-2455.Lam HY, Cheung KY, Law SW, et al. Crystal arthropathy of the lumbar spine: a report of 4 cases. J Orthop Surg (Hong Kong) 2007;15:94–101.LamHY]]CheungKY]]LawSW&etal;Crystal arthropathy of the lumbar spine: a report of 4 cases.J Orthop Surg (Hong Kong)20071594-1016.Feydy A, Lioté F, Carlier R, et al. Cervical spine and crystal-associated diseases: imaging findings. Eur Radiol 2006;16:459–468.FeydyA]]LiotéF]]CarlierR&etal;Cervical spine and crystal-associated diseases: imaging findings.Eur Radiol200616459-4687.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;28:1417–1420.BouvetJP]]le ParcJM]]MichalskiB&etal;Acute neck pain due to calcifications surrounding the odontoid process: the crowned dens syndrome.Arthritis Rheum1985281417-14208.Dirheimer Y, Wackenheim C, Dietemann JL. Calcification of the transverse ligament in calcium dihydrate deposition disease (CPPD). Neuroradiology 1985;27:87.DirheimerY]]WackenheimC]]DietemannJLCalcification of the transverse ligament in calcium dihydrate deposition disease (CPPD).Neuroradiology198527879.Baysal T, Baysal O, Kutlu R, et al. The crowned dens syndrome: a rare form of calcium pyrophosphate deposition disease. Eur Radiol 2000;10:1003–1005.BaysalT]]BaysalO]]KutluR&etal;The crowned dens syndrome: a rare form of calcium pyrophosphate deposition disease.Eur Radiol2000101003-100510.Sato T, Hagiwara K, Sasaki M, et al. Crowned dens syndrome. Intern Med 2005;44:160.SatoT]]HagiwaraK]]SasakiM&etal;Crowned dens syndrome.Intern Med20054416011.Doherty M, Hamilton E, Henderson J, et al. Familial chondrocalcinosis due to calcium pyrophosphate dihydrate crystal deposition in English families. Br J Rheumatol 1991;30:10–15.DohertyM]]HamiltonE]]HendersonJ&etal;Familial chondrocalcinosis due to calcium pyrophosphate dihydrate crystal deposition in English families.Br J Rheumatol19913010-1512.Cardenal A, Masuda I, Haas AL, et al. Specificity of porcine 127-kd nucleotide pyrophosphohydrolase for articular tissue. Arthritis Rheum 1996;39:245–251.CardenalA]]MasudaI]]HaasAL&etal;Specificity of porcine 127-kd nucleotide pyrophosphohydrolase for articular tissue.Arthritis Rheum199639245-25113.Register TC, Wuthier RE. Effect of pyrophosphate and two diphosphonates on 45Ca and 32Pi uptake and mineralization by matrix vesicle-enriched fractions and by hydroxyapatite. Bone 1985;6:307–312.RegisterTC]]WuthierREEffect of pyrophosphate and two diphosphonates on 45Ca and 32Pi uptake and mineralization by matrix vesicle-enriched fractions and by hydroxyapatite.Bone19856307-31214.Ryan LM, McCarty DJ. Understanding inorganic pyrophosphate metabolism: toward prevention of calcium pyrophosphate dehydrate crystal deposition. Ann Rheum 1995;54:939–941.RyanLM]]McCartyDJUnderstanding inorganic pyrophosphate metabolism: toward prevention of calcium pyrophosphate dehydrate crystal deposition.Ann Rheum199554939-941