A Case Of Crowned Dens Syndrome

2015-09-20 06:04:58

Category: Miscellaneous, Region: Head-Bones, Plane: Coronal

CROWNED DENS SYNDROME- A RARE CASE REPORT. DR. V.L .RATNAKUMARI, DR. DIVYA.S.NAIR A 45 year old lady presented with acute onset of severe neck pain and vomiting of 2 days duration. She had history of neck pain of one year duration associated with restricted neck movements. The pain aggravated at night and with neck extension. Past history of hypothyroidism , on medical treatment. Clinical examination-- Pulse rate- 70 per minute. Blood pressure- 120/ 80 mm Hg. Chest – clear, heart sound- S1 S2 – normal. Neurological examination- Fundus- normal, cranial nerves- normal, Power- normal, Deep tendon reflexes- normal, plantar- bilateral equivocal, no neck stiffness. Blood routine and other lab investigations- Hb- 11.4 mg%, TC-6500, DC- N 57,L 35, E 3 ESR- 15mm/hr, FBS- 108 mg%,serum creatinine-0.8 mg%, S.Na/K- 145/3.7meq/l , SGOT/PT- 15/27 IU TSH- 3.78, S. bilirubin-0.4 mg% X-Ray cervical spine done showed calcification adjacent to the dens. Otherwise no abnormality noted in the cervical spine. MR done showed no abnormality in the cervical cord and brain.CT scan showed calcification around dens- crown like density around odontoid.Diagnosis of crowned dense syndrome was made. The crowned dens syndrome (CDS) is a radioclinical entity defined-by the association of radiological calcifications of the cruciform ligament around the odontoid process and periodic acute cervico-occipital pains with fever, neck stiffness and biological inflammatory syndrome. The term derives from the crown-like density surrounding the odontoid process and was first coined in 1985.1 Patients with crowned dens syndrome typically present with severe neck pain and have calcium deposits around the odontoid process .. These crystalline deposits, most often calcium pyrophosphate dehydrate (CPPD) crystals or hydroxyapatite crystals, can remain asymptomatic or be responsible for chronic cervical pains or spinal cord compression Periodontoid calcification is seen on cervical computed tomography (CT) scan but is not typically visible on plain radiographs, making CT scanning invaluable in diagnosis. CT is the gold standard in identifying crowned dens syndrome, as it is able to depict the shape and site of calcification and any bone erosions.. Crowned dens syndrome misdiagnosed as polymyalgia, rheumatica, giant cell arteritis, meningitis or spondylitis References: 1. Rheumatology 2004;43:1508–1512doi:10.1093/rheumatology/keh370 Advance Access publication 17 August 2004 Crowned dens syndrome misdiagnosed as polymyalgiarheumatica, giant cell arteritis, meningitis or spondylitis: an analysis of eight cases A. Aouba, V. Vuillemin-Bodaghi, C. Mutschlerand M. De Bandt Crowned Dens syndrome Goto S1 etal 2007 Dec;89(12):2732-6. 2. Crowned Dens Syndrome Masami Matsumura, M.D., and Satoshi Hara, M.D. N Engl J Med 2012; 367:e34December 6, 2012DOI: 10.1056/NEJMicm1100764 3. Crowned Dens Syndrome Shinichi Goto, MD; Jutaro Umehara, MD; Toshimi Aizawa, MD; Shoichi Kokubun, MD J Bone Joint Surg Am, 2007 Dec 01; 89 (12): 2732 -2736 . http://dx.doi.org/10.2106/JBJS.F.01322 4. Severe Neck Pain Due to Crowned Dens Syndrome Fri, 10/12/12 Authors: Luciana funtowicz, MD,Elizabeth b. Windgassen, MD,Lester e. Mertz, MD Mayo Clinic, Scottsdale, Arizona 5. The crowned dens syndrome as a cause of neck pain: clinical and computed tomography study in patients with calcium pyrophosphate dihydrate deposition disease F. Salaffi,M. Carotti, G. Guglielmi, G. Passarini, W. Grassi Cattedra di Reumatologia, Dipartimento di Patologia Molecolare e Terapie Innovative, and Dipartimento di Radiologia S.O.D. Radiologia Clinica, Universit à Politecnica delle Marche, Ancona, Italy; Cattedra di Radiologia, Universit à di Foggia, IRCCS Ospedale San Giovanni Rotondo, Foggia, Italy; Servizio di Radiologia, Ospedale di Jesi, Ancona, Italy. 6. CPPD of the cervical spine Case contributed by Dr Erik Ranschaert • Crowned dens syndrome: a manifestation of hydroxy-apatite rheumatism • S. A. Malca, P. H. Roche, W. Pellet, A. Combalbert 7. J Bone Joint Surg Am. 2007 Dec;89(12):2732-6. Crowned Dens syndrome. Goto S1, Umehara J, Aizawa T, Kokubun S.