A Case of Acute Neck Pain: The Crowned Dens Syndrome

Ojemolon, Pius; Edigin, Ehizogie; Annapureddy, Narender; Manadan, Augustine

doi:10.7759/cureus.9555

Cited by 3 publications

(3 citation statements)

References 15 publications

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“…CPPD is often deposited around the C1-C2 joints involving the odontoid process and surrounding ligaments amongst which calcifications of the cruciate ligament, ligament flavum, and transverse ligament of the atlas are more common. CPPD crystal deposition can cause focal aseptic inflammation to stimulate C1 or C2 nerve roots that in turn can cause pain when the neck is rotated ( 10 – 12 ). However, the reason why CPPD is deposited is not yet clear.…”

Section: Discussionmentioning

confidence: 99%

Crowned Dens Syndrome: A Case Report and Literature Review

Huang

2022

Front. Med.

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Section: Discussionmentioning

confidence: 99%

Crowned Dens Syndrome: A Case Report and Literature Review

Huang

2022

Front. Med.

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“…Most authors have recommended treatment with NSAIDs and/or steroids (especially prednisolone) ( 13 , 24 , 25 ). Oka et al ( 17 ) summarized in their review that 85% of the patients with CDS were treated with NSAID alone or NSAID with another drug.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Posterolateral Epidural Supra-C2-Root Approach (PESCA) for Biopsy of a Retro-Odontoid Lesions in Same Sitting After Occipitocervical Fixation and Decompression in a Case of Crowned Dens Syndrome With Brainstem Compression and Displacement

et al. 2022

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Background‘Crowned dens syndrome' (CDS) is a special form of calcium pyrophosphate dihydrate deposition disease which is characterized radiologically by a halo-like or crown-like distribution in the periodontoid region and clinically by cervical pain. Herein, we will describe our experience of posterolateral epidural supra-C2-root approach (PESCA) for biopsy of retro-odontoid lesions in one surgical session after occipitocervical fixation and decompression in a patient with CDS and massive brainstem compression.Case PresentationA 70-year-old woman presented to our department with a 4-week history of progressive walking impairment, neck pain, neck rigidity, fever, dizziness, slight palsy of the left hand, and multiple fall episodes. Magnetic resonance imaging (MRI) of the craniovertebral junction (CVJ) and cervical spine revealed a lesion of the odontoid process and the retro-odontoid region with mainly solid components, as well as small cystic components, and brainstem compression and displacement. In first step, fusion surgery of the CVJ C0–C4 was performed with occiptocervical decompression. After fusion and decompression the lower lateral part of the C1 arc and the lateral superior part of the left side of the C2 arc were removed. The entry point was located directly above the superior part of the C2 root. A biopsy of the lateral portions of the lesions was obtained by bioptic forceps under microscope guidance. Pathologic examination of the mass revealed deposition of birefringent crystals compatible with calcium pyrophosphate. In addition to the clinical symptoms (especially neck pain), the diagnosis of CDS was made. Non-steroidal inflammatory drugs (NSAIDs) and colchicine (and later magnesium) were started. At follow-up examination 6 months after surgery, an MRI scan of the cervical spine revealed regression of the pannus and the cyst with replacement of the brainstem, clinical improvement of walking, and increased strength of the left hand.ConclusionsThis study demonstrates that PESCA can be used to obtain tissue for pathological analysis in one surgical sitting after fusion and decompression and that fusion, decompression, and PESCA (in the same session) together with subsequent conservative management could be a good alternative for the treatment of CDS.

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“…As seen in our patient, CDS can mimic and lead to an incorrect diagnosis of meningitis; when neck stiffness is associated with pain in the shoulder girdle and jaw claudication, polymyalgia rheumatica (PMR) and/or giant cell arteritis (GCA) may also be close differentials. CT scan focusing on C1/C2 is the gold standard for diagnosis [ 11 ]. This also helps delineate the anatomical subtypes of CDS [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Crowned Dens Syndrome Masquerading as Meningitis

Bamgboje¹,

Mohandas²

2021

Cureus

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The classic symptoms of meningismus, including fever, neck stiffness, and headache, should automatically trigger a prime differential of meningitis, but a close masquerader, albeit rare, is crowned dens syndrome. Herein, we report the case of a 71-year-old woman with clinical features of meningismus with elevated inflammatory biomarkers. However, computed tomography of the cervical spine revealed the presence of calcium deposits encircling the dens. Hence, an alternate diagnosis of crowned dens syndrome was considered. This was confirmed by the presence of similar pathology in other joints and the dramatic resolution of symptoms and inflammatory markers with the administration of nonsteroidal anti-inflammatory drugs.

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A Case of Acute Neck Pain: The Crowned Dens Syndrome

Cited by 3 publications

References 15 publications

Crowned Dens Syndrome: A Case Report and Literature Review

Crowned Dens Syndrome: A Case Report and Literature Review

Case Report: Posterolateral Epidural Supra-C2-Root Approach (PESCA) for Biopsy of a Retro-Odontoid Lesions in Same Sitting After Occipitocervical Fixation and Decompression in a Case of Crowned Dens Syndrome With Brainstem Compression and Displacement

Crowned Dens Syndrome Masquerading as Meningitis

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