Periodontoid calcium pyrophosphate dihydrate deposition disease: “pseudogout” mass lesions of the craniocervical junction

Restricted access

✓ Between 1984 and 1996, seven patients with symptomatic masses located posterior to the odontoid process and containing calcium pyrophosphate dihydrate crystals were evaluated by the senior author (A.H.M.). All patients presented with distal paresthesias and myelopathy and underwent transoral-transpharyngeal resection of the anterior arch of C-1, the odontoid process, and the compressing mass. Histological examination revealed the characteristic changes of calcium pyrophosphate dihydrate (CPPD) deposition disease, with nodular deposits of birefringent rhomboid crystals. On magnetic resonance imaging, the masses appeared predominantly isointense with neural tissue on T1-weighted images and iso- to hyperintense on T2-weighted images. On computerized tomography scans, small areas of calcifications within the masses were apparent in all cases. All patients improved postoperatively, with six of seven patients requiring posterior fixation for instability as a second procedure. Calcium pyrophosphate dihydrate deposition causing periodontoid mass lesions is a distinct clinical disease entity that probably is underdiagnosed. In the authors' opinion, the diagnosis can often be established preoperatively by the distinctive neuroradiological appearance of the masses. Therefore, CPPD deposition disease should be considered in the differential diagnosis of masses of the craniocervical junction, because it is amenable to early surgical intervention. The consulting neuropathologist should be made aware of this diagnostic possibility at the time of surgery.

Article Information

Address reprint requests to: Arnold H. Menezes, M.D., Division of Neurosurgery, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, Iowa 52244.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Case 6. Neuroimaging studies obtained in an 83-year-old man with quadriparesis caused by a large epidural mass extending from the inferior clivus to the retroodontoid space. The typical neurodiagnostic imaging findings of retroodontoid calcium pyrophosphate dihydrate (CPPD) deposition disease are seen. A: Sagittal pregadolinium T1-weighted magnetic resonance (MR) image showing a predominantly iso- to hypointense mass. B: Sagittal T2-weighted magnetic resonance (MR) image showing a mixed-density mass that is mainly isointense with areas of hyperintense signal. C: Postgadolinium sagittal T1-weighted MR image displaying avid peripheral enhancement around the isointense center of the CPPD mass. D: Noncontrast axial computerized tomography scan of the C1–2 spinal level, demonstrating small globular areas of calcification within the retroodontoid mass.

  • View in gallery

    Case 7. Neuroimaging studies obtained in an 84-year-old man with left hand numbness and weakness and a large retroodontoid mass lesion. The typical neurodiagnostic imaging findings of retroodontoid calcium pyrophosphate dihydrate deposition disease are seen. A: Sagittal pregadolinium T1-weighted magnetic resonance (MR) image showing the mass as mostly isointense with respect to neural tissue, with a distinctive curvilinear hypointense signal caused by the tectorial membrane, which bisects the mass in the vertical plane. B: Sagittal T2-weighted MR image displaying a mixed signal intensity, being predominately hyperintense with an isointense center. C: Sagittal postgadolinium T1-weighted MR image demonstrating a thin rim of peripheral enhancement of the mass, which has a large isointense center. D: Axial noncontrast computerized tomography (CT) scan of the craniocervical junction showing multiple small globular and linear areas of calcification within the retroodontoid mass. E: Three-dimensional CT reconstruction demonstrating the spatial configuration of this patient's craniocervical junction as well as calcification within the retroodontoid space (arrows).

  • View in gallery

    Case 6. Photomicrographs showing histological specimens from the resected retroodontoid mass. Original magnifications × 50. Left: Unstained intraoperative touch preparation viewed under polarized light. Birefringent rhomboid crystals, consistent with calcium pyrophosphate dihydrate deposition disease, are seen. Due to the greater thickness of this specimen some of the crystals appear to be out of focus. Center: Formalin-fixed, nondecalcified section of the retroodontoid mass lesion viewed with standard light microscopy. H & E. Right: Same area of specimen shown in center panel, examined under polarized light, now revealing a small nodular deposit of numerous birefringent crystals.

References

  • 1.

    Chuzin YPanush RS: CPPDDD: what it is and why it is under-recognized. Bull Rheum Dis 44:351995Chuzin Y Panush RS: CPPDDD: what it is and why it is under-recognized. Bull Rheum Dis 44:3–5 1995

    • Search Google Scholar
    • Export Citation
  • 2.

    Ciricillo SFWeinstein PR: Foramen magnum syndrome from pseudogout of the atlanto-occipital ligament. Case Report. J Neurosurg 71:1411431989Ciricillo SF Weinstein PR: Foramen magnum syndrome from pseudogout of the atlanto-occipital ligament. Case Report. J Neurosurg 71:141–143 1989

    • Search Google Scholar
    • Export Citation
  • 3.

    Dirheimer YBensimon CChristmann Det al: Syndesmoodontoid joint and calcium pyrophosphate dihydrate deposition disease (CPPD). Neuroradiology 25:3193211983Dirheimer Y Bensimon C Christmann D et al: Syndesmoodontoid joint and calcium pyrophosphate dihydrate deposition disease (CPPD). Neuroradiology 25:319–321 1983

    • Search Google Scholar
    • Export Citation
  • 4.

    Doherty MDieppe P: Clinical aspects of calcium pyrophosphate dihydrate deposition. Rheum Dis Clin North Am 14:3954151988Doherty M Dieppe P: Clinical aspects of calcium pyrophosphate dihydrate deposition. Rheum Dis Clin North Am 14:395–415 1988

    • Search Google Scholar
    • Export Citation
  • 5.

    El-Khoury GYTozzi JEClark CRet al: Massive calcium pyrophosphate crystal deposition at the craniovertebral junction. AJR 145:7777781985El-Khoury GY Tozzi JE Clark CR et al: Massive calcium pyrophosphate crystal deposition at the craniovertebral junction. AJR 145:777–778 1985

    • Search Google Scholar
    • Export Citation
  • 6.

    Fam AGMorava-Protzner IPurcell Cet al: Acceleration of experimental lapine osteoarthritis by calcium pyrophosphate microcrystalline synovitis. Arthritis Rheum 38:2012101995Fam AG Morava-Protzner I Purcell C et al: Acceleration of experimental lapine osteoarthritis by calcium pyrophosphate microcrystalline synovitis. Arthritis Rheum 38:201–210 1995

    • Search Google Scholar
    • Export Citation
  • 7.

    Gantz BJRedleaf MIMenezes AH: Management of clivus and parasellar space neoplasms in Jackler RKBrackmann DE (eds): Neurootology. St. Louis: Mosby1994 pp 11011125Gantz BJ Redleaf MI Menezes AH: Management of clivus and parasellar space neoplasms in Jackler RK Brackmann DE (eds): Neurootology. St. Louis: Mosby 1994 pp 1101–1125

    • Search Google Scholar
    • Export Citation
  • 8.

    Imai SHukuda S: Cervical radiculomyelopathy due to deposition of calcium pyrophosphate dihydrate crystals in the ligamentum flavum: historical and histological evaluation of attendant inflammation. J Spinal Disord 7:5135171994Imai S Hukuda S: Cervical radiculomyelopathy due to deposition of calcium pyrophosphate dihydrate crystals in the ligamentum flavum: historical and histological evaluation of attendant inflammation. J Spinal Disord 7:513–517 1994

    • Search Google Scholar
    • Export Citation
  • 9.

    Ishida TDorfman HDBullough PG: Tophaceous pseudogout (tumoral calcium pyrophosphate dihydrate crystal deposition disease). Hum Pathol 26:5875931995Ishida T Dorfman HD Bullough PG: Tophaceous pseudogout (tumoral calcium pyrophosphate dihydrate crystal deposition disease). Hum Pathol 26:587–593 1995

    • Search Google Scholar
    • Export Citation
  • 10.

    Kingdom TTNockels RPKaplan MJ: Transoral-transpharyngeal approach to the craniocervical junction. Otolaryngol Head Neck Surg 113:3934001995Kingdom TT Nockels RP Kaplan MJ: Transoral-transpharyngeal approach to the craniocervical junction. Otolaryngol Head Neck Surg 113:393–400 1995

    • Search Google Scholar
    • Export Citation
  • 11.

    McCarty DJ JrKohn NNFaires JS: The significance of calcium phosphate crystals in the synovial fluid of arthritis patients: the “pseudogout syndrome.” I. Clinical aspects. Ann Intern Med 56:7117371962McCarty DJ Jr Kohn NN Faires JS: The significance of calcium phosphate crystals in the synovial fluid of arthritis patients: the “pseudogout syndrome.” I. Clinical aspects. Ann Intern Med 56:711–737 1962

    • Search Google Scholar
    • Export Citation
  • 12.

    Menezes AH: Congenital and acquired abnormalities of the craniovertebral junction (children and adults) in Youmans J (ed): Neurological Surgeryed 4. Philadelphia: WB Saunders1995 Vol 2 pp 10351089Menezes AH: Congenital and acquired abnormalities of the craniovertebral junction (children and adults) in Youmans J (ed): Neurological Surgery ed 4. Philadelphia: WB Saunders 1995 Vol 2 pp 1035–1089

    • Search Google Scholar
    • Export Citation
  • 13.

    Menezes AH: Rheumatological disorders in Menezes AHSonntag V (eds): Principles of Spinal Surgery. New York: McGraw-Hill1996 Vol 1 pp 705722Menezes AH: Rheumatological disorders in Menezes AH Sonntag V (eds): Principles of Spinal Surgery. New York: McGraw-Hill 1996 Vol 1 pp 705–722

    • Search Google Scholar
    • Export Citation
  • 14.

    Menezes AH: Transoral approaches to the clivus and upper cervical spine in Menezes AHSonntag V (eds): Principles of Spinal Surgery. New York: McGraw-Hill1996 Vol 2 pp 12411251Menezes AH: Transoral approaches to the clivus and upper cervical spine in Menezes AH Sonntag V (eds): Principles of Spinal Surgery. New York: McGraw-Hill 1996 Vol 2 pp 1241–1251

    • Search Google Scholar
    • Export Citation
  • 15.

    Menezes AHVanGilder JC: Transoral-transpharyngeal approach to the anterior craniocervical junction. Ten-year experience with 72 patients. J Neurosurg 69:8959031988Menezes AH VanGilder JC: Transoral-transpharyngeal approach to the anterior craniocervical junction. Ten-year experience with 72 patients. J Neurosurg 69:895–903 1988

    • Search Google Scholar
    • Export Citation
  • 16.

    Resnick DPineda C: Vertebral involvement in calcium pyrophosphate dihydrate crystal deposition disease. Radiographic-pathological correlation. Radiology 153:55601984Resnick D Pineda C: Vertebral involvement in calcium pyrophosphate dihydrate crystal deposition disease. Radiographic-pathological correlation. Radiology 153:55–60 1984

    • Search Google Scholar
    • Export Citation
  • 17.

    Rosenthal AKRyan LM: Treatment of refractory crystal-associated arthritis. Rheum Dis Clin North Am 21:1511611995Rosenthal AK Ryan LM: Treatment of refractory crystal-associated arthritis. Rheum Dis Clin North Am 21:151–161 1995

    • Search Google Scholar
    • Export Citation
  • 18.

    Ryan LMMcCarty DJ: Calcium pyrophosphate crystal deposition disease; pseudogout; articular chondrocalcinosis in McCarty DJ (ed): Arthritis and Allied Conditions. A Textbook of Rheumatologyed 10. Philadelphia: Lea & Febiger1985 pp 15151546Ryan LM McCarty DJ: Calcium pyrophosphate crystal deposition disease; pseudogout; articular chondrocalcinosis in McCarty DJ (ed): Arthritis and Allied Conditions. A Textbook of Rheumatology ed 10. Philadelphia: Lea & Febiger 1985 pp 1515–1546

    • Search Google Scholar
    • Export Citation
  • 19.

    Salcman MKhan ASymonds DA: Calcium pyrophosphate arthropathy of the spine: case report and review of the literature. Neurosurgery 34:9159181994Salcman M Khan A Symonds DA: Calcium pyrophosphate arthropathy of the spine: case report and review of the literature. Neurosurgery 34:915–918 1994

    • Search Google Scholar
    • Export Citation
  • 20.

    Schumacher HR: Pathology of crystal deposition disease. Rheum Dis Clin North Am 14:2692881988Schumacher HR: Pathology of crystal deposition disease. Rheum Dis Clin North Am 14:269–288 1988

    • Search Google Scholar
    • Export Citation
  • 21.

    Smoker WRKKeyes WDDunn VDet al: MRI versus conventional radiologic examinations in the evaluation of the craniovertebral and cervicomedullary junction. Radiographics 6:9539931986Smoker WRK Keyes WD Dunn VD et al: MRI versus conventional radiologic examinations in the evaluation of the craniovertebral and cervicomedullary junction. Radiographics 6:953–993 1986

    • Search Google Scholar
    • Export Citation
  • 22.

    VanGilder JCMenezes AH: Craniovertebral junction abnormalities in Wilkins RHRengachary SS (eds): Neurosurgery. New York: McGraw-Hill1996 Vol 3 pp 35873592VanGilder JC Menezes AH: Craniovertebral junction abnormalities in Wilkins RH Rengachary SS (eds): Neurosurgery. New York: McGraw-Hill 1996 Vol 3 pp 3587–3592

    • Search Google Scholar
    • Export Citation
  • 23.

    Wackym PABlackwell KE: Abnormalities of the craniovertebral junction in Jackler RKBrackmann DE (eds): Neurootology. St. Louis: Mosby1994 pp 12031231Wackym PA Blackwell KE: Abnormalities of the craniovertebral junction in Jackler RK Brackmann DE (eds): Neurootology. St. Louis: Mosby 1994 pp 1203–1231

    • Search Google Scholar
    • Export Citation
  • 24.

    Weber MGerber H: Akutes Zervikalsyndrom bei Chondrocalcinose. Drei ältere Patienten mit Verkalkungen des Ligamentum transversum atlantis. Schweiz Med Wochenschr 121:6426451991Weber M Gerber H: Akutes Zervikalsyndrom bei Chondrocalcinose. Drei ältere Patienten mit Verkalkungen des Ligamentum transversum atlantis. Schweiz Med Wochenschr 121:642–645 1991

    • Search Google Scholar
    • Export Citation
  • 25.

    Wells CRMorgello SDicarlo E: Cervical myelopathy due to calcium pyrophosphate dihydrate deposition disease. J Neurol Neurosurg Psychiatry 54:6586591991Wells CR Morgello S Dicarlo E: Cervical myelopathy due to calcium pyrophosphate dihydrate deposition disease. J Neurol Neurosurg Psychiatry 54:658–659 1991

    • Search Google Scholar
    • Export Citation
  • 26.

    Willoughby DADunn CJYamamoto Set al: Calcium pyrophosphate-induced pleurisy in rats: a new model of acute inflammation. Agents Actions 43:2212241994Willoughby DA Dunn CJ Yamamoto S et al: Calcium pyrophosphate-induced pleurisy in rats: a new model of acute inflammation. Agents Actions 43:221–224 1994

    • Search Google Scholar
    • Export Citation
  • 27.

    Wittchow RLandas SSchelper R: Correlative light microscopy, scanning electron microscopy and energy dispersive spectroscopy of pseudogout presenting as spinal masses. Am J Clin Pathol 95:2741991 (Abstract)Wittchow R Landas S Schelper R: Correlative light microscopy scanning electron microscopy and energy dispersive spectroscopy of pseudogout presenting as spinal masses. Am J Clin Pathol 95:274 1991 (Abstract)

    • Search Google Scholar
    • Export Citation

Cited By

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 106 106 9
Full Text Views 130 130 0
PDF Downloads 91 91 0
EPUB Downloads 0 0 0

PubMed

Google Scholar