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Synovial chondromatosis (SC) is a benign proliferative process of synovial tissue creating multiple cartilaginous nodules in joints. It most commonly occurs in the large joints of the knee, hip, and shoulder, uncommonly in the small joints of the hand and foot, and only rarely in the tenosynovial membrane of tendon sheath, termed tenosynovial chondromatosis (TC). Unlike SC, TC predisposes to the foot or hand. The rarity and unfamiliarity of imagers with TC, as well as the variability of its histologic features often lead to an erroneous diagnosis of extraskeletal chondroma or even chondrosarcoma as in the present case. Calcium pyrophosphate dehydrate (CPPD) crystals are usually deposited in the articular cartilage or periarticular structures such as synovium and capsule, and rarely in other soft tissue structures including bursa, tendon, subcutaneous tissue, and dura mater. CPPD crystals may also be deposited in extraskeletal chondroma and SC. We present an exceptionally rare case of huge tophaceous pseudogout associated with TC that is considered to arise from the flexor digitorum longus tendon sheaths of the foot, initially mistaken for a chondrosarcoma.

Alkaptonuria is a rare disease with autosomal recessive inheritance and variable expression. The weight-bearing joint involvement and spondylitis-like vertebral changes occur only after the 3rd decade. Musculoskeletal ultrasonographic findings in alkaptonuria were only rarely described, consisting mainly into enthesopathy and non-synovial tendon degeneration. We present the case of a 50 years old man with alkaptonuria and discuss the ultrasonographic findings and the relationship of the disease with chondrocalcinosis. The tendinous and synovial aspect may be peculiar and it could therefore allow recognition and screening for alkaptonuria, along with clinical and radiologic data.

We report a case of an 80-year-old woman with an acute carpal tunnel syndrome. This was based on an acute episode of chondrocalcinoses and accompagnied by a prolaps of the median nerve into a gap of the forearm fascia as result of a cut injury in childhood., (© Georg Thieme Verlag KG Stuttgart · New York.)

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is one of the most common forms of crystal-associated arthropathy in the elderly. However, cervical spine is rarely affected, especially in upper cervical area. There have been previous reported cases of symptomatic retro-odontoid CPPD deposition disease in English literature but this case is the first reported in Thai patients. This is a case report of a 67-year-old man who presented with neck pain with progressive myelopathy. Neurologic examination demonstrated a cervical myelopathy with muscle weakness and sensory disturbance of both extremities. Imaging studies showed extradural retro-odontoid mass compressing the spinal cord. The patient underwent occiput to C3 fusion with plating, posterior arch of atlas resection, transoral odontoidectomy, and mass removal. Histological examination of the mass revealed fibrocartilage tissue and rhomboid shaped crystals that showed positive biferingent in polarized light microscopy consistent with CPPD crystals. After surgery, no complication was found, and his neurological function had improved.

Background and Purpose: Crowned dens syndrome is an ill-known etiology of acute neck pain., Methods: We carried out a retrospective study of 18 cases of patients with crowned dens syndrome, assessing clinical and radiological features., Results: The results of our study are comparable to data from the literature. The clinical presentation of acute febrile neck pain, occipital headache and multidirectional stiff neck especially affects women aged over 60. No predisposing factor was recognized. However, a history of peripheral joint chondrocalcinosis may reinforce the diagnosis. In more than 50% of cases, laboratory tests showed a marked inflammatory syndrome. The diagnosis was obtained with cervical CT-scan focusing on the C1/C2 joint. This gold standard test was able to show a calcification of the cruciform ligament in connection with deposits of calcium pyrophosphate crystals in almost 80% of cases. Other imaging tests provided little information, including standard radiographs of the cervical spine. MRI can eliminate some differential diagnoses such as infections or neurological emergencies. Complications are infrequent. The standard treatment is based on anti-inflammatory drugs (NSAID, colchicine) or corticosteroids. These treatments are highly effective: a drammatic full recovery of cervical mobility may be observed within 48 hours. In over half of cases, a different diagnosis was initially made, responsible of unnecessary additional tests and treatment., Conclusion: A comprehensive consultation, a complete clinical examination and a precise analysis of the imaging will avoid certain investigations and rule out differential diagnoses., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Introduction: Ulnar nerve lesions at the wrist (UNLW) are always difficult to localize clinically and sometimes electrophysiologically. Finding conduction block when studying ulnar motor nerve conduction (CB) across the wrist is sometimes the only way to demonstrate that the ulnar deep motor branch (UDMB) is entrapped., Methods: An elderly woman who had bilateral carpal tunnel syndrome (CTS) and thumb osteoarthritis for many years experienced worsening of left hand impairment recently., Results: Electrodiagnostic and ultrasound examinations revealed an acute and severe UDMB lesion related to pisotriquetral joint effusion. The patient received a local injection of a corticosteroid that provided rapid recovery., Conclusions: The diagnosis of UDMB lesion is especially difficult when CTS coexists, but CTS may allow for early diagnosis, if CB at the wrist is not overlooked. Chondrocalcinosis was responsible for the systemic inflammation, the CTS, the pisotriquetral joint effusion, and the UDBM compression, which has not been reported previously., (Copyright © 2012 Wiley Periodicals, Inc.)

Acute monoarthritis is one of the most common rheumatologic presentations. However, it is clinically difficult to distinguish between an inflamed joint due to crystal-induced arthritis and an inflamed joint due to septic arthritis. Arthrocentesis and synovial fluid analysis are used to differentiate between these 2 conditions. The presence of crystals and positive synovial fluid culture confirm the diagnosis of crystal-induced arthritis and septic arthritis, respectively. Although uncommon, these 2 arthritides can coexist, and presence of crystal does not exclude bacterial arthritis. We reported a case of 85-year-old woman whose synovial fluid contained crystals and was initially diagnosed with crystal-induced arthritis. However, her joint fluid culture subsequently grew Staphylococcus aureus, and she was treated with arthroscopic debridement and antibiotics., (Copyright © 2013 Elsevier Inc. All rights reserved.)

We report a case of closed rupture of the flexor tendons of the little finger caused by calcium pyrophosphate dihydrate crystal deposition disease of the pisotriquetrum joint. The patient could not flex the little finger and did not have wrist pain. Plain radiographs of the affected wrist joint showed severe arthritic changes of the pisotriquetrum joint and calcification around the joint. At operation, the pisotriquetrum joint capsule was ruptured and involved the flexor tendon of the little finger. The distal stump of the flexor tendon was transferred to the flexor tendon of the ring finger, and the pisiform was resected. Histological examination with polarized light microscopy revealed crystals showing weakly positive birefringence in the calcification.

Acute hip pain is a common reason for attendance to hospital. Immediate diagnoses include occult fractures, infectious and non-infectious inflammatory processes such as gout and pseudogout. Calcium pyrophosphate deposition disease (CPDD) is a rheumatological syndrome affecting articular cartilage and/or synovial fluid. It can occur as a clinical presentation (pseudogout) where calcium pyrophosphate dihydrate (CPPD) crystals are deposited within the joint space or radiographically, as chondrocalcinosis (CC), where CPPD crystals are deposited onto articular cartilages. Extraarticular manifestations of CPDD are rare. The authors report a case of CPPD deposition in the trochanteric bursa of a 35-year-old woman presenting as acute hip pain. The patient was treated successfully during arthroscopy with removal of the deposits and excision of the bursa. The patient remains well at 6 month follow-up.

Background: Coexistence of degenerative arthritis and calcium pyrophosphate dihydrate (CPPD) crystals (or radiological chondrocalcinosis) with osteoarthritis (OA) of the knees is frequent at the time of arthroplasty. Several studies suggest more rapid clinical and radiographic progression with CPPD than with OA alone. However, it is unclear whether chondrocalcinosis predisposes to higher risks of progression of arthritis in other compartments., Question/purposes: We questioned whether chondrocalcinosis influences clinical scores, degeneration of other compartments, rupture of the ACL, survivorship, reason for revision, or timing of failures in case of UKA., Methods: We retrospectively reviewed 206 patients (234 knees) who had UKAs between 1990 and 2000. Of these 234 knees, 85 had chondrocalcinosis at the time of surgery and 63 of the knees subsequently had radiographic evidence of chondrocalcinosis observed during followup. We evaluated patients with The Knee Society rating system and compared function and radiographic progression in the other compartments of patients without and with chondrocalcinosis., Results: The use of conventional NSAIDs, radiographic progression of OA in the opposite femorotibial compartment of the knee, failure of the ACL, and aseptic loosening did not occur more frequently among patients with chondrocalcinosis. The 15-year cumulative survival rates were 90% and 87% for the knees without and with chondrocalcinosis, respectively, using revision to TKA as the end point., Conclusion: Our findings show chondrocalcinosis does not influence progression and therefore is not a contraindication to UKA.

Calcium pyrophosphate dihydrate deposition disease (CPDD, tophaceous pseudogout) is a rare crystal arthropathy characterized by pyrophosphate crystal deposition in joints, synovitis and chondrocalcinosis on imaging. We present the case of a 72-year-old man with 6 months of left chest pain; magnetic resonance imaging revealed a T9/T10 herniated disc. Intraoperatively, the material was sent for pathological analysis revealing pseudogout. Axial calcium pyrophosphate crystal deposition is rare but reported in the literature and found at the craniocervical junction and skull. Spinal calcium pyrophosphate crystal deposition is rare in the thoracic spine. It is often asymptompatic and can involve the disc or ligaments. This case demonstrates a unique presentation of CPDD.

Gout is the most common cause of inflammatory arthritis affecting at least 1% of the population in industrialized countries. It is closely associated with hyperuricemia and is characterized by formation and reversible deposition of monosodium urate crystals in joints and extra-articular tissues. Several studies suggest that the prevalence and incidence of gout are rising. Numerous risk factors may in part explain this increasing trend including dietary and lifestyle changes, genetic factors, diuretic use and comorbid conditions such as hypertension, diabetes, cardiovascular disease, chronic renal disease and the metabolic syndrome. Chondrocalcinosis is characterized by the deposition of calcium pyrophosphate crystals in articular tissues, most commonly fibrocartilage and hyaline cartilage. Sporadic chondrocalcinosis is a common condition in the elderly and frequently associates with osteoarthritis. Hereditary haemochromatosis, hyperparathyroidism and hypomagnesaemia are metabolic disorders that predispose to secondary chondrocalcinosis.The prevalence of chondrocalcinosis is still rather uncertain and varies depending on the diagnostic criterion used in different studies.

Calcium pyrophosphate dihydrate deposition disease typically involves the wrist joint in the form of calcifications of the triangular fibrocartilage and the distal radioulnar joint. We describe an 87-year-old male who presented to us with asymptomatic form of deposition with multiple flexor tendon calcifications causing chronic median nerve compression. Simple carpal tunnel decompression relieved his median nerve symptoms.

Objectives: Chondrocalcinosis is frequently associated with osteoarthritis. The role of osteoarthritis in the onset and progression of disability is well known. The impact of chondrocalcinosis on disability has never been investigated in epidemiological studies., Methods: Progetto Veneto Anziani is a survey of 3099 older Italians, focusing on chronic diseases and disability. Assessment was by questionnaires, physical performance tests and clinical evaluations. Chondrocalcinosis was determined by x-ray readings of 1629 consecutive subjects. Knee and hip osteoarthritis severity was evaluated by summing the radiographic features score (RFS) assigned during x-ray reading., Subjects: with chondrocalcinosis were older and more frequently women (age-adjusted p<0.0001). The gender association disappeared following adjustment for osteoarthritis severity. However, at the knee, the prevalence of osteoarthritis was higher in chondrocalcinosis patients independently of age and sex (age-adjusted p<0.0001). No difference was found between chondrocalcinosis and controls in sociodemographic variables and comorbidity. Knee chondrocalcinosis was strongly associated with clinical features of knee osteoarthritis and with disability assessment parameters in the bivariate analysis. Most associations remained after adjusting for age. After further adjustment for RFS, a significant association remained for knee deformity and pain, the need for a cane, difficulty walking 500 m, using a toilet, shopping and repeatedly rising from a chair., Conclusions: Pain and physical function are the outcome measures of choice for assessing disability in osteoarthritis patients. The presence of chondrocalcinosis contributes to both, independently of age and osteoarthritis severity, thus compromising the quality of life and worsening comorbidity.

We report a case of pseudogout manifested by severe posterior neck pain. Pseudogout of the neck, also known as the crowned dens syndrome, causes acute neck pain characterized by calcium pyrophosphate dehydrate deposition around the odontoid process. Crowned dens syndrome is typified clinically by severe cervical pain and stiffness, often in conjunction with raised inflammatory markers. A 71-year-old man presented with severe neck pain. On admission, elevation of serum CRP level was confirmed. Magnetic resonance images showed no responsible abnormalities except for degenerating change of the spine. The patient was diagnosed as having pseudogout caused by calcium pyrophosphate dehydrate deposition based on cervical computed tomographic imaging, which showed linear calcification in the transverse ligament of the axis. After administration of non-steroidal anti-inflammatory drugs, the fever and neck pain disappeared and the CRP level returned to within the normal range. Pseudogout of the cervical spine should be considered as a differential diagnosis when we examine patients with acute neck pain. Cervical spinal computed tomographic scan is a more sensitive and useful examination method to diagnose this disease rather than magnetic resonance images.

Background: Pseudogout, or calcium pyrophosphate deposition, is a rare cause of pain, swelling, and trismus of the temporomandibular joint (TMJ). Diagnosis and management of the lesion are discussed., Case Description: A 58-year-old female had a 2-month history of progressive swelling of right TMJ associated with trismus and facial pain. Imaging of the TMJ revealed a mixed radiolucent and radiopaque lesion associated with the right TMJ joint space. Surgical excision was performed successfully via preauricular approach. Pathology was consistent with calcium pyrophosphate deposition of the TMJ, also known as pseudogout. Surgical excision successfully treated her symptoms as expected. She is now disease free without recurrence., Clinical Implications: Pseudogout is a rare cause of TMJ pain, swelling, and trismus that should be included in the differential of joint pain and dysfunction. It can be treated successfully with surgery., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Purpose: We reviewed 21 consecutive patients who underwent a total wrist arthroplasty as a primary procedure between October 2001 and February 2007. The purposes of the present study were to communicate our midterm results and to compare them with previously published series., Methods: We evaluated all patients clinically and radiologically. We used the Patient-Related Wrist Evaluation a primary outcome measure. The mean follow-up was 5.5 years (range, 3-8 years). A total of 14 patients had rheumatoid arthritis, including 1 with juvenile arthritis, and 1 each had psoriatic arthritis, systemic lupus erythematosus, and undifferentiated spondyloarthropathy. Of the remaining 4 patients, 2 had grade IV Kienböck disease, 1 had degenerative arthrosis, and 1 had chondrocalcinosis., Results: Postoperative Patient-Related Wrist Evaluation scores averaged 24 points (SD, 21 pints) out of 100 (worst score). When the patients were specifically asked about pain and function of the arthroplasty, 20 claimed to be satisfied or very satisfied with the procedure. Two early and 3 late complications occurred. One patient had a wound hematoma and another had a superficial wound infection, both of which resolved with no further complications during the immediate postoperative period. In 2 patients, there was some osteolysis around the screw inserted into the medullary canal of the index metacarpal, but not in the trapezoid bone. One patient had a slight loosening of the distal component with subsidence on the ulnar side of the carpus. There have been no dislocations or surgical revisions of the components., Conclusions: Based on our study, a total wrist arthroplasty should be considered as a good alternative to arthrodesis for patients who wish to preserve some degree of mobility of the wrist., Type of Study/level of Evidence: Therapeutic IV., (Copyright © 2011 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

The contraindications for unicompartmental knee replacement (UKR) remain controversial. The views of many surgeons are based on Kozinn and Scott's 1989 publication which stated that patients who weighed more than 82 kg, were younger than 60 years, undertook heavy labour, had exposed bone in the patellofemoral joint or chondrocalcinosis, were not ideal candidates for UKR. Our aim was to determine whether these potential contraindications should apply to patients with a mobile-bearing UKR. In order to do this the outcome of patients with these potential contraindications was compared with that of patients without the contraindications in a prospective series of 1000 UKRs. The outcome was assessed using the Oxford knee score, the American Knee Society score, the Tegner activity score, revision rate and survival. The clinical outcome of patients with each of the potential contraindications was similar to or better than those without each contraindication. Overall, 678 UKRs (68%) were performed in patients who had at least one potential contraindication and only 322 (32%) in patients deemed to be ideal. The survival at ten years was 97.0% (95% confidence interval 93.4 to 100.0) for those with potential contraindications and 93.6% (95% confidence interval 87.2 to 100.0) in the ideal patients. We conclude that the thresholds proposed by Kozinn and Scott using weight, age, activity, the state of the patellofemoral joint and chondrocalcinosis should not be considered to be contraindications for the use of the Oxford UKR.

Objectives: Osteoarthritis (OA) is the most common articular disease. Common OA treatments are either not effective or associated with side effects. Calcium-containing crystals are quite common in primary OA and they worsen or may cause OA through induction of inflammation by neutrophils. Colchicine inhibits urate-crystal and calcium-pyrophosphate (CPP) crystal induced inflammation and elastase, a matrix-metalloproteinase (MMP) that play a pivotal role in degenerative joint processes. Hence, it was hypothesized that it may have symptom-modifying effects on OA., Methods: Sixty-one postmenopausal patients with primary knee OA were enrolled. None of them had joint involvement atypical for OA or evidences of chondrocalcinosis in radiographic studies suggesting the presence of calcium-pyrophosphatedeposition-disease (CPPD). Participants were allocated to two groups receiving 0.5mg colchicines BID or placebo. Both groups received common OA treatments. Acetaminophen less than 2gr/day was used as rescue-analgesic. The efficacy end points were: patients' global assessment and physician's global assessment, recorded on a VAS (visual analogue scale). Statistical analysis was performed 3 months later., Results: Thirty-one patients were assigned to the colchicine group. Fifty-eight patients were present for the last survey. Only 1 patient in colchicine group encountered adverse effect of colchicine without significant difference between the two groups. Acetaminophen consumption was significantly less in the colchicine (879.3±369.7) compared to placebo group (1620.7±393.1, p=0.000). Improvement rate at the end of 3 months was significantly higher in the colchicine group for both patients' global assessment and physician's global assessment measures compared to placebo group, (11.14±4.06 vs. 3.14±2.18, p=0.000) and (9.83±3.799 vs. 3.72±3.35, p=0.000), respectively., Conclusions: The efficacy and safety of colchicine for pain reduction in OA was affirmed by our double-blind randomised controlled trial.

Purpose: We report the unusual case of a 63-year-old man with gout and coexisting pseudogout (calcium pyrophosphate dihydrate crystal deposition disease) affecting the same joint. In this report, we describe the clinical features of the patient in addition to the characteristics of the crystals extracted from his affected joint, examined using compensated polarized microscopy., Methods: Synovial fluid and specimens of the lateral meniscus of the patient's knee joint taken during arthroscopy were analyzed using compensated polarized microscopy to evaluate the presence and type of crystals., Results: Compensated polarized microscopy revealed both monosodium urate (MSU) and calcium pyrophosphate dihydrate (CPPD) crystals extracted from the knee joint., Conclusions: Our findings suggest that the coexistence of gout and pseudogout should be considered in the diagnosis of crystal-induced arthritis. Careful crystal evaluation using compensated polarized microscopy may reveal similar cases with MSU and CPPD crystals in the same joint.

Objectives: To develop evidence-based recommendations for management of calcium pyrophosphate deposition (CPPD)., Methods: A multidisciplinary guideline development group of 15 experts, representing 10 European countries, generated key propositions for management of CPPD using a Delphi consensus approach. For each recommendation research evidence was searched systematically. Whenever possible, the effect size and number needed to treat for efficacy and RR or OR for side effects were calculated for individual treatment modalities. Strength of recommendation was assessed by the European League Against Rheumatism visual analogue scale., Results: Nine key recommendations were generated, including topics for general management, treatment of acute attacks, prophylaxis against recurrent acute attacks and management of chronic symptoms. It was recommended that optimal treatment requires both non-pharmacological and pharmacological treatments. For acute CPP crystal arthritis, cool packs, temporary rest and joint aspiration combined with steroid injection are often sufficient. For prophylaxis or chronic inflammatory arthritis with CPPD, oral non-steroidal anti-inflammatory drugs with gastroprotective treatment and/or low-dose colchicine 0.5-1.0 mg daily may be used. Other recommendations included parenteral or oral corticosteroid for acute CPP arthritis in those unresponsive or unsuited to other measures, and low-dose corticosteroid, methotrexate or hydroxychloroquine for chronic inflammatory arthritis with CPPD. Asymptomatic CPPD requires no treatment. Strength of recommendations varies from 79% to 95%., Conclusion: Nine key recommendations for management of CPP crystal associated arthritis were developed using both research evidence and expert consensus. Strength of recommendations was provided to assist the application of these recommendations.

Calcium pyrophosphate dihydrate crystal-deposits (CPPD) at the spine are rare but the lesions detected with CT scans or MRI are often interpreted as a spondylodiscitis or osteitis. CPPD is a disease of the elderly without major sex predominance. The diagnosis of CPPD requires typical manifestations on a radiograph and/or detection of positively birefringent crystals in the synovial fluid of (peripheral) joints by compensated polarized light microscopy. CPPD crystal deposition at the spine has been associated with clinical manifestations, typically spine stiffness, and is sometimes associated with bony ankylosis or diffuse idiopathic skeletal hyperostosis. The preferred treatment of CPPD in the acute phase is oral non-steroidal anti-inflammatory medication or alternatively oral or intravenous glucocorticoids. CPPD should be considered in patients with non-specific spinal lesions., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

Calcium pyrophosphate dihydrate (CPPD) disease is the second most common crystal-induced form of arthropathy, frequently seen in the knee, shoulder, wrist, elbow, and ankle. The acute form of the disorder is referred as pseudogout, which can cause a severe joint inflammation. We present a case of monoarticular pseudogout of the hip joint whose symptoms mimicked septic arthritis. The definitive diagnosis was only confirmed after the microscopic analysis of joint aspirate.

We report a case of CPPD crowned dens syndrome in an 87 year white old male with a known history of pseudogout, with clinical and radiological features characteristic of this syndrome. Interestingly, there was significant mass effect on the clivus, with clivus erosion and destruction, a finding that has not previously been described with this syndrome. The clinical and radiological characteristics of Crowned Dens syndrome, as well as CPPD are reviewed. We suggest that CPPD crowned dens syndrome may be included in the differential diagnosis when clivus destruction or erosion, in association with a soft tissue mass with calcification, is seen.

Acute pseudogout of the neck, also known as the crowned dens syndrome, is a rare cause of neck pain characterised by crystalline deposition in periodontoid articular tissues. It is typified clinically by severe cervical pain and stiffness, often in conjunction with pyrexia and raised inflammatory markers. As such, it is often misdiagnosed. We report 2 cases of crowned dens syndrome masquerading respectively as meningitis and polymyalgia rheumatica, and review the literature with particular attention to the clinical and radiological aspects of this under-recognised condition.

Introduction: Chondrocalcinosis is a microcrystalline arthropathy that principally affects the knee. It is a rare disorder, usually asymptomatic, that occurs mainly in the elderly people., Purpose: To report a case of a temporomandibular joint chondrocalcinosis with ossicular contact revealed by a conductive hearing loss., Case Report: We describe the case of a 57-year-old man with a right conductive sudden hearing loss of 15 dB. The CT scan revealed a lytic lesion in the right attic extended to the middle cerebral fossa in contact with the ossicles with a suspicion of lysis of the head of the malleus. MRI showed a lesion enhancing after gadolinium injection on T1 weighted images. A biopsy revealed a chondrocalcinosis of the temporomandibular joint. Due to the complexity of surgical excision and the benin character of the lesion, a medical treatment and a radiologic follow-up every six months were proposed., Conclusion: Chondrocalcinosis of the temporo-mandibular joint is rare especially when it is revealed by a hearing loss. We present here a review of the literature.

Patients with parathyroid disease can have important musculoskeletal problems.Hypoparathyroidism can cause subcutaneous calcifications, tetany, muscle cramps,and paresthesias, but also myopathies and an ankylosing spondylitis-like back disease. Hypoparathyroidism can occur in SLE caused by antiparathyroid antibodies.Patients with hyperparathyroidism can develop bone disease with cysts, erosions,and deformities. They can also develop pseudogout, gout, myopathies, and tendon ruptures., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is one of the most common forms of crystal-associated arthropathy in the elderly. However, CPPD deposition on the cervical spine is less well known, and only a limited number of cases have been reported to date. Here, we report our recent clinical experience with CPPD crystal deposition disease of the cervical spine and describe the clinical features of this disease., Methods: Fourteen patients with clinically diagnosed CPPD crystal deposition disease of the cervical spine at our department during the period from January 2005 to December 2008 were analyzed retrospectively., Results: Patients ranged in age from 54 to 92 (mean+/-SD, 77.5+/-8.5). Chief symptoms of patients were acute posterior neck pain and fever. All patients had markedly restricted neck rotation. Serum CRP level was highly elevated in all patients (10.16+/-5.35 mg/dL). Computed tomography of the cervical spine demonstrated linear calcific deposits in the transverse ligament of atlas (crowned dens syndrome) in all patients. Calcific deposits were also found in other periodontoid structures and the ligamenta flava in some patients. Posterior neck pain, fever, and increased serum inflammatory indicators were relieved within 1 to 3 weeks by nonsteroidal antiinflammatory drugs (NSAIDs) or a combination of NSAIDs and prednisolone. Most of the patients were misdiagnosed as having other diseases before consultation., Conclusions: CPPD crystal deposition disease of the cervical spine is one of the most common underrecognized causes of acute neck pain in the neurology department, especially in elderly patients., (2010 Elsevier B.V. All rights reserved.)

Calcium pyrophosphate dihydrate deposition disease (CPPD), also known as pseudogout or chondrocalcinosis, is a variety of metabolic arthropathy caused by the deposition of calcium pyrophosphate dihydrate crystals in and around joints. Despite many case reports, extra-articular CPPD often goes unrecognized. Here, we report a unique case of pancreatic tail pseudocyst and CPPD of the spleen. To the best of our knowledge, CPPD of the spleen has not been reported in the literature., (2010 Elsevier Inc. All rights reserved.)

Tophaceous pseudogout in the knee joint is rare. We report an 82-year-old man who presented with a one-year history of pain and swelling of the right knee joint. Treatment with non-steroidal anti-inflammatory drugs and aspiration of the joint effusion had not been effective. The mass continued to enlarge, and the patient had difficulty walking. Radiographs and computed tomography showed meniscal calcification with an abnormal soft-tissue mass surrounded by calcification. After excision, massive calcified deposits were seen both inside and on the surface of the tophaceous pseudogout. The deposits showed birefringence under polarised light, suggestive of calcium pyrophosphate dihydrate crystals. At the 2-year follow-up, the patient could walk independently without knee pain or swelling, although his range of knee motion was slightly limited due to joint contracture that developed before surgery.

Chondrocalcinosis may be asymptomatic or take three classical forms (acute recurrent inflammatory, chronic inflammatory, with osteoarthritis). Apart form that, CPP crystal arthropathies can mimic several rheumatic diseases, including polymyalgia, septic arthritis and spondylodiscitis. Several conditions can predispose to chondrocalcinosis, including hemochromatosis, hyperparathyroidism, familial hypocalciuric hypercalcemia, hypomagnesemia and treatment with tacrolimus or diuretics. The diagnostic sensitivity of ultrasound seems better than radiography and CT is useful in spinal forms but whenever possible, the identification of crystals in synovial fluid remains essential. NSAIDs and/or glucocorticoids are frequently sufficient to control symptoms but methotrexate, at anti-inflammatory doses (10-20 mg/wk) appears useful in refractory forms.

Calcium pyrophosphate dihydrate crystal deposition disease has various clinical features, and pseudogout is one of the six clinical forms. Chondrocalcinosis is the term used to describe the radiographic appearance of the disease. A review of the literature revealed that the appearance of this type of arthropathy in the foot is infrequent. We offer a review of the disease and report an atypical bilateral case of pseudogout in a patient 56 years of age without a history who presented with symptoms of arthritis localized in the first metatarsophalangeal joint associated with hallux valgus and was treated surgically. Radiographic evaluation of the feet did not reveal signs of chondrocalcinosis. The patient had no metabolic abnormalities, except for high uric acid values. Chemical analysis of the surgical samples demonstrated the presence of calcium pyrophosphate dihydrate crystals, confirming the diagnosis. We believe that arthropathy by deposition of calcium pyrophosphate dihydrate in the foot, although rare, must be considered in the podiatric physician's differential diagnosis when a patient presents with articular pain in the foot associated or not with deformities.

Objective: This study seek to establish the prevalence of calcification of the transverse ligament of the atlas (TLA) and of crowned dens syndrome (CDS) in patients with articular chondrocalcinosis. It also seeks to assess the main computed tomography appearances of these calcifications., Patients and Methods: Twenty-eight patients with articular chondrocalcinosis were prospectively evaluated for evidence of calcifications of the transverse ligament of the atlas. A group of patients with gout, fibromyalgia, and osteoarthritis were used as control., Results: Twenty-eight patients with articular chondrocalcinosis were prospectively evaluated for evidence of calcifications of the transverse ligament of the atlas. Twenty of the patients (71%) showed this calcification by computed tomography, disclosing 3 patterns of calcification: curvilinear in shape, thick, and forming a double band and mottled. Calcifications were not found in the controls (P < 0.0001). Nine patients were symptomatic (crowned dens syndrome). Patients with chondrocalcinosis and calcification of the transverse ligament of the atlas were older than the rest of the patients. Unlike the patient group, the majority of the patients in the control group were male (P = 0.02)., Conclusion: The presence of calcifications of the transverse ligament of the atlas in patients with chondrocalcinosis occurs more frequently than that reported earlier. The CT scan is the most sensitive method to detect it. Taking into account that calcifications of TLA may manifest as CDS in a high percentage of these patients, such possibility should be considered in all patients with neck pain, stiffness, fever, and inflammatory response so as to avoid invasive diagnostic methods and more aggressive treatments than the use of nonsteroidal anti-inflammatory drugs.

Background: The infratemporal fossa is a well-concealed, complex anatomical space. Neoplasms arising in this area are heterogenous in nature and have an insidious onset and usually present late., Methods and Results: We present a case of a 71-year-old woman who presented with worsening facial pain, trismus, and a large mass in her infratemporal fossa with minimal associated temporomandibular joint destruction. She underwent a surgical excision of the mass, which revealed a pseudogout deposit., Conclusion: The clinical and radiological features of patients with tophaceous pseudogout frequently mimic those of a benign or malignant neoplasm of the infratemporal fossa, often resulting in more radical surgery.

Gitelman syndrome is a rare inherited tubulopathy, characterized by hypomagnesemia, hypokalemia, metabolic alkalosis, hypocalciuria and hyperreninemic hyperaldosteronism. The clinical spectrum is wide and includes: cramps, myalgias, muscle weakness, until episodes of carpo-podalic spasm, tetania, rhabdomyolysis and paralysis. Some cases have been described in literature underlining the association of this condition with chondrocalcinosis, as a typical example of hypomagnesemia-induced crystal deposition disease. The therapy of Gitelman syndrome consists on the administration of defective electrolytes, although not always effective. We describe two cases of Gitelman syndrome associated with chondrocalcinosis showing the wide range of presentation of this clinical condition.

Background: Calcium pyrophosphate dihydrate crystal deposition disease (CPPD-CDD) has been associated to hypercalcemia. Familial hypocalciuric hypercalcemia (FHH) is a rare but important consideration in the differential diagnosis of hypercalcemia. This autosomal dominantly inherited condition is characterized by elevated plasma calcium levels, relative or absolute hypocalciuria, and normal to moderately elevated plasma PTH level. The disease is caused by inactivating mutations in the calcium-sensing receptor gene., Case Report: We describe a 77-year-old Italian man with arthritis secondary to CPPD-CDD and hypercalcemia. Clinical and biochemical data (s-Ca: 2.94 mmol/L; PTH: 5.9 pmol/L; 24 h urinary calcium: 69.6 mg; calcium/creatinine clearance: 0.004) suggested the diagnosis of FHH. Mild hypocalciuric hypercalcemia was also found in five of seven relatives confirming the diagnosis, of these one showed chondrocalcinosis., Conclusions: It is important to screen for FHH using fractional urinary excretion of calcium in subjects with CPPD-CDD associated to hypercalcemia, this approach may prevent unnecessary parathyroidectomy.

Objectives: Pyrophosphate arthropathy has been linked to diverse clinical subtypes. The two most common are: acute synovitis (pseudogout) and chronic pyrophosphate arthropathy ("pseudo-osteoarthritis"). We have conducted a study to examine whether these are overlapping syndromes., Methods: We reviewed all synovial fluid (SF) analyses performed in our laboratory from January 1988 to May 1997 to determine if patterns of SF leukocyte counts and Alizarin red stains in patients with repeated samples suggest that some patients were prone to acute attacks and some to chronic pyrophosphate arthropathy and whether acute attacks superimposed on chronic symptoms were common. Joint x-rays were screened for osteoarthritis (OA) and chondrocalcinosis., Results: We identified 67 patients who had Calcium pyrophosphate dehydrate (CPPD) in their SF and had more than one SF examined (185 SF). We divided the patients into 2 groups. Group A (n=25) had at least one SF leukocyte count > than 2000 per mm(3) and group B (n=42) had SF leukocyte counts always < than 2000 per mm(3). Chondrocalcinosis detected on x-ray was more common in group A versus group B, 48% versus 19% (p<0.05, Fisher's exact test). OA was mild (grades 0-1) in 39% of group A versus 12.5% of group B patients, but the difference between groups was not significant. CPPD crystals were not detected in 13.5% SFs previously having CPPD crystals. Alizarin red staining for suspected hydroxyapatite was more often 2+ to 3+ in group B (31.6%) compared to group A (15.5%; p<0.05, Fisher's exact test)., Conclusion: Acute synovitis and chronic pyrophosphate arthropathy are often two distinctive syndromes with some patients never having inflammatory attacks. Acute synovitis is more common in patients with chondrocalcinosis while chronic pyrophosphate arthropathy is associated with increased alizarin red staining and a trend suggestive of increased severity of OA.

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.

We describe the case of a 71-year-old man with recurrent pseudogout attacks affecting multiple joints. He had end-stage renal failure that contra-indicated the use of non-steroidal anti-inflammatory drugs and was resistant to therapy with glucocorticoids. Based on the recent findings that interleukin (IL)-1beta is involved in crystal-induced inflammation, the patient received anakinra, a specific IL-1 inhibitor, in order to treat an acute attack of pseudogout. In addition, anakinra was administered as preventive therapy 3days per week after each hemodialysis session. Under this treatment, he did not present any severe episode of arthritis after a follow-up of 8 months. This observation suggests that anakinra is efficacious and safe for the prevention of crystal-induced arthritis in patients with severe renal failure.