Cases reported "Chondrocalcinosis"

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1/17. A very rare benign tumour in the parotid region: calcium pyrophosphate dihydrate crystal deposition disease.

    calcium pyrophosphate dihydrate crystal deposition disease, exhibits several clinical manifestations, from absence of symptoms to severely destructive arthropathy or conditions simulating neoplasm, which is frequently related to the temporomandibular joint. Fifteen of the 31 reported cases of tophaceous pseudogout were found in the head and neck region. A patient presented with a parotid swelling, which initially was suspected to be malignant because of the following findings: radiodensity, progression into the joint, osseous destruction of the major ala of the sphenoid and a fine needle aspirate with crystals, osteoblasts, megakaryocytes and irregular cells of varying size. At surgery there was found a tumour consisting of a white, firm gritty material. It progressed to the skull base where material had to be left, because of the presence of the nerves and vessels. A frozen specimen was reported to be benign. Histological examination showed inflammatory cells, macrophages, a chondroid material with embedded metaplastic chondroid cells and giant cells of foreign body type. Crystal examination of x-ray diffraction revealed calcium pyrophosphate dihydrate.
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2/17. diagnosis of calcium pyrophosphate dihydrate deposition disease by fine needle aspiration biopsy: a case report.

    BACKGROUND: calcium pyrophosphate dihydrate deposition disease is a relatively rare disease with variable clinical presentations. CASE: A 73-year-old man presented with worsening lower back pain and fever. Fine needle aspiration biopsy of the lumbar vertebral bodies (L3-L4) revealed abundant neutrophils admixed with small, birefringent, rhomboid crystals in Diff-Quik-stained smears. These crystals were confirmed as calcium pyrophosphate dihydrate on cell block sections. A diagnosis of osteomyelitis and calcium pyrophosphate dihydrate deposition disease was rendered. The patient was treated with antibiotics and responded well. CONCLUSION: calcium pyrophosphate dihydrate deposition disease can be diagnosed by fine needle aspiration biopsy, and an accurate diagnosis can be greatly facilitated by cell block sections. However, such a diagnosis may be neglected if the specimen is not carefully inspected.
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3/17. A case of calcium pyrophosphate dihydrate crystal deposition disease presenting as an acute polyarthritis.

    We report a case of calcium pyrophosphate dihydrate crystal deposition disease (CPDD) presenting as an acute polyarthritis. A 66-yr-old woman was admitted with a 5-day history of fever and multiple joint pain including wrists, elbows, shoulders, knees, and ankles developed 5 days before admission. Her plain radiographs of wrists, elbows, shoulders, knees, and ankles showed chondrocalcinosis. The pubic symphysis, lumbar intervertebral discs, and both hip joints, which were asymptomatic, also had calcium deposits. The compensated polarized microscopic examination of the joint fluid, aspirated from the right knee revealed intracellular and extracellular weakly positive birefringent crystals, confirming the CPDD. This case showed that CPDD may manifest as an acute polyarthritis mimicking acute onset rheumatoid arthritis.
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4/17. Tumoural calcium pyrophosphate dihydrate crystal deposition disease presenting clinically as a malignant soft tissue mass diagnosed on fine needle aspiration biopsy.

    Tumoural calcium pyrophosphate dihydrate crystal deposition is a rare manifestation of calcium pyrophosphate deposition disease (CPPD). We present the case of a 75-year-old male with a previously resected rectal adenocarcinoma who developed a 5-cm right-sided mass at the base of his neck. Clinically and radiologically the lesion was suspicious for malignancy, possibly of metastatic origin. A bedside fine needle aspirate was performed and the smears were mildly cellular showing histiocytes with numerous intracellular and extracellular crystals. These colourless crystals were mostly short and rhomboid shaped and demonstrated weakly positive birefringence. A diagnosis of tumoural CPPD was made. This case is only the second in the English literature diagnosed on fine needle aspiration biopsy. Tumoural CPPD is well known to be a clinical, radiological and occasionally pathological mimic of malignancy. Several cases have been reported where unnecessary radical surgery was performed for this condition. Fine needle aspiration biopsy, as in this case, can provide a rapid and accurate diagnosis of CPPD, avoiding the need for invasive procedures. Polarisation microscopy is a vital adjunct to confirm this diagnosis.
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5/17. Crowned dens syndrome misdiagnosed as polymyalgia rheumatica, giant cell arteritis, meningitis or spondylitis: an analysis of eight cases.

    BACKGROUND: The crowned dens syndrome, related to microcrystalline deposition in the peri-odontoid articular and abarticular structures, is mainly responsible for acute or chronic cervical pain. patients: We report eight cases of crowned dens syndrome with atypical presentations mimicking giant cell arteritis, polymyalgia rheumatica, meningitis or discitis. The clinical and radiological aspects of these cases are presented and discussed. RESULTS: For all patients, fever, cervical stiffness, headaches and biological inflammatory syndrome were reported. For three patients, impairment of general condition, occipito-temporal or mandible pain and weakness with inflammatory pain of the shoulder girdle was suggestive of giant cell arteritis and/or polymyalgia rheumatica, leading to temporal artery biopsy and/or long-term steroid treatment. recurrence of clinical symptoms when tapering steroids was noted. In two cases, previous breast carcinoma led to the initial diagnosis of metastatic spondylitis. For three patients with vomiting, nausea and Kernig's and/or Brudzinski's sign, the first diagnosis was meningitis, leading to unhelpful lumbar puncture. In all cases, diagnosis of crowned dens syndrome once evoked, was confirmed by cervical CT scanning and dramatic improvement with non-steroidal anti-inflammatory drugs or colchicine. CONCLUSION: This under-recognized entity must be considered as a differential diagnosis of meningitis and discitis, but also of giant cell arteritis and polymyalgia rheumatica, as well as a possible aetiology for fevers of unknown origin. CT scanning is necessary for diagnosis. Clinicians should be aware of such misleading clinical presentations.
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6/17. chondrocalcinosis of the temporomandibular joint: an external ear canal pseudotumor.

    An unusual case of calcium pyrophosphate arthropathy (pseudogout) involving the temporomandibular joint of a 53-year-old woman presented clinically as an ear canal tumor. Further evaluation and surgical exploration disclosed chondrocalcinosis of the temporomandibular joint with involvement of the bones at the left base of the skull. This case report emphasizes the importance of recognizing the lesion by frozen-section examination because excellent results can be obtained by intraoperative curettage of the calcareous deposits and local irrigation with saline solution.
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7/17. Autosomal dominant early childhood seizures associated with chondrocalcinosis and a mutation in the ANKH Gene.

    We describe the pattern of early childhood seizures within a family with autosomal dominant chondrocalcinosis (CCAL, which causes adult-onset arthritis). All affected family members with CCAL experienced seizures in early childhood, usually, but not always, associated with fever. Similarities exist to the syndrome of generalized epilepsy with febrile seizures plus (GEFS ). A mutation within the ANKH gene on chromosome 5p has been found previously in this family; other patients with familial CCAL (but without seizures) have mutations in the same gene. ANKH codes for a transmembrane protein involved in the regulation of extracellular pyrophosphate ion levels, although its precise mechanism of action remains unclear. It is highly expressed in the brain, and its expression may be influenced by seizure activity. The mutation within this family creates a premature initiation codon, adding four amino acids to the N-terminus of the protein. We postulate that this may lead to a gain of function, causing seizure susceptibility as well as chondrocalcinosis. Mutations within this gene may underlie other forms of genetic epilepsy and febrile seizures.
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8/17. Tumoral calcium pyrophosphate deposition disease.

    A report of two patients in which a soft tissue mass, initially regarded as a malignant tumor, was shown to be the result of calcium pyrophosphate deposition disease. The first case, a woman aged 71 years, presented with a mass involving the right fifth finger. In the second case, also a woman aged 71 years, the lesion involved the tissues adjacent to the right hip. Each lesion consisted of a mass of highly cellular tissue containing deposits of calcium pyrophosphate dihydrate crystals. The clinical, radiological, and pathological features of the two cases are compared with those of seven similar cases reported in the literature.
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9/17. Pseudogout presenting with low synovial fluid glucose: identification of crystals by gram stain.

    A man developed acute monoarticular ankle arthritis caused by calcium pyrophosphate dihydrate (CPPD) crystals. The clinical syndrome resembled that of a pyogenic arthritis. synovial fluid analysis revealed a glucose concentration of 13 mg/dL and 99,000 white blood cells/mm3. Only one other report of an extremely low synovial fluid glucose associated with pseudogout could be found. The diagnosis of pseudogout was initially suggested when rhomboidal forms were seen during synovial fluid Gram stain examination. synovial fluid examination with polarized microscopy was initially negative, but revealed numerous CPPD crystals when repeated on the third hospital day. This case serves to illustrate how pseudogout can mimic pyogenic arthritis in both clinical presentation and low synovial fluid glucose concentration. The examination of Gram-stained synovial fluid can reveal the rhomboidal forms of CPPD crystals. The appearance of these crystals is documented in this report.
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10/17. Clinical features of pseudogout attack. A survey of 50 cases.

    Fifty patients (15 men and 35 women; average age, 70.8 years) with pseudogout were studied. Arthroscopic biopsy was useful for diagnosis in one case with no light microscopic evidence of crystals in the synovial fluid. Attacks affected 93 sites in 50 patients. Attacks in ten patients occurred after mild trauma, long distance walks, total knee arthroplasty, or intraarticular injection of microcrystalline glucocorticoid. Twenty-five patients had fevers averaging 38 degrees. In five patients the fever was associated with mental confusion. Fourteen patients were initially suspected or misdiagnosed as having septic arthritis. Six patients were treated surgically under this misdiagnosis, with synovectomy complicated by limitation of motion of both knees in one patient. The involvement of more than one joint was noted in 21 patients. Polyarthritis could be an important clue to the diagnosis performed early; the combination of arthrocentesis and injection of microcrystalline glucocorticoid provided excellent therapy.
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