Orthostatic hypotension as an unusual presentation of spinal calcium pyrophosphate deposition disease: case report and review of literature.

Calcium pyrophosphate crystal deposition disease (CPPD), also known as pseudogout, with spinal involvement, is associated with clinical manifestations of acute nerve compression or chronic spinal stenosis. Precipitation of crystals of calcium pyrophosphate dihydrate in connective tissues can lead to acute inflammatory arthritis, degenerative chronic arthropathies, and radiographic evidence of cartilage calcification. We present a case of an 87-year-old woman, with unstudied chronic polyarthralgia and symptomatic orthostatic hypotension. It were documented acute calcium pyrophosphate deposition wrist arthritis, and cervical CT and MRI was suggestive of spinal involvement of CPPD. Workup excluded other causes of OH. Surgical approach could be indicated to minimize the symptoms, but it was contra-indicated due to the patient's performance status, so histological diagnosis was not possible. Muscle atrophy played an important part in the rapid progression of this insidious chronic disease. Conservative and symptomatic treatment achieve scarce short-term clinical improvement. Spinal involvement of CPPD was thought to be rare but recent studies show a higher prevalence than expected. We call for attention to the extent of structural changes that may occur when not early diagnosed nor treated. High clinical suspicion is required and this is, to our knowledge, the first report of orthostatic hypotension as a presentation of CPPD.

Pseudogout growing from the temporomandibular joint into the middle cranial fossa.

Calcium pyrophosphate dihydrate deposition disease (CPDD or pseudogout) is a degenerative joint disease. It is defined by the presence of calcium pyrophosphate dihydrate crystals. It usually manifests in the knee and wrist. Manifestation in the temporomandibular joint (TMJ) is only reported in case reports. We present a patient with CPDD mimicking a malignant tumor of the TMJ. A 53-year-old woman presented with progressive pain and a slow-growing swelling of the left TMJ. Imaging showed an extensive mass in the infratemporal fossa extending into the middle cranial fossa and compressing the temporal lobe. Assuming a potential malignancy, we excised the growth, which extended into the dura. We covered the resulting tissue defect within the primary surgery using a microsurgically anastomosed scapular flap and performed further reconstructive surgeries. Calcium pyrophosphate dihydrate crystals were found in the histopathologic examination of the excised tissue, resulting in the diagnosis of CPDD. That is a benign diagnosis, but we treated it like a malignancy. This leads us to the question, was there overtreatment? Tumoral CPDD in the TMJ can be a difficult diagnosis to obtain. The treatment remains controversial, but complete excision of the mass was performed in most reported cases.

Magnetic resonance imaging of rheumatological diseases.

Magnetic resonance imaging (MRI) is extremely useful in the early diagnosis of rheumatologic diseases, as well as in the monitoring of treatment response and disease progression to optimize long-term clinical outcomes. MRI is highly sensitive and specific in detecting the common findings in rheumatologic diseases, such as bone marrow oedema, cartilage disruption, articular erosions, joint effusions, bursal effusions, tendon sheath effusions, and synovitis. This imaging modality can demonstrate structural changes of cartilage and bone destruction years earlier than radiographs. Rheumatoid arthritis, crystal deposition diseases (including gouty arthropathy and calcium pyrophosphate deposition disease), seronegative spondyloarthropathies (including psoriatic arthritis, reactive arthritis, ankylosing spondylitis), and osteoarthritis have characteristic appearances on MRI. Contrast-enhanced MRI and diffusion-weighted imaging can provide additional evaluation of active synovitis. This article describes the MRI findings of normal joints, as well as the pathophysiological mechanisms and typical MRI findings of rheumatoid arthritis, gouty arthritis, calcium pyrophosphate deposition disease, psoriatic arthritis, reactive arthritis, ankylosing spondylitis, and osteoarthritis.

Chemical Diagnosis of Calcium Pyrophosphate Deposition Disease of the Temporomandibular Joint: A Case Report.

Calcium pyrophosphate dihydrate (CPPD) deposition disease is a benign disorder characterized by acute gouty arthritis-like attacks and first reported by McCarty. CPPD deposition disease rarely occurs in the temporomandibular joint (TMJ), and although confirmation of positive birefringence by polarized light microscopy is important for diagnosis, it is not reliable because other crystals also show birefringence. We reported a case of CPPD deposition disease of the TMJ that was diagnosed by chemical analysis. A 47-year-old man with a chief complaint of persistent pain in the right TMJ and trismus was referred to our department in 2020. Radiographic examination revealed destruction of the head of the mandibular condyle and cranial base with a neoplastic lesion involving calcification tissue. We suspected CPPD deposition disease and performed enucleation of the white, chalky masses. Histopathologically, we confirmed crystal deposition with weak birefringence. SEM/EDS revealed that the light emitting parts of Ca and P corresponded with the bright part of the SEM image. Through X-ray diffraction, almost all peaks were confirmed to be CPPD-derived. Inductively coupled plasma atomic emission spectroscopy revealed a Ca/P ratio of nearly 1. These chemical analyses further support the histological diagnosis of CPPD deposition disease.

Symptomatic retro-odontoid pseudotumor causing calcium pyrophosphate dihydrate deposition combined with multilevel cervical spondylotic myelopathy.

INTRODUCTION: Symptomatic retro-odontoid pseudotumor (ROP) caused by cervical compression and myelopathy is rare. Pathological diagnosis is recommended for differential diagnosis including the following: inflammatory disease, primary bone tumor, metastatic disease and calcium pyrophosphate dihydrate deposition (CPPD) also known as "crowned dens syndrome". The authors report a rare case of ROP caused by CPPD deposition combined with multilevel cervical spondylotic myelopathy (MCSM) which was treated by tumor resectioning using a transoral approach combined with posterior decompression and fusion. CASE PRESENTATION: A 66-year-old male presented with progressive neck pain and spastic gait with no history of trauma. Radiographic imaging revealed degenerative change involving the atlanto-axial and atlanto-occipital joints with calcified enhancing soft tissue around the odontoid process causing cord compression and cervical instability at the C1-C2 level combined with MCSM and spinal cord compression at C3 to C7. Microscopic assisted transoral tumor resection combined with posterior decompression and fusion was performed at the occiput to T2. The pathology report describes a rhomboid-shaped crystal caused by calcium pyrophosphate dihydrate deposition (CPPD) disease. At the 6-month follow-up following the operation, the patient's neck pain and spastic gait were improved compared to the preoperative examination. DISCUSSION: Cervical compression and myelopathy from ROP causing CPPD combined with MCSM is rare. Pathology diagnosis and surgical management are highly recommended. CONCLUSION: In this case, a combined surgical approach: tumor resection using a transoral approach and a posterior approach for decompression and fusion at occiput to T2 was an effective option for this condition.

Calcium pyrophosphate dihydrate deposition disease in the temporomandibular joint: diagnosis and treatment.

BACKGROUND: Calcium pyrophosphate dihydrate deposition disease (CPDD) is a rare disease in the temporomandibular joint (TMJ) space. It forms a calcified crystal mass and induces a limitation of joint movement. CASE PRESENTATION: The calcified mass in our case was occupied in the left TMJ area and extended to the infratemporal and middle cranial fossa. For a complete excision of this mass, we performed a vertical ramus osteotomy and resected the mass around the mandibular condyle. The calcified mass in the infratemporal fossa was carefully excised, and the segmented mandible was anatomically repositioned. Scanning electronic microscopy (SEM)/energy-dispersive X-ray spectroscopy (EDS) microanalysis was performed to evaluate the calcified mass. The result of SEM/EDS showed that the crystal mass was completely composed of calcium pyrophosphate dihydrate. This result strongly suggested that the calcified mass was CPDD in the TMJ area. CONCLUSIONS: CPDD in the TMJ is a rare disease and is difficult to differentially diagnose from other neoplasms. A histological examination and quantitative microanalysis are required to confirm the diagnosis. In our patient, CPDD in the TMJ was successfully removed via the extracorporeal approach. SEM/EDS microanalysis was used for the differential diagnosis.

Calcium pyrophosphate dihydrate deposition disease in the temporomandibular joint: diagnosis and treatment / 대한악안면성형재건외과학회지

BACKGROUND: Calcium pyrophosphate dihydrate deposition disease (CPDD) is a rare disease in the temporomandibular joint (TMJ) space. It forms a calcified crystal mass and induces a limitation of joint movement. CASE PRESENTATION: The calcified mass in our case was occupied in the left TMJ area and extended to the infratemporal and middle cranial fossa. For a complete excision of this mass, we performed a vertical ramus osteotomy and resected the mass around the mandibular condyle. The calcified mass in the infratemporal fossa was carefully excised, and the segmented mandible was anatomically repositioned. Scanning electronic microscopy (SEM)/energy-dispersive X-ray spectroscopy (EDS) microanalysis was performed to evaluate the calcified mass. The result of SEM/EDS showed that the crystal mass was completely composed of calcium pyrophosphate dihydrate. This result strongly suggested that the calcified mass was CPDD in the TMJ area. CONCLUSIONS: CPDD in the TMJ is a rare disease and is difficult to differentially diagnose from other neoplasms. A histological examination and quantitative microanalysis are required to confirm the diagnosis. In our patient, CPDD in the TMJ was successfully removed via the extracorporeal approach. SEM/EDS microanalysis was used for the differential diagnosis.

[Tooth-pick? Picking the Right Tooth]. / Manchmal muss man einen Zahn zulegen.

We report about an 80-year-old patient, who underwent the extraction of an upper molar tooth because of facial pain. In the course of time the patient developed a maxillary sinusitis in presence of an ectopic tooth. Given that the patient got fever, neck pain and -stiffness, a purulent meningitis was first suspected. The liquor analysis was normal and the CT-scan showed a calcification around the dens axis. We finally diagnosed a "Crowned Dens"-syndrome.

A Case of Acute Calcium Pyrophosphate Crystal Arthritis in the Lumbar Facet Joint

Calcium pyrophosphate dihydrate deposition disease most commonly presents with acute arthritis of the peripheral joints. Infrequently, a mass effect of this disease can cause axial symptoms, such as spinal stenosis, radiculopathy, or myelopathy. Herein, we report on the first Korean case of acute arthritis in the lumbar facet joint due to calcium pyrophosphate dihydrate crystal deposition disease. A 73-year-old female presented with acute fever, severe lumbago, and knee arthralgia, 11 days after partial parathyroidectomy. Plain radiographs showed multiple chondrocalcinosis, while a bone scan, computed tomography, and magnetic resonance imaging showed right L5-S1 facet arthritis. In synovial fluid from the facet and knee joints, positively birefringent calcium pyrophosphate dihydrate crystals were observed under polarized light microscopy. Under the diagnosis of acute calcium pyrophosphate dihydrate crystal arthritis (formerly known as 'pseudogout') in the facet joint, an intra-articular triamcinolone injection was administered, which resulted in dramatic improvement of the symptoms within 24 hours.

Ultrasonographic Assessment in Crystal-induced Arthritis / 대한내과학회지

The latest recommendations for the diagnosis and management of crystal-induced arthritis, such as gout and calcium pyrophosphate dihydrate (CPPD) deposition disease, recognize the diagnostic potential of musculoskeletal ultrasonography (MSUS). MSUS allows rapid, highly sensitive, non-invasive detection of microcrystal aggregates in multiple anatomic areas, and can be used as a safe, reliable guide for aspiration of articular and periarticular specimens suitable for microscopic analysis. MSUS can also be used to monitor disease after treatment. Ultrasonographic differentiation between gout and CPPD deposition disease is based on the characteristics of crystal aggregates and their preferential localization in different anatomical areas. This rapid assessment may profoundly affect the clinical process, avoiding expensive, time-consuming diagnostic procedures. This article reviews the current status of and recent advances in MSUS imaging in crystal-induced arthritis.

Calcium pyrophosphate crystal deposition disease: diagnosis and treatment.

Calcium pyrophosphate dihydrate crystal deposition disease (CPPD) is an inflammatory arthritis produced by the deposition of calcium pyrophosphate (CPP) crystals in the synovium and periarticular soft tissues. It is the third most common inflammatory arthritis. Diagnosis is suspected on the basis of the clinical picture and radiographic/laboratory findings. The reference standard for the diagnosis of CPPD is based on the identification of CPP crystals in synovial fluid by light microscopy, compensated polarized light microscopy, or phase contrast microscopy. Most treatment approaches for CPPD are based upon clinical experience and not upon controlled trials. They range - depending on the subtype and the characteristics of symptoms - from no treatment to interleukin-1 blockade antibodies or specific therapy for an underlying disease. This review summarizes all we know so far about the diagnosis and management of CPPD.

Axial calcium pyrophosphate dihydrate deposition disease revealed by recurrent sterile spondylodiscitis and epidural abscess.

Spondylodiscitis are frequent and clinical challenge for practionners. Axial calcium pyrophosphate dihydrate deposition disease (CPDD) is well known for cervical spine involvement with the crowned dens syndrome but other localisations are probably underdiagnosed in sterile spondylodiscitis. We report a case of recurrent sterile spondylodiscitis with epidural abscess related to CPDD proved by vertebral percutaneous needle biopsy with rapid favourable course under colchicine therapy. Axial CPDD could mimic septic spondylodiscitis with epidural abscess on MRI. Sterile spondylodiscitis are probably underdiagnosed forms of microcrystalline disease. Investigations of the presence of microcrystals should be systematically undertaken with tamponed formalin fixed biopsies. If axial CPDD is suspected, colchicine therapy could be a good therapeutic test and would avoid unnecessary antibiotic treatment.

Tophaceous pseudogout in a patient undergoing peritoneal dialysis.

Calcium pyrophosphate dihydrate deposition disease (pseudogout) is a crystal arthritis characterized by pyrophosphate dihydrate crystal deposition in the articular cartilage or synovium. In chronic kidney disease patients, the major causes of crystal arthritis are calcium oxalate crystals and basic calcium phosphate crystals. However, pseudogout is apparently infrequent among such patients. This report describes a rare case of tophaceous pseudogout (tumoral form of pseudogout) involving the hip joint in a peritoneal dialysis patient, as revealed by CT scan and synovial fluid aspiration. Fluid aspiration and oral NSAIDs improved the clinical symptoms. This report is the first to describe tophaceous pseudogout in a peritoneal dialysis patient, which suggests that pseudogout should be suspected in dialysis patients with unexplained arthritis.

A Case of Calcium Pyrophosphate Dihydrate Deposition Disease Associated with Primary Hyperparathyroidism

Calcium pyrophosphate dihydrate (CPPD) deposition disease is a heterogeneous group of diseases with CPPD crystal deposition. Aging is the most common risk factor for CPPD deposition, followed by osteoarthritis and previous injury. Occasionally, CPPD depositions are associated with familial predisposition and metabolic diseases, including hemochromatosis, primary hyperparathyroidism, hypophosphatasia, and hypomagnesemia. CPPD deposition diseases associated with primary hyperparathyroidism in Koreans have rarely been reported. Thus, we report a case of a relatively young female patient with CPPD deposition disease associated with primary hyperparathyroidism, which was diagnosed through a polarized microscopic examination of the synovial fluid and a subtotal parathyroidectomy.

CPPD crowned dens syndrome with clivus destruction: a case report.

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.

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, shoul-ders, 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.