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PubMedDate not supplied

[New pathogenetic aspects of ankylosing spondylitis].

Ankylosing spondylitis is one of the oldest diseases in humans; however, it is still one of the most fascinating and mysterious in human pathology. The unusual combination of both fundamental pathological processes: inflammation and ossification (which are mostly independent in respect to time and place) is unique. Until 1973, ankylosing spondylitis did not attract much immunological research. After the detection of an association between HLA B27 and the disease, clinical and immunological research was stimulated. It was supposed that HLA B27 may be a pathogenic factor. Meanwhile, it has become well known that HLA B27 itself is not required for development of the disease; however, discovery of immunological cross-reactivity between HLA B27 and some Klebsiella antigens inspired pathogenic considerations. It is discussed that the structural similarities between enteric bacteria and HLA B27 induce autoantibodies, or that HLA B27 plays a role in antigen recognition. Possibly, HLA B27 may also act as a receptor for infectious agents and their products. Fascinating, but controversely discussed is the hypothesis that bacterial products modify the B27 molecule and, in this way, trigger the disease. All present theories about pathogenesis of ankylosing spondylitis are unsatisfactory, because many important questions cannot be answered. There are no explanations for the unusual affinity of possible pathogenic immune reactions to the spine and other organs, the induction of ossification, the merging of cartilage, or the development of sacroilitis. Especially, we do not know the important bridge (if one exists) between inflammation and ossification. The typical ossification of the spine is of dramatic consequence for the patient in respect to function and mobility.(ABSTRACT TRUNCATED AT 250 WORDS)

Autoantibodies↗
PubMed1990

Ossification of the cervical posterior longitudinal ligament with severe myelopathy and fatal outcome.

Ossification of the posterior longitudinal ligament is rare among Caucasians. We describe a new case with lethal outcome and we discuss the radiographic and pathologic findings. Posterior longitudinal ligament ossification is a benign disease in most cases, but it may cause severe myelopathy due to spinal stenosis. Diagnosis may be suspected or made on lateral views of the cervical spine and ascertained by CT.

Aged↗
PubMed2002

Elbow heterotopic ossification in head-trauma patients: diagnosis and treatment.

Heterotopic ossification is a disorder characterized histologically and radiographically by normal bone formation in soft tissues that normally have no ossification properties. In severe head-trauma patients, a high incidence of heterotopic ossification occurs. The diagnosis of this pathology in these patients often is difficult for residual neurological damage. The etiology of heterotopic ossification following head trauma is unknown. Similarities have been found between heterotopic ossification and myositis ossificans, a hereditary autosomal dominant disease.

Adolescent↗
PubMed1986

Development of heterotopic ossification around the hip. A long-term follow-up of patients who underwent surgery with two different types of endoprostheses.

Heterotopic ossification has been reported in many pathological situations, most important clinically as a sequel to hip arthroplasty and spinal trauma. The etiology of heterotopic ossification is yet not clear, but the disease is supposed to be connected with trauma. Heterotopic bone was found in 53% (1.2% with the severe form) of 623 patients operated on at the Orthopaedic Hospital of the Invalid Foundation, Helsinki, Finland; the operations included 849 arthroplasties. The rate of heterotopic ossification was higher after revision arthroplasty, following operation of the contralateral side, in men, and in primary coxarthrosis, and the incidence was higher with the Brunswik (metal-on-plastic) endoprosthesis than in the McKee-Farrar type (metal-on-metal). Heterotopic bone formation generally seemed to increase and to be more manifest during long-term observation.

Calcinosis↗
PubMed1998

Surgical outcome in occipital lobe epilepsy: implications for pathophysiology.

Medically refractory occipital lobe epilepsies are increasingly treated with surgery, but outcome and its relationship to etiology, pathological substrate, occipital lobe location, surgical approach, and electroclinical features have not been systematically investigated in a substantial group of patients. Thirty-five patients who underwent surgery for intractable occipital lobe seizures were retrospectively evaluated. Outcome and occipital lobe location were analyzed with respect to surgical procedure, pathology, clinical seizure characteristics, seizure onset and termination locations, and localization of interictal spikes. Most patients had developmental abnormalities (14) or tumors (13, all gliomas). Developmental abnormalities consisted of focal cortical dysplasia (5), heterotopia (2), hamartoma (3), cortical duplication (1), polymicrogyria (1), Sturge-Weber syndrome (1), and tuberous sclerosis (1). There was 1 patient with a vascular abnormality, 1 with chronic inflammatory changes, 4 with gliosis, 1 with cerebral ossification, and 1 with normal pathology. Developmental abnormalities had significantly worse outcome (45% excellent/good) than tumors (85% excellent/good). In the developmental group, low-grade focal cortical dysplasias had better outcome than heterotopia and hamartoma regardless of type of surgical procedure. Pathological groups did not significantly differ with respect to location within the occipital lobe (overall medial [50%] or lateral [38%]); clinical seizure characteristics referable to specific lobe (occipital [14%], temporal [34%], frontal [23%], more than one type [29%]); electroencephalographic localization (to occipital [17%], temporal [27%], or other/multifocal locations [56%]); or intracranial ictal onset or termination location. Electroclinical variables were also unrelated to the occipital lobe location of abnormality. Surgical outcome was not predicted by surgical approach (lesion excision with margins or lobectomy). The main pathological substrates of uncontrolled occipital lobe epilepsy are gliomas and developmental abnormalities. Whereas resection of occipital lobe tumors associated with chronic epilepsy produces nearly uniform seizure control, outcome after resection of occipital lobe developmental abnormalities is less uniform.

Adolescent↗
PubMed1989

Pathologic bone formation.

The literature on pathologic bone formation is reviewed first on the basis of clinical syndromes and second in relation to newer knowledge of the origin of the bone-forming cells and regulatory molecules. Pathologic bone formation can be categorized into three groups based on the initiating stimulus: trauma, tumors, and idiopathic causes. In the trauma category, the formation of ectopic bone is concerned with major and minor traumatic incidents, surgery, burns, and other causes. In the tumor category, direct and reactive pathologic bone formation is discussed with an emphasis on the different neoplasms capable of ectopic bone formation. The category of idiopathic causes involves the formation of pathologic bone following neurologic injury and in systemic ossification disorders. The origin of the bone-forming cells in all forms of pathologic bone has not been unequivocally determined. However, there is evidence suggesting that these cells may arise from osteogenic stromal elements. Potent bone formation growth-regulating factors have been recently identified, and these molecules must also participate in the formation of pathologic bone. Increased understanding of the processes that control pathologic bone formation will lead to better methods of preventing and treating disorders of ossification.

Bone Marrow Cells↗
PubMedDate not supplied

From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation.

Chondrosarcoma is a malignant tumor that produces cartilage matrix, and lesions that arise de novo are called primary. Primary chondrosarcoma is the third most common primary malignant tumor of bone, constituting 20%-27% of all primary malignant osseous neoplasms. There are numerous types of primary chondrosarcomas, including conventional intramedullary, clear cell, juxtacortical, myxoid, mesenchymal, extraskeletal, and dedifferentiated. The conventional intramedullary chondrosarcoma is the most frequent type, and it most commonly involves the long bones or pelvis in up to 65% of cases. Although the pathologic appearance varies with specific lesion type, chondrosarcomas grow with lobular type architecture, and these hyaline cartilage nodules demonstrate high water content and peripheral enchondral ossification. Imaging features directly reflect this pathologic appearance, and the various subtypes often show distinctive features. Radiographic findings often suggest the diagnosis of chondrosarcoma because of identification of typical "ring-and-arc" chondroid matrix mineralization (representing the enchondral ossification) and aggressive features of deep endosteal scalloping and soft-tissue extension. These latter features are usually best assessed, as is lesion staging, with computed tomography (CT) or magnetic resonance (MR) imaging. CT is optimal to detect the matrix mineralization, particularly when it is subtle or when the lesion is located in anatomically complex areas. Both CT and MR imaging depict the high water content of these lesions as low attenuation and very high signal intensity with T2-weighting, respectively. Understanding and recognizing the spectrum of appearances of the various types of primary chondrosarcoma allow improved patient assessment and are vital for optimal clinical management including diagnosis, biopsy, staging, treatment, and prognosis.

Academies and Institutes↗
PubMed1988

[Studies on induction mechanism of ossification of the posterior longitudinal ligament of the spine--especially on the cultured cells from the human spinal ligament].

To determine the mechanism of ossification of the posterior longitudinal ligament (OPLL) of the spine using cultured cells derived from the human spinal ligament, the ossification group and the non-ossification group were compared morphologically and biochemically. The results are as follows: 1) There is a difference in the characteristics of the two groups. The non-ossification group consists chiefly of fibroblast-like cells, while the ossification group consists chiefly of osteogenic un-differentiated mesenchymal cells. 2) In the ossification group, the posterior longitudinal ligament is composed of high osteogenic potential cells, with a resulting general tendency of systemic ossification. This systemic ligamentous ossification factor is a primary cause of OPLL. When localized anatomical stress and/or abnormalities of hormone and bone metabolism stimulate this ligament which is in a high osteogenic activity, this may result in initial ossification which may develop into the pathological OPLL.

Adult↗
PubMed1999

The neurologic complications of Paget's disease.

Paget's disease of bone is associated with involvement of the central and peripheral nervous system. The brain, spinal cord, cauda equina, spinal roots, and cranial nerves can be affected in Paget's disease due to their anatomic relationship to bone. Neurologic syndromes are uncommon but include headache, dementia, brain stem and cerebellar dysfunction, cranial neuropathies, myelopathy, cauda equina syndrome, and radiculopathies. The central complications result from pagetic involvement of the skull. Expansion of diseased bone can result in compression of cranial nerves as they exit their bony foramina. Softening of the skull leads to basilar invagination with compression of the brain stem, cerebellum, and lower cranial nerves. Brain stem compression can cause hydrocephalus. Rarely, there is direct compression of the brain from acute epidural hematoma or hypertrophy of the calvarium. Myelopathy, cauda equina syndrome, and radiculopathies most commonly result from hypertrophy of the spine with direct compression. Spinal stenosis can also result from ossification of extradural structures or pathologic fractures. Ischemia from vascular compression or a steal syndrome has also been described. Neurologic complications rarely occur due to sarcomatous transformation of pagetic bone. Magnetic resonance imaging (MRI), computerized tomography (CT)-myelography, and bone X-rays are helpful to localize the lesion and direct therapy. Treatment options include surgical decompression, ventricular shunt placement, and medical management with calcitonin and/or the bisphosphonates. The selection of treatment will vary depending upon the rate of progression and the severity of the neurologic deficit.

Humans↗
PubMedDate not supplied

[Diffuse idiopathic skeletal hyperostosis of the cervical spine in a patient with ankylosing spondylitis. Description of a personal case].

Diffuse idiopathic skeletal hyperostosis (DISH) is a well-described disorder of middle-aged people, with a unique spinal pathology characterized by calcification and ossification of the antero-lateral aspect of at least four contiguous vertebral bodies, with the sparing of intervertebral spaces and sacroiliac joints. DISH has rarely been reported associated with ankylosing spondylitis (AS), a chronic inflammatory articular disease most commonly involving the spine and sacroiliac joints. A 63-year-old man with clinical and radiological findings of DISH with associated AS is described here. The authors conclude that these two diseases may, albeit rarely, coexist.

Cervical Vertebrae↗
PubMed1983

[Pyogenic sacroiliac arthritis in children. Analysis and commentary apropos of 7 cases].

Seven cases of pyogenic arthritis of the sacro-iliac joint in children were observed between 1968 and 1981. Their analysis is discussed with the conclusions of the too much rare publications in the recent literature. Taking in to account the anatomic particularities the necessity of very precise roentgenographic technics in order to assess the sacro-iliac joint, especially in children because of incomplete ossification, the authors replace this pathology into the osteomyelitis in children. From the clinical aspects of the disease, with its typically localized pain, and its acute clinical and biological septicemic syndrome, the authors attempt to demonstrate that the classical idea of a delayed diagnosis, is not absolute. Even in this particular localization, an early "up to date" diagnosis is possible allowing rapid treatment with efficient antibiotherapy directed towards the responsible germ after their isolation (especially staphylococcus aureus). Indubitable progress has been gained with systematic radio-nuclide bone scanning in emergency.

Adolescent↗
PubMed1994

[Sequelae of septic arthritis of the hip in newborns].

The sequelae of arthritis of the hip in neonatal period have been analyzed in a series of 75 hips in 68 patients aged from 2 months to 14 years. The diagnosis was set on the basis of history, medical documentation, clinical and radiological examination with standard radiographs, arthrography and sonography included; sometimes as late as at surgery. In 15 joints with synovial type of septic arthritis pathological dislocation has been found. In 60 hips the inflammatory process involved proximal end of the femur. The sequelae strictly depended on localization of the pathology. On the basis of ossification disturbances, deformities and defects found within the head and neck on radiographs in 35 per cent of cases damaged epiphysis, physis and metaphysis were detected. In 23 per cent the neck was involved; in 42 per cent extensive damage to the head and neck was noted.

Adolescent↗
PubMed1998

Rare temporal bone pathology of the Singa calvaria from Sudan.

Evidence has recently accumulated that the Singa calvaria from Sudan probably dates from Oxygen Isotope Stage 6 (>130 ka). Morphological studies have indicated a mixture of archaic and more modern human traits, but such analyses are complicated by the possibility that the vault is pathologically deformed, although the exact etiology has not been established. Now computed tomography (CT) has revealed that the right temporal bone lacks the structures of the bony labyrinth. The most likely cause of this rare pathological condition appears to be labyrinthine ossification, in which newly deposited bone obliterates the inner ear spaces following an infectious disease or occlusion of the labyrinthine blood supply. A possible cause of vascular compromise could have been the presence of an expanding acoustic neuroma in the internal acoustic meatus, which is suggested by a significantly wider right meatus compared with the left side. Interestingly, labyrinthine ossification is also consistent with the controversial diagnosis that an anemia caused the characteristic diploic widening at the parietal bosses, because prime etiological factors of ossification are among the common complications of some of these blood diseases. CT examination of the vault and a review of the literature suggest that a blood disorder may well have caused the unusual parietal morphology. Given the nature of these pathological conditions, the Singa individual must have experienced a period of considerable disability. The morphological evidence from the normal bony labyrinth on the left side and from the CT evaluation of the vault is consistent with the interpretation of Singa as a late archaic hominid or an early representative of Homo sapiens drawn from a population which might be directly ancestral to modern humans.

Animals↗