SAPHO syndrome of the temporomandibular joint associated with sudden deafness.

We report a case of arthritis of the temporomandibular joint (TMJ) associated with sclerosing osteomyelitis of the mandible and temporal bone, causing deafness. The presence of a palmoplantar pustulosis established the diagnosis of SAPHO syndrome. SAPHO (an acronym referring to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis) syndrome is defined by the association of characteristic osteoarticular and dermatologic manifestations, with diffuse sclerosing osteomyelitis of the mandible being a part of this entity. We review the literature of SAPHO syndrome with mandibular manifestations and discuss the mechanisms of inflammatory spread from the TMJ to the cochlea. To our knowledge, this is the first description of skull base involvement in a patient with SAPHO syndrome leading to sudden deafness.

Atlantoaxial fusion and retrodental pannus in rheumatoid arthritis.

STUDY DESIGN: This study analyzed the influence of atlantoaxial fusion in rheumatoid arthritis patients on inflammatory retrodental pannus. OBJECTIVES: To determine the value of fusion on the magnitude of pannus as a compressive structure on the spinal cord. SUMMARY OF BACKGROUND DATA: Transverse and vertical instability may lead to neurologic deficits from spinal cord compression. Increased size of the retrodental pannus can exacerbate the neurologic deterioration. Anterior removal of dens and pannus followed by posterior fusion has been proposed in such situations as a method to relieve spinal cord compression. METHODS: Twenty-two patients with atlantoaxial instability and verified pannus on magnetic resonance imaging underwent posterior fusion of the upper cervical spine. These patients were followed 12 to 75 months after surgery by clinical, radiologic, and magnetic resonance imaging evaluations. The size of the pannus was compared before and after surgery. RESULTS: In all patients, the retrodental pannus had significantly decreased or disappeared postoperatively. CONCLUSIONS: Pannus reduction occurred even in patients whose disease was active or progressing, supporting the hypothesis that the pannus is more a reactive fibrous tissue resulting from instability rather than a direct consequence of the inflammatory process itself.

Foraminal changes with distraction and compression of the L4/5 and L5/S1 segments.

The width of the foramen in the lumbar spine is directly related to the position of the vertebrae. In an MRI study the measurements of the cross-sectional area of the neuroforamen of L4/5 and L5/S1 in neutral position, segmental distraction and compression were calculated. Nine cadaver specimens were investigated and the foraminal width of L4/5 and L5/S1 was measured. In both segments of all specimens the foraminal space significantly enlarged under distraction and decreased under compression. In the L4/5 segment the average relative difference between distraction and compression was 27%.

[Radiological assessment of degenerative lumbar stenosis]. / Die radiologische Abklärung der degenerativen lumbalen Stenose.

The evaluation of central and lateral lumbar spinal stenosis is equally well performed by CT, CT-myelography and MRI. Stenosis can be quantified and localized in order to optimize orthopedic treatment. Each of these modalities has inherent strengths and weaknesses. CT-myelography is still the best modality for evaluation of central spinal stenosis because absolute stenosis can be more precisely quantified than by plain CT and MRI. Myelography can image the entire spine and localize stenosis that is induced by functional positions. Lateral lumbar spinal stenosis can be localized at the lateral recessus or more distally at the neural foramina. Stenosis of the lateral recessus occurs only at the lower levels of the lumbar spine. Rarely stenosis at the entry of the lateral recessus can be missed by MRI and is only depicted by thin section (1-2 mm) CT. The anatomy and therefore stenosis of the neural foramina are exquisitely demonstrated by direct sagittal MRI.

Case report 726. Enchondromatosis of the left femur and hemipelvis (Ollier's disease).

A case of Ollier's disease is demonstrated. Large osteolytic lesions in the left femur and hemipelvis visible on the roentgenograms represent enchondromas. On CT and MRI studies numerous fine translucencies are demonstrated in the surrounding bone, corresponding to multiple cartilaginous foci. These findings may be considered pathognomonic for enchondromatosis (Ollier's disease). In addition, the nature of Ollier's disease and its predilection for malignant transformation are considered. The major differential diagnostic entity in this case was probably fibrous dysplasia.

Elevated muscle tissue oxygen tension in short-term insulin-dependent diabetes mellitus.

In order to assess hemodynamic changes in early phases of insulin-dependent diabetes mellitus, muscle tissue oxygen tensions were determined in 7 newly diagnosed insulin-dependent diabetic patients and in 6 healthy control subjects matched for sex, age and weight. Muscle tissue oxygen tension was measured by polarrographic needle probes while breathing ambient air and oxygen enriched air with an oxygen concentration of 40 per cent as well. Three times during the experiment, heart rates, blood pressures, blood glucose concentrations, and capillary oxygen tensions were obtained. Heart rate, blood pressure, peripheral and autonomic nerve functions did not differ between the 2 groups studied. While breathing ambient air, muscle tissue oxygen tensions were significantly (p = 0.025) higher in the diabetic patients than in the control subjects. In contrast to the control subjects, diabetic patients did not increase muscle tissue oxygen tensions after inhalation of oxygen enriched air despite of an equal increase in capillary oxygen tensions (2p less than 0.05). Local tissue hypoxia does not seem to be a leading cause of hemodynamic abnormalities in the early phases of diabetes.

Functional evaluation of the spinal cord by magnetic resonance imaging in patients with rheumatoid arthritis and instability of upper cervical spine.

Thirty-four patients with atlanto-axial instability due to rheumatoid arthritis were examined with plain x-ray views and functional magnetic resonance imaging (MR), and were neurologically evaluated. Transcranial brain stimulation was performed in 25 patients. In 22 cases, the authors observed inflammatory tissue thicker than 3 mm behind the odontoid peg. The spinal canal diameter was significantly decreased in the flexed position. Nine patients showed signs of cranial migration of the axis. The diameter of the spinal cord was measured to be 7.4 mm in the neutral position, and 6.5 mm in flexion. The difference between the diameter of the neutral and flexed positions was highly significant. Twelve of the 34 patients displayed clinical signs of cervical myelopathy, and 13 showed a significant delay of central motor latency, as calculated from the motor evoked potentials. Surgical intervention, either by a posterior approach only or combined with a transoral dens and inflammatory tissue resection, is recommended in patients with progressive atlanto-axial instability, pathologic clinical and neurophysiologic findings, and a spinal cord diameter of less than 6 mm in flexion. Severe pain and cranial migration of the axis, as measured by the MRI, also justify a surgical intervention.

Muscle PO2 in the initial phase of increased local oxygen demand (rhythmical muscle contraction) in rats with portacaval shunt (PCA).

The influence of a hyperdynamic syndrome caused by PCA on PO2 distribution in skeletal muscle of rats during the initial phase of muscle activity was examined. Rhythmical muscle contraction of the m. biceps femoris was induced by direct electrical stimulation. Tissue PO2 of the contracting muscle was recorded continuously from the start of the 210 s-long activity period up to 140 s after the last contraction using a multiwire surface electrode. In comparison with controls no different behaviour of mean muscle PO2 in the initial phase of contraction was found. After muscle activity mean PO2 decreased to a lower level in rats with PCA than in controls. This might be a further indication of the disturbing influence of a hyperdynamic syndrome on the regulating mechanisms of the microcirculation.

Circadian course of PO2-oscillations in skeletal muscle of intact and portacaval shunted (PCA) rats.

In rats several circadian rhythms such as heart rate, body temperature, and locomotor activity are known. Several authors found a loss of day-night-rhythm (locomotor activity, EEG) after portacaval shunting (PCA). The aim of this study was to evaluate whether muscle PO2 oscillations are circadian and whether they are altered after time-limited hypercirculation caused by PCA. 126 days after operation tissue PO2 of m. rectus abdominis of 9 rats with PCA and 10 controls was measured with a multi-wire surface electrode. All animals were kept under constant conditions and each animal was measured 6 times at intervals of 4 hours in order to get a circadian PO2 course. In controls the circadian course of mean muscle PO2 resembled a sine oscillation with high values at night and low values in the afternoon. In PCA-rats the time course of mean muscle PO2 showed 3 oscillations with different amplitudes, each with a period length of 24 hours. Our results indicate that oscillations of muscle PO2 are determined principal by circadian locomotor activity and that time-limited hypercirculation influences the circadian course of mean muscle PO2.

[Computed tomography of intra-articular calcaneal fractures]. / Computertomographie intraartikulärer Kalkaneusfrakturen.

The increasing number of operations on intraarticular calcaneal fractures created the need for a fast, reproducible method to examine the weight-bearing posterior talo-calcaneal joint. High resolution CT scanning in an almost coronal plane without reformating was done in 25 calcaneal fractures. Joint alignment and fragment dislocation were demonstrated more precisely than would have been possible with conventional studies. Three typical fractures were found: The lambda type, the y type and the comminution type. Follow-up studies showed osteo-arthritis, intraarticular degenerative changes of the articular surface and impingement of personeal tendons as possible causes of pain.

[Mucopolysaccharidosis IV-A (Maroteaux-Lamy disease, severe form): incipient compressive myelopathy, cerebrospinal fluid fistula and tracheal stenosis in an adult patient]. / Mukopolysaccharidose VI-A (Morbus Maroteaux-Lamy, schwere Form): beginnende kompressive Myelopathie, Liquorfistel und Trachealstenose bei einem erwachsenen Patienten.

A 25-year-old male with the severe form of Maroteaux-Lamy disease (mucopolysaccharidosis VI-A) developed rhinoliquorrhea of undetermined origin. The head was held permanently in extension, and there was both inspiratory and expiratory stridor. Flexion of the head worsened the stridor and caused dyspnea. Radiological examination showed a massive narrowing of the trachea and a marked retropharyngeal and retrotracheal swelling. Hyperreflexia and slight impairment of the sensation of the lower limbs were found on neurological examination. Computed tomography revealed hydrocephalus and extreme narrowing of the subarachnoid space in the region of the occipito-cervical junction, caused by marked epidural soft tissue thickening and a dysplastic arch of the atlas protruding dorsally into the foramen magnum, and displacement of the cervical spinal cord. At the age of 26 years rhinoliquorrhea suddenly stopped and the patient developed acute signs of occlusive hydrocephalus. Emergency ventriculo-peritoneal shunting was performed.

Quantification of body fat distribution in the abdomen using computed tomography.

A computed tomographic method was used to assess the pattern of abdominal fat distribution in normal males and females at different abdominal levels. The method permitted site specific calculations of total body volume (TA), total fat volume (TF), subcutaneous fat volume (SF), and intraabdominal fat volume (IF) in each computed tomography scan. The ratio of TF/TA, SF/TF and IF/TF were calculated for the L1, L3, and L5 vertebral levels. Regression analysis of IF versus SF, SF versus TF, IF versus TF, TF versus TA, and TF versus body mass index and age were calculated. A significant linear correlation between the measured variables TA, SF, IF, and TF and between TF and body mass index was found for virtually all correlations attempted at all scanned levels. Females had a higher total fat volume and greater percentage of subcutaneous fat at all levels. Males accumulated more fat intraabdominally than subcutaneously at the L1 and L3 levels. The male-female differences were greatest at L1 and the ratio SF/IF statistically significant at the L1 and L5 levels. Our results demonstrate that computed tomography can noninvasively quantify abdominal fat distribution at various sites. There is an inherent difference in abdominal fat distribution between males and females that is not related to weight. The distribution of body fat in males and females varies markedly from level to level.

Immunosuppressive serum factors in viral hepatitis. III. Prognostic relevance of rosette inhibitory factor and serum inhibition factor in acute and chronic hepatitis.

Two immunosuppressive serum factors, serum inhibition factor (SIF) and rosette inhibitory factor (RIF), were studied in sera from patients with acute and chronic viral hepatitis. In a study of 30 patients with acute viral hepatitis, an association was found between RIF, SIF, and biochemical and virological parameters in 27 patients (90%), 25 of whom recovered completely; two had a protracted course. In three patients, the clinical course was not reflected by the immunosuppressive factors. In 26 patients with chronic persistent hepatitis, 3 had RIF and 7 had SIF of low activity. In patients with HBsAg-positive and -negative chronic active hepatitis, 32 of 47 had RIF and 24 had SIF. SIF activity was significantly increased in HBsAg positive as compared to -negative cases. There was no correlation between RIF and SIF activity at any stage of viral hepatitis. Although SIF was demonstrated in patients with various infectious and other inflammatory diseases, RIF was infrequently detected in nonviral liver disorders, and was not present in any of the nonhepatic diseases tested. It was confirmed that RIF is associated with the beta-lipoprotein fraction. RIF was easily separated from SIF by density gradient ultracentrifugation. The evaluation of SIF and RIF may be helpful in determining the outcome of acute viral hepatitis. In chronic hepatitis, RIF was a better indicator of disease activity than was SIF. These clinical data support previous findings that SIF may be related to the immune response whereas RIF is associated with liver cell damage.

Measurements of local PO2 in the resting skeletal muscles of rats with portocaval anastomosis (PCA) under normoxic conditions.

Direct measurements of local oxygen pressure by means of platinum multiwire electrode were performed in rats to investigate the effect of an end-to-side portocaval shunt procedure on muscle tissue oxygenation. Compared with intact rats and/or sham operated animals, rats with portocaval anastomosis showed a significant increase of muscle tissue oxygenation with nearly bellshaped pO2 histograms. This considerable increase in tissue oxygenation might appear to be due to the well known PCA induced hyperdynamic cardiovascular state.