Sweet's syndrome and mediastinal lymphadenopathy due to sarcoidosis: three cases of a new association.
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Biomedical subjects
Publications and source records attributed to J Pouchot.
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PURPOSE: Ossification of the posterior longitudinal ligament (OPLL) is a common, well-recognized cause of spinal stenosis and myelopathy in Japan. Although also common in whites, especially among the elderly, it has received little scientific attention. We wish to increase awareness of this important cause of myelopathy, and to determine if the clinical characteristics of OPLL are similar in non-Japanese and Japanese patients. PATIENTS AND METHODS: The clinical and radiologic features of eight cases of OPLL are presented. These cases combined with 73 non-Japanese cases gathered from the English literature are contrasted with 2,125 Japanese cases of OPLL. RESULTS: Similarities among non-Japanese and Japanese cases included: (1) male predominance; (2) peak age at onset of symptoms in the sixth decade; (3) clinical presentation, which ranged from asymptomatic to quadriplegia, with progressive or acute onset of neurologic deterioration; (4) greater than 95% localization to the cervical spine, spastic quadriparesis being the most common neurologic presentation; (5) an association with several rheumatic conditions including diffuse idiopathic skeletal hyperostosis (DISH), spondylosis, and ankylosing spondylitis; and (6) neurologic improvement with either conservative or surgical treatment in a significant proportion of patients. Differences between the two groups were minimal and included a higher mean age at onset (although onset in both groups occurred within the sixth decade) and a greater proportion of patients with DISH and with the continuous type of OPLL in the non-Japanese group. CONCLUSION: The clinical characteristics of OPLL are similar in Japanese and non-Japanese patient populations. Increased awareness of this condition, which has potentially devastating neurologic complications, will favorably influence diagnosis, treatment, and outcome.
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Clinical and laboratory manifestations, disease course, outcome, and HLA associations were studied in an inception cohort of 62 subjects with adult Still's disease (ASD) from 5 Canadian universities. Twenty-eight patients (45%) were female and the median age at disease onset was 24 years. In general, the clinical features observed in our patients were identical to those in other published series. However, significantly higher frequencies of sore throat (92%), weight loss (76%), lymphadenopathy (74%), pleuritis (53%), pneumonitis (27%), and abdominal pain (48%) were noted in our patients compared to those in a recent literature review. Liver involvement with hepatomegaly (44%) or abnormal liver function tests (LFTs) (76%) was common and was responsible for the 2 deaths attributed to Still's disease in our series. Severe liver failure always occurred in conjunction with aspirin or NSAID therapy. Therefore, whether or not aspirin or other NSAIDs are used, we recommend close monitoring of LFTs in patients with ASD, especially early in the disease course. Laboratory manifestations were similar to those already reported. Leukocytosis (greater than or equal to 15,000/mm3) was present in 50 patients (81%), a normochromic, normocytic anemia (hemoglobin less than or equal to 10 g/dl) in 42 (68%), and an elevated ESR in all. The mean follow-up of the 62 patients was 70 months (range, 2-163). Twenty-one patients (34%) had a self-limited disease course, 15 (24%) an intermittent course, and 22 (36%) a chronic disease course. Four patients (6%) died, and 2 of these deaths were attributed to Still's disease. For those patients who experienced a recurrence of ASD, the flares were usually of shorter duration and milder in severity than the initial episode. No initiating factor for disease exacerbation was identified in our patients. Although 22 of 62 patients (36%) had a chronic disease course, 52 (90%) were in ARA Functional Class I, and only 4 and 2 patients were in ARA Functional Class II and III, respectively. Patients with Still's disease had higher scores than the controls on the Pain (P less than 0.01) and Physical Disability (P less than 0.05) subscales of Arthritis Impact Measurement Scales health status questionnaire. Joint radiographs performed at the follow-up evaluation disclosed typical carpometacarpal and intercarpal involvement in 16 of 39 patients. In our series, HLA-B17, B18, B35, and DR2 were significantly associated with ASD. Three significant predictors of an unfavorable outcome, either a chronic disease course or a longer time to clinical remission, were identified.(ABSTRACT TRUNCATED AT 400 WORDS)
Increases in body temperature may be due to hyperthermia, i.e. imbalance between production and elimination of heat, or to fever, i.e. disregulation of the central thermostat by a humoral mediator now identified as interleukin I. The same mediator is involved in the production by the liver of inflammation proteins which, besides other actions, encourage the formation of rouleaux and produce an increase in erythrocyte sedimentation rate. However, it is not exceptional to find a rise in temperature associated with a normal erythrocyte sedimentation rate. When this situation does not reflect a technical error, it may guide the diagnostic approach of a febrile state and may suggest the existence of a non-inflammatory hyperthermia or of a fever associated with "paradoxical" normalisation of the erythrocyte sedimentation rate. The 1st hypothesis raises the possibility of a series of diagnoses ranging from fever of central origin to hyperthyroidism and drug-induced fever. The 2nd hypothesis leads to a search for plasma factors (low plasma fibrinogen level, hyperviscosity, cryoglobulin) or erythrocytic factors (polycythaemia, haemoglobinopathy) which inhibits the joining of red cells and artificially lower the erythrocyte sedimentation rate.
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The Arthritis Impact Measurement Scales (AIMS) questionnaire was administered to 57 patients with adult Still's disease (ASD) and 104 controls. Six of the 9 scales were consistent for both groups (Cronbach's alpha greater than or equal to 0.70). Two scales, Mobility and Dexterity, were consistent for ASD only (alpha greater than or equal to 0.70). The reliability of a French version of the AIMS was similar to the standard AIMS. Of the 13 associations evaluating criterion related validity of the AIMS in ASD, 11 were statistically significant. We conclude that the French and standard AIMS are comparable and that this questionnaire is reliable and valid for assessing outcome in ASD.
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Sacroiliac joint (SIJ) involvement has been reported in up to 9.7 percent of patients with skeletal tuberculosis. Lack of awareness of this now uncommon form of infection often leads to diagnostic delay and increased morbidity. Eleven consecutive cases of SIJ tuberculosis are reported; clinical and radiologic features, diagnosis, treatment, and outcome are discussed. Buttock pain was the presenting complaint in all patients. However, radicular pain in the lower back (seven patients) or lower limb (10 patients) was common and in one patient precipitated an unnecessary surgical intervention. SIJ tuberculosis is frequently an isolated phenomenon. Therefore, direct sampling of the SIJ is necessary to establish the diagnosis. The recently described technique of closed needle biopsy of the SIJ was employed in all 11 patients and established the diagnosis in nine of the 11.
The association of ossification of the posterior longitudinal ligament (OPLL) and diffuse idiopathic skeletal hyperostosis (DISH) has been recently described. It may result in devastating compressive myelopathy. We report a case of quadriplegia complicating OPLL in a patient with DISH. In addition, we present a brief review of the literature on OPLL. This report illustrates the importance of appropriate neurologic and radiologic evaluation of persons with DISH, to help prevent severe neurologic complications.
We are reporting 4 cases of unnoticed sacral fractures in osteoporotic women. Their mean age was 73 years. Only in one case the fracture was seen on a standard X-Ray. On the other hand, hyperfixation with isotopes on the sacrum formed a very characteristic H-shaped image. In two of these patients, the sacral fracture was confirmed by tomodensitometry, but this examination was unavailing in the third patient. In the 4th case, hyperfixation of the isotope was not significant. Only a guided bone biopsy and the favorable evolution of the disease enabled the diagnosis of sacral fracture.