Neurological features in overlap syndrome.

We report the frequency of neurological findings in 33 patients with overlap syndrome and describe 2 patients presenting unusual neurological involvement. Five of the 33 patients (15%) had one or more neurological findings. Unilateral trigeminal sensory neuropathy was presents, in 4 patients, in 3 of whom this was the only neurological feature. In the other patient it was associated with other manifestations of central as well as peripheral nervous system involvement, with an overall clinical pattern highly suggestive of a demyelinating disorder. The fifth patient, after an aseptic meningitis-like illness, developed a slowly progressive paraparesis, likewise pointing to a demyelinating disease. Our findings suggest that neurological manifestations, mainly trigeminal neuropathy, are frequent features in overlap syndrome; occasionally, central nervous system involvement mimicking a demyelinating disease may also be observed.

Lymphadenopathy secondary to silicone hand joint prostheses.

The occurrence of lymphadenopathies was investigated in 23 patients with diverse rheumatic conditions who had silastic prosthesis in joints of the hands, to determine whether these adenopathies were due to the presence of silicone particles. Five cases had clinically detectable lymph node enlargement and tissue samples were studied by light and scanning electron microscopy (SEM) and energy dispersive X-ray analysis (EDXA). In 3 out of the 5 cases foreign body granulomas were observed by light microscopy, SEM showing a highly irregular distribution of foreign body material with a peak for silicone by EDXA. Foreign body particle distribution closely correlated with silicone concentration. No granulomas were found in the two remaining patients with adenopathies who presented a non-specific reactive lymphadenitis. Our findings show that silicone lymphadenopathy is a more frequent complication (13%) of silastic arthroplasty than is usually recognized and therefore should be considered in the differential diagnosis of patients with lymph node enlargement who have previously received a silicone arthroplasty.

Evaluation of the measurement methods for protrusio acetabuli in normal children.

The normal values of the radiological lines most frequently used as references in the diagnosis of adult protrusio acetabuli were prospectively determined in 150 children (300 hips) without femoral pathology and aged between 2 and 15 years, mean age 8 years. The teardrop shape was modified according to the child's age, the "crossed" type predominating (48.7%). The mean centre edge (CE) angle fluctuated, with a median value of 31.2 degrees +/- 6.2 degrees, increasing with age and with slightly greater values in females; a large spread of CE angle values was observed within age groups. Acetabular ilioischial line values ranged from +7 to -5 mm, with a mean of +1.8 +/- 2 mm. This last method was the most suitable because it was not modified by changes in incidence of X-rays in radiological studies. With this method protrusio is diagnosed in children when the acetabular line is projected medially, provided that the ilioischial line is 1 or 3 mm or more for boys and girls, respectively. We conclude that the most trustworthy method available to evaluate PA in children should be "line crossing".

Clinical and serologic characteristics of patients with overlap syndrome: is mixed connective tissue disease a distinct clinical entity?

We discuss the clinical and serologic features of 27 patients with overlap syndrome followed prospectively by our group. The findings are similar to those of other reports, but we have drawn attention to the presence of peritendinous nodules in these patients and mentioned some peculiar neurologic manifestations. Rheumatoid arthritis was the most common diagnosis in our patients. The presence of high-titer antibodies against the nuclear ribonucleoprotein fraction of extractable nuclear antigen (nRNP) did not allow the identification of a particular subgroup. However, patients with this antibody tended to fulfill more criteria of more diseases than those without it. The findings lead us to conclude that antibodies to nRNP do not identify a particular subgroup within the overlap syndromes and that mixed connective tissue disease does not appear to be a distinct entity.

Radiologic review: the cervical spine in juvenile rheumatoid arthritis.

In our patients, zygapophyseal joint fusion was the most frequent and characteristic roentgenographic finding of cervical spine involvement in JRA. It was not associated with any subset of patients. Atlanto-dens interval greater than 4.5 mm was present in 20% of patients, but was not related to the development of neurologic manifestations. Mild enthesopathic-like changes around the upper cervical area were observed in some children. In patients with severe long standing disease and extensive zygapophyseal joint fusion, longitudinal ligament calcification was observed. Growth disturbances of the cervical spine were more frequently observed in patients with zygapophyseal joint fusion and earlier onset of disease.

Tarsal ankylosis in juvenile and adult onset rheumatoid arthritis.

It has been suggested that tarsal arthritis with ankylosis may be characteristic of late onset Still's disease. In our study 16 of 88 (18%) patients with juvenile rheumatoid arthritis (JRA) and 22 of 97 (23%) patients with adult RA had radiographic findings of tarsal joint ankylosis. In our patients, tarsal bony fusion was not related to type of disease onset, age of onset or sex; rather it was related to a longer duration of disease. Tarsal ankylosis should not be regarded as a special feature of Still's disease since it can also be seen in adult RA.

Spinal artery steal syndrome in Paget's disease of bone.

We report a case of vertebral Paget's disease with paraplegia and no evidence of spinal cord compression, in which the neurological condition improved dramatically after treatment with calcitonin. Spinal cord vascular steal syndrome is suggested as another mechanism responsible for this neurologic complication.

Carcinomatous arthritis of the elbow caused by metastatic breast carcinoma.

We report a case of persisting chronic monoarthritis of the elbow in a 54-year-old woman with previous breast surgery for carcinoma. An open biopsy of the elbow showed a chronic nonspecific synovitis and a poorly differentiated metastatic adenocarcinoma on the radial head. This is the first report of elbow arthritis due to metastatic breast carcinoma.

Piroxicam in juvenile rheumatoid arthritis.

There is little consistency in drug treatment of children with juvenile rheumatoid arthritis, in part because there are few controlled studies of the use of nonsteroidal anti-inflammatory drugs (NSAIDs) in this disease. To determine the safety and efficacy of piroxicam in children with juvenile rheumatoid arthritis, 26 patients ranging in age from three to 25 years were randomly assigned to treatment with piroxicam or naproxen. The number of painful and swollen joints decreased significantly (p less than 0.05) in the piroxicam group. The overall assessment of the investigators was that 67% of the patients in the piroxicam group showed clinical improvement, in contrast to 38% of the naproxen-treated patients. Side effects in the piroxicam group were mild and transient, and no patient was removed from the study because of side effects. The recurrence of a cutaneous rash necessitated the removal of one naproxen-treated patient from the study. Although the results should be interpreted with caution because of the small sample size, piroxicam appears to be more effective and better tolerated than naproxen in this study.

Registro electrocardiográfico continuo de 24 hs. en pacientes con espondilitis anquilopoyética durante la terapéutica con metilprednisolona en "pulso" / Continuous 24 hours electrocardiographic recording in patients with ankylosing spondylitis during treatment with methyl-prednisolone pulse

Recientemente se han comunicado casos de muerte súbita luego de la administración terapéutica de esteroides en "pulso". Se efectuó registro electrocardiográfico continuo 24 hs. antes, durante y 24 hs. después de cada una de tres administraciones de altas dosis de metilprednisolona por vía intravenosa efectuadas a 5 pacientes con espondilitis anquilopoyética grave y sin respuesta al tratamiento convencional. No se observaron arritmias supraventriculares o ventriculares, bradiarritmias, alteraciones de la conducción, ni cambios isquémicos. No se presentaron síntomas cardiovasculares, ni cambios en los controles clínicos en ningún caso. En todos los pacientes se produjo una elevación transitoria en la glucosa plasmática. Aunque se registró un aumento leve y transitorio del potasio en orina y un descenso en la excreción urinaria de sodio, los niveles séricos permanecieron dentro de límites normales

Registro electrocardiográfico continuo de 24 hs. en pacientes con espondilitis anquilopoyética durante la terapéutica con metilprednisolona en "pulso" / Continuous 24 hours electrocardiographic recording in patients with ankylosing spondylitis during treatment with methyl-prednisolone pulse

Recientemente se han comunicado casos de muerte súbita luego de la administración terapéutica de esteroides en "pulso". Se efectuó registro electrocardiográfico continuo 24 hs. antes, durante y 24 hs. después de cada una de tres administraciones de altas dosis de metilprednisolona por vía intravenosa efectuadas a 5 pacientes con espondilitis anquilopoyética grave y sin respuesta al tratamiento convencional. No se observaron arritmias supraventriculares o ventriculares, bradiarritmias, alteraciones de la conducción, ni cambios isquémicos. No se presentaron síntomas cardiovasculares, ni cambios en los controles clínicos en ningún caso. En todos los pacientes se produjo una elevación transitoria en la glucosa plasmática. Aunque se registró un aumento leve y transitorio del potasio en orina y un descenso en la excreción urinaria de sodio, los niveles séricos permanecieron dentro de límites normales (AU)

Peritendinous nodules in overlap syndrome.

Peritendinous nodules were observed in 4 patients with overlap syndrome. In all cases they were multiple, minute and located around the flexor tendons of the forearms and the extensor tendons of the hands. Similar nodules were also found on the extensor aspect of elbows, dorsum of the feet, hamstring muscles and Achilles tendons. Common histologic features were inflammatory granuloma with scattered areas of fibrinoid necrosis, mononuclear cell infiltration and occasional vasculitic lesions. Multiple peritendinous nodules should be considered another clinical feature of overlap syndrome.