Cerebral hypoperfusion detected by SPET in early neuro-Behçet syndrome.

We used brain single-photon emission tomography (SPET) to detect hypoperfused areas in 15 consecutive Behçet syndrome patients. Five were suspected of having neuro-Behçet syndrome, having at least one neurological symptom. For these patients, SPET was performed within 1 month of the onset of nervous system involvement. The 15 patients fulfilled the criteria of the International Study Group for Behçet syndrome. Neurological assessment and SPET were complemented by EEG in all five patients with suspected neuro-Behçet syndrome and by magnetic resonance imaging in three. Brain SPET detected hypoperfused regions in all five neurological patients; EEG showed abnormalities in three. Magnetic resonance imaging was normal in the three patients in whom it was performed. SPET was negative in all patients without neurological involvement and 20 healthy controls. SPET detected a reduction in brain blood flow in early neuro-Behçet syndrome, but there was no definitive correlation between the hypoperfused brain regions and the clinical features. Further studies are required to evaluate the significance of brain hypoperfusion and the value of SPET in the early diagnosis of neuro-Behçet syndrome.

[Changes in the small and large intestines in systemic sclerosis]. / Alterazioni dell'intestino tenue e crasso nella sclerosi sistemica.

A series of 21 subjects (2 males and 19 females) affected with systemic sclerosis, was examined by small bowel (oral and intubation methods) and colon enema. The underlying process responsible for abnormalities in the small bowel and colon in systemic sclerosis is a variable and pacthy destruction of the muscularis propria, that produces the structural and functional changes detected on X-ray. Pathologic condition is the same affecting the esophagus. The scout film of the abdomen often reveals colonic distension and fecal impaction, so that it may be quite difficult to prepare adequately the patients for a barium enema. Peristalsis may be virtually absent in short segments, and transit time may be several time longer than that in normal patients. For these reasons, intestinal pseudo-obstruction may appear in systemic sclerosis. The observed radiographic changes are: 1) in the small bowel: a) dilatation of the gut, especially in its proximal portions (duodenum and jejunum), in which the valvulae conniventes are straightened, normal or thinned; b) presence of diverticula, 2-4 cm in diameter, with hemispherical shape without the neck-like opening into the bowel lumen; 2) in the colon, the characteristic finding is an increase in size of individual haustra, forming sacculations or pseudo-diverticula, usually on the antemesenteric border of the transverse colon, better demonstrated on post-evacuation film. Moreover, loss of colonic haustration is also observed associated to colonic elongation and dilatation.

Factor VIII-related activities after 1-deamino-8-D-arginine vasopressin in multicentric reticulohistiocytosis.

A woman with multicentric reticulohistiocytosis, having symptoms including bleeding and a borderline level of factor VIII, was given an i.v. infusion of DDAVP as a pharmacological test to evaluate the release of factor VIII-related properties from storage sites. The results show a reduced response to DDAVP in the patient in comparison with a normal control subject. This could be due either to a defect in the release of the factor VIII complex after DDAVP or to a reduced synthesis and/or storage capacity by the storage cells.

Erythroblastic islands in the bone marrow of rheumatoid arthritis with anaemia.

An increase of erythroblastic islands with recognizable central macrophages was observed in bone marrow of six rheumatoid arthritis (RA) patients suffering from anaemia. Since erythroblastic islands are more prominent in anaemic conditions associated with ineffective erythropoiesis, it is possible that this morphological finding might be an expression of the increased ineffective erythropoiesis observed in RA patients with anaemia.

[Pathogenesis of ankylosing spondylitis: relation between HLA-B 27 and Klebsiella aerogenes]. / Ricerche eziopatogenetiche sulla apondilite anchilosante: rapporti tra HLA-B 27 e Klebsiella aerogenes.

The Authors attested the evidence of a cross-reactivity among some Klebsiella antigens and HLA B 27 positive lymphocytes, suggesting the hypothesis that Klebsiella may be an initiating agent in ankylosing spondylitis (AS). The Authors determined also serum antibody response against Klebsiella antigens in AS patients. The results obtained are discussed.

Serum copper and ceruloplasmin levels in rheumatoid arthritis and degenerative joint disease and their pharmacological implications.

Serum copper concentration and ceruloplasmin activity were measured in patients with clinically established rheumatoid arthritis (R.A.) during the active phase, in patients with degenerative joint disease (D.J.D.) and in normal subjects. Copper and ceruloplasmin serum levels are significantly increased (P less than 0.01) in the arthritic group, but not in the degenerative joint disease group. Copper and ceruloplasmin levels are high significantly correlated in all the groups. This parallel enhancement of serum copper and ceruloplasmin in R.A. is commented in view of a possible protective role of endogenous copper and/or ceruloplasmin in inflammation.

Folic acid deficiency in beta-thalassaemia heterozygotes.

Plasma and red cell folate mean contents have been found to be significantly lower in 41 symptom-free beta-thalassaemia heterozygotes than in 21 controls. Such decreases must be considered as effects of an increased folate utilization caused by the enhanced total, both effective and ineffective, erythropoiesis. Since no close correlation has been found between packed cell volume and plasma or red blood cell folate levels, it seems that in 'healthy' beta-thalassaemia subjects, the degree of anaemia is not influenced by folate body reserves. However, the frequent finding of reduced red cell folate contents suggests that further folate imbalance might lead to clinically significant degrees of folate deficiency.

Controlled multicenter trial of tiopronin and d-penicillamine for rheumatoid arthritis.

Fifty-seven patients took part in a controlled double-blind trial between tiopronin and D-penicillamine as basic treatment for rheumatoid arthritis. Thirty-nine (19 receiving tiopronin, 20 receiving D-penicillamine) completed the trial after 1 year. Both drugs resulted in a decrease of the erythrocyte sedimentation rate, Ritchie index, and Lee index and in a sparing effect on symptomatic antiinflammatory therapy. Improvement in these variables was statistically highly significant at any interval with tiopronin, but was sometimes less or not at all significant with D-penicillamine. Nevertheless, the difference in effects between the 2 drugs never reached statistical significance. Six patients receiving tiopronin and 6 receiving D-penicillamine were taken out of the experiment because of side effects.