[Behcet's syndrome with neurological manifestations. Report of 2 cases and review of the literature].
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OBJECTIVE: To describe a case of central diabetes insipidus that was associated with Behçet's syndrome. METHODS: We present a case report, including clinical, laboratory, and radiologic data. The pertinent literature is reviewed relative to diabetes insipidus and Behçet's syndrome, and a discussion about a possible association is presented. RESULTS: A 32-year-old man presented with diplopia and severe headaches and was found to have transverse sinus thrombosis. He reported having recurrent mouth and genital ulcers for 3 months before the current consultation. On the basis of the clinical picture, Behçet's syndrome was diagnosed. During hospitalization of the patient, polyuria and polydipsia developed, along with hypernatremia and hypotonic urine, indicative of diabetes insipidus. After desmopressin treatment was initiated, considerable improvement in clinical status and laboratory data was evident and persisted during follow-up. CONCLUSION: Behçet's syndrome, probably through vasculitic mechanisms, may result in central diabetes insipidus. This case report emphasizes that Behçet's syndrome should be considered in the differential diagnosis of diabetes insipidus.
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The gastrointestinal manifestation of Behçet's syndrome including colitis and esophageal ulceration has been reported in the past few years. However, esophageal ulceration associated with Behçet's syndrome is a rare condition. There appears to be only seven reports of esophageal ulceration associated with Behçet's syndrome. We have recently observed esophageal ulceration in a 64-year-old male known to be on case of untreated Behçet's syndrome (three major symptoms), who presented with epigastralgia and transient substernal discomfort just after swallowing food. In this patient, endoscopic examination revealed multiple esophageal ulceration and kissing-type gastric ulceration. The esophageal ulcers were not improved by frequent antacid therapy for six weeks, while gastric ulcers responded well to the therapy. Steroid therapy was begun and esophagoscopy revealed scarred ulcers after only two weeks. Therefore, steroid hormone therapy was thought to be of benefit in this patient. Esophageal ulceration associated with Behçet's syndrome, a rare condition, is reported and discussed in accordance with some reviews previously reported.
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A brother and sister of first cousin Pakistani parents presented with recurrent mouth ulcers and chronic diarrhoea in the neonatal period. Diarrhoea persisted in spite of treatment with oral prednisolone and sulphasalazine. Both children required subtotal colectomy with ileostomy. Histopathology of the resected colons was virtually identical and showed multiple deep "flask"-shaped ulcers, often penetrating to the serosa, in the presence of chronic inflammation, but without any of the characteristic histological features of Crohn's disease. The appearance closely resembled the colitis of Beh,cet's syndrome. Both children (aged 6 and 3.5 years, respectively) have continued to have recurrent perianal disease with intermittent bloody diarrhoea since the operation. Apart from the initial symptoms of oral aphthous ulcerations in both children, no other major criteria have developed so far. Intestinal Behçet's syndrome should be considered in the differential diagnosis of chronic inflammatory bowel disease in childhood.
INTRODUCTION: Deep cerebral vein thrombosis is a specific clinical pattern of neuroBehçet. The clinical features of ischemic stroke by Rosenthal or internal cephalic vein thrombosis predominate. METHOD: A retrospective analysis of twelve cases of Rosenthal vein thrombosis revealing Behçet disease were analyzed. These cases accounted for one-quarter of the NeuroBehçet patients treated in our unit. RESULTS: The typical pattern of Rosenthal vein thrombosis clinical was present in all patients who developed a diencephalo-mesencephalic syndrome. CONCLUSION: The clinical presentation is highly suggestive of diagnosis confirmed by imaging. Outcome is favorable with corticosteroids and anticoagulation.
A 27-year-old Kosovo-Albanian woman with recurrent oral and genital ulcerations, arthralgias, erythema nodosum, and eye symptoms, was admitted for dyspareunia. Objective findings met the diagnostic criteria for Behçet's syndrome (BS). An unexpected narrow fibrotic ring was found in the vagina. Two incisions were made and the diameter was normalised. She was treated with prednisolone and Imuran, with good symptom relief. BS might be a differential diagnostic hint when inexplicable vaginal stenosis occurs.
A case of 22-years old man with Behçet's syndrome is presented. Apart from typical aphtous ulcers in the mouth and on the scrotum the patient had oesophageal ulcerations and inflammatory changes in the ileum. During 8 years follow up the patient had several bleeding episodes from the gastrointestinal tract that required blood transfusions.
Behçet's syndrome is characterized by the recurrent ulceration of the genitals, aphthous lesions of the mouth, uveitis, or iridocyclitis followed by hypopyon. Morbidity is increasing in the regions of the Central and North Europe. The disease is endemic in the region of Mediterranean Sea and Japan. Clinical course of Behçet's syndrome in WHO classification in 4 patients in discussed. Aphthous stomatitis, ulceration of the genitals and iridocyclitis dominated in the symptomatology. Several laboratory tests including complement, immunoglobulins and HLA B-27 were carried out. No abnormal values were seen. A survey of literature on the epidemiology, pathophysiology, and therapy is given.
Among the systemic manifestations of Behçet's syndrome, venous and arterial involvements are discussed. Venous thrombosis is a common manifestation and may be considered as a cardinal sign in the diagnosis. Venous and arterial thrombotic complications may be regarded as agents of morbidity and mortality. The pathogenesis of the occlusive process is still some what obscure, although vasculitis and impaired local vascular fibrinolytic activity play a part in it. Therapeutic measures using fibrinolytic enhancing agents may prevent and control the thrombotic complications of the disease.
Medullary ischemia is an uncommon and serious complication of subrenal aortic surgery. It generally occurs after surgery for ruptured aneurysms and second operations on the aorta. We report the case of a 29-year-old patient who had Behçet's disease with multiple arterial complications. The patient underwent emergency surgery for ruptured aneurysm of the abdominal aorta. An aorto-aortic prosthetic graft was implanted. The postoperative period was complicated by paraplegia and thrombosis of the prosthetic graft with dissection of the iliac arteries. The patient was reoperated and an aorto-bifemoral bypass was installed. After two years, the patient has recovered part of his neurological deficiency. The pathogenesis of this complication is well known, but prevention remains a challenge.
An autopsy case of Neuro-Behçet's syndrome is reported with review of the previous 13 cases. A 48-year-old woman who had recurrent oral aphthae and genital ulcers beginning at the age of 27 years suddenly developed several neurological symptoms including headache, paraplegia and disturbance of speech. She died of septicemia due to the urinary infection 22 years after the appearance of the first symptoms and one year after the neurological manifestation. Autopsy revealed a marked atrophy of the frontal lobes of the cerebral hemispheres. Microscopic changes in the central nervous system were consisted of edema and lymphocytic cuffing around the vessels, small foci of softening of the white matter and accumulation of fat-laden cells around these foci, and demyelination. These histologic lesions showed a diffuse distribution in the entire brain without a characteristic localisation, but involvement of the basilar nuclei and the pons was most intense in the present case as observed in most of the previous reports. Microscopic examination of other organs revealed severe pyelonephritis and fatty changes of the liver.
OBJECTIVE: Behçet's syndrome (BS), a common chronic inflammatory disease in Turkey, results in considerable morbidity and increased mortality. We aimed to estimate its direct and indirect costs. METHODS: We studied 119 (78 male/41 female) patients with BS at our multidisciplinary BS out-patient clinic in Istanbul, between March and July 2005. The mean age and disease duration were 35 +/- 9 and 10 +/- 6 S.D. yrs. According to the primary clinical problem, patients were divided into: (i) ocular; (ii) vascular; (iii) neurological and (iv) mucocutaneous-joint disease subgroups. They were interviewed with a standardized questionnaire addressing: (a) direct costs such as medication, diagnostic tests, hospital visits, hospitalization fees and lodging and transportation expenses and (b) indirect costs such as lost workdays and wages. The costs were assessed retrospectively by assessing the expenses within a year of the survey and were expressed as US$ mean +/- S.D. RESULTS: The mean annual total cost per patient was US$ 3226 +/- 3488 (S.D.). The direct cost accounted for 68% of the total cost. Medication expenses made up 79% of the total direct cost. About 42% of the patients reported lost workdays that averaged 119 +/- 96 days S.D. Among the clinical subgroups mucocutaneous-joint involvement (US$ 1180 +/- 1053) had the lowest economic impact while the neurological disease (US$ 5005 +/- 2707) had the highest. CONCLUSION: In this first cost of illness study in BS, the neurological involvement had the highest annual cost. Direct costs were higher than the indirect costs. Drug costs were the major cost driver. BS causes considerable economic burden for the health care system in Turkey.
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