‘Neuro-Behçet’s Syndrome in the United Arab Republic

Abstract

‘Neuro-Behçet’ is not a rare disease in the United Arab Republic, as it was possible to detect 22 cases in just 3 years. The detection of the true incidence of Behçet syndrome and the true percentage of ‘Neuro-Behçet’ depends on the combined search by all specialists concerned. The similarity between the presenting clinical pictures and the accepted, though sometimes etiologically vague, clinical syndromes is discussed in an attempt to render the consideration of ‘neuro-Behçet’ in differential diagnosis reasonably acceptable. This consideration is justified in the presence of the oro-genital and ocular manifestations, or a history thereof. The mere affection of the nervous system does not indicate a bad prognosis. The prognosis depends, as in many other neurological diseases, on the site of the lesion. Initial big doses of systemic steroids, with a permanent maintenance dose, are recommended. Further evidence is discussed favouring autoimmune pathogenesis, possibly triggered by a virus infection.

Behçet’s Disease: An Unusual Case with Bilateral Obliterating Retinal Panarteritis and Ischemic Optic Atrophy

“…Immunologi cal tests and cerebrospinal fluid examina tion were negative, whereas the electro encephalogram showed signs of a modest aspecific disorder of the cerebral electric activity, which is in accord with the other cases described in the literature [Cooper et al, 1974;Wolf et al, 1965;Fadli et al, 1973],…”

Neurological manifestations of Behçet's disease in a Caribbean population: clinical and imaging findings

“…This result must be interpreted with caution, given the small number of patients and the unknown prevalence of HLA B5 or B51 in the Guadeloupean population. The frequency of neurological involvement in BD varies from 5.3 to 25 % in the large patient series [4,25] and 3.3 % in a nationwide survey in Iran [9]. Out of 13 patients, seven had neurological forms which is much higher than expected and may reflect a selection of patients with neurological involvement or severe systemic symptoms more than a high level of neurological involvement in Afro-Caribbean cases.…”

“…Neurological involvement is reported mainly during the active phase of the disease and is inaugural in 3 % of cases [1]. The disease is frequently encountered in Japan, the Middle East and many Mediterranean countries particularly Turkey [9,22,28,33]. In these areas, it is a major cause of blindness, and has been linked to HLA-B5 or B51 antigens [8].Although isolated cases have been reported in African and AfroCaribbean individuals [13,14,24,26], the prevalence of BD in these people is unknown.…”

Papilledema as an Initial Manifestation of Behcet's Syndrome: Report of Four Cases with Cerebral Venous Sinus Thrombosis

“…Papilledema should be included as a separate minor criterion for diagnosis of Behcet's syndrome. It is often referred to as "benign intracranial hypertension," 4,7,[20][21][22]28 which is not correct as it is due to cerebral venous sinus thrombosis.…”

“…4,7,8,10,13,14 In reviewing large series, papilledema has been quoted variously as being found in 4.6% , 17 9% , 18 20% , 4 and 25% 7 of patients with neurologic manifestations, the frequency of the latter varying from 10% to as high as 49% of all cases. [1][2][3][4] In view of this, we suggest that papilledema should be included among the minor diagnostic criteria for Behcet's syndrome. Certainly, it appears to be at least as common as some of the other less frequently encountered manifestations such as epididymitis, gastroenterological dysfunction, or cardiac involvement.…”