Neurological manifestations of Behçet’s disease in Japan: a study of 54 patients

Abstract

The type and frequency of neurological manifestations of Behçet's disease (BD) vary with ethnicity. We analyzed the neurological manifestations of BD in Japanese patients. All patients undergoing treatment at one of the two Yokohama City University hospitals from July 1991 to December 2007 and who fulfilled the Japanese criteria for BD revised in 1987 were studied retrospectively by chart review. Patients had been neurologically assessed by neurologists. We recorded neurological signs and symptoms, magnetic resonance imaging or computed tomography findings, and results of cerebrospinal fluid examinations from the records of each patient. We studied 412 patients with BD, of whom 54 (13%) had neurological involvement (neuro-Behçet's disease: NB). NB patients included a significantly higher proportion of males (61%) than non-NB patients (42%, P = 0.009). The majority of patients (n = 38, 70%) had acute parenchymal NB, 15 (28%) had chronic progressive parenchymal NB, and 1 (2%) had the non-parenchymal type. Headache and fever were more frequently reported by patients with acute parenchymal NB. Personality changes, sphincter disturbances, involuntary movements, and ataxia occurred predominantly in patients with chronic progressive parenchymal NB. Lesions were distributed throughout the CNS, but mainly in the brainstem, white matter, and basal ganglia. Analysis of end-point clinical outcomes revealed a poor prognosis for patients with chronic progressive NB. In Japan, most NB patients have the parenchymal type, and male gender is a predisposing factor. Because of the unfavorable prognosis associated with chronic progressive NB, development of effective therapies are urgently needed.

Atypical manifestations in Brazilian patients with neuro-Behçet’s disease

“…Female predominance for NBD was previously reported only in a study including Italian patients and another with Korean NBD patients [19,20]. The frequency of systemic manifestation of NBD agreed with previous reports [3,6,8,10,17,18]. In our study, the frequency of neurologic manifestation in BD was 20%.…”

“…According to studies done in different ethnic groups such as Turkish, Japanese, Caribbean, Iranian, and Caucasian patients with BD, men are more affected by NBD [3,6,8,10,17,18]. Nonetheless, epidemiologic features of BD seem to be different in Brazilian patients due to the higher female/male ratio (2.2/ 1.0), for the overall prevalence of BD observed in a previous study [13] and to the higher female ratio for neurological manifestations of BD in this study (1.4/1.00).…”

“…Thirty-six patients fulfilling the international study group (ISG) for BD criteria were included in the study [7,10]. Three patients that presented typical brainstem involvement and oral ulcers were considered as having probable NBD and were excluded from the study.…”

“…For example, intracranial hypertension is more common in patients of Middle Eastern origin and less often seen in those of Japanese and Caucasian ancestry [6,8,10]. Additionally, poor prognosis in NBD is associated with a number of variables that include male gender, younger age at onset of NBD, combined involvement of brainstem and other CNS areas, spinal cord involvement, frequent attacks, steroid dependency, progressive course, presence of pleocytosis, and increased protein content in cerebral spinal fluid (CSF) [11][12][13].…”

Prevalência de manifestações clínico-laboratoriais e comorbidades na polimiosite segundo o gênero

“…1 The male gender is considered a good prognostic factor regarding remission in patients with RA on anti-TNF, 2 with better disease activity indices. 3 In Behçet's disease and primary Sjögren's syndrome, the male gender is related with higher frequency of neurological manifestations 4 and pulmonary impairment, 5 respectively.…”