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

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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.

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Abbreviations

BD:

Behçet’s disease

CNS:

Central nervous system

CSF:

Cell counts in cerebrospinal fluid

CT:

Computed tomography

EDSS:

Expanded Disability Status Scale

FLAIR:

Fluid attenuated inversion recovery

ISG:

International Study Group

MRI:

Magnetic resonance imaging

MRV:

Magnetic resonance venography

NB:

Neuro-Behçet’s disease

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Acknowledgments

This work was supported in part by grants from Behçet’s Disease Research Committee, Research on Specific Disease of the Health Science Research Grants from the Ministry of Health, Labour, and Welfare (Y. Ishigatsubo). The source of funding had no role on the writing of the report or the decision to publish the results. The authors are greatly indebted to Mr. Tom Kiper (Yokosuka, Japan) for his review and invaluable suggestions in preparing the manuscript.

Conflict of interest statement

The authors have received no financial support or other benefits from commercial sources for the work reported here, and the authors have no other financial interests that could create a potential conflict of interest or the appearance of a conflict of interest with regard to the present study.

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Correspondence to Yoshiaki Ishigatsubo.

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Ideguchi, H., Suda, A., Takeno, M. et al. Neurological manifestations of Behçet’s disease in Japan: a study of 54 patients. J Neurol 257, 1012–1020 (2010). https://doi.org/10.1007/s00415-010-5454-2

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  • DOI: https://doi.org/10.1007/s00415-010-5454-2

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