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The central nervous system is sometimes affected in patients with Behçet’s disease.1 Meningoencephalitis and brainstem lesions are the most common problems. The appearance of subdural effusion has been rarely reported. Our patient, who had neuro-Behçet’s disease with massive bilateral subdural effusion, was successfully treated with steroid pulse therapy.
The patient (a 45 year old man) first developed polyarthralgia, fever, recurrent oral aphthosis, and headache in 1986. In 1987 he had a genital ulcer and positive pathergy test. Thus, he fulfilled the international criteria for the diagnosis of Behçet’s disease. He had two cycles of steroid pulse therapy, and his symptoms including headache subsided. In 1995 an episodic exacerbation of the neuro-Behçet’s disease occurred, accompanying parkinsonism and abnormal cerebral spinal fluid (CSF) findings (cell 5/3, protein 0.97 g/l, IgG 0.07 g/l). The symptoms improved after treatment with prednisolone 40 mg/day + colchicine 1 mg/day.
In December 2000 he began to have severe oral aphthosis and folliculitis, even though he had been taking low doses of steroids + colchicine. On 4 January 2001 he was admitted to our hospital because of fever (38–39°C), steppage gait, headache, and intentional tremor. Physical examination also disclosed mild rigidity (bilateral), dysarthria, poor finger-nose-finger test, poor heel-knee test, Kernig sign (−), and nuchal rigidity (−). No focal sign was evident. On admission, his white blood cell count was 8.8×109/l, erythrocyte sedimentation rate 22 mm/1st h, C reactive protein 81 mg/l, and serum IgD 21 mg/l. Other laboratory examinations of blood and urine, including antivirus antibodies were normal. The CSF showed: cell 18/3 (mononuclear cells 14, polymorphonuclear cells 4), protein 430 mg/l, glucose 3.1 mmol/l, Cl 105 mEq/l. An electroencephalogram was almost normal. Magnetic resonance imaging (MRI) at 010104 disclosed no interval change as compared with MRI at 971208 (mild brain atrophy only) (fig 1).
We first suspected infection as the origin of his symptoms, but he did not respond to antibiotics. MRI at 010110 showed the sudden appearance of bilateral subdural effusion. The effusion was iso-intensity at T1 weighted image and high intensity at T2 weighted image, suggesting exudate or transudate. We made a diagnosis of exacerbation of neuro-Behçet’s disease, and gave methylprednisolone 250 mg/day for three days followed by oral prednisolone 40 mg/day. Thereafter, the subdural effusion disappeared (MRI at 010222 and 010418). His neurological symptoms, including ataxia and dysarthria, gradually improved. His condition is being managed in the outpatient clinic and he has improved and his neurological symptoms have remained stable until now.
Bacterial meningitis of childhood sometimes accompanies subdural effusion.2 Because no direct infectious cause was shown by the laboratory tests, and steroid pulse therapy was effective, we suggest that the finding was due to neuro-Behçet’s disease. Previous studies have shown both a lymphocytic or neutrophilic meningoencephalitis and multifocal necrotic foci, predominantly in the brainstem and basal ganglion region, as the pathology of the central nervous system involvement.1,3,4 Bilateral subdural effusion in patients with neuro-Behçet’s disease is a rare complication of this disease.3–8
It is not always easy to distinguish exacerbation of the inflammatory disease from infection. However, the pathology of Behçet’s disease is generally considered to be vasculitis of small vessels, and differences in the symptoms may reflect the differences of the affected vessels.1,9 Thus, possibly, transient subdural venulitis led to the appearance of subdural effusion in this patient, and steroid pulse therapy brought about improvement of the subdural effusion. This case further emphasises the wide spectrum of neurological manifestations of Behçet’s disease.