The Clinical Features and Risk Factors of Parenchymal Neuro-Behcet’s Disease

Table 5

Biological agents in 6 severe/refractory p-NBD cases.

(a)

Case	Age	M/F	History of treatment	Clinical manifestations of p-NBD
				Symptoms	Lesion sites	CSF tests
1	24	M	GC^a CTX	Pyramidal sign, ataxia, irritating cough	Brainstem, cerebellum, spinal cord	Normal
2	45	M	GC^a CTX THD	Pyramidal sign, dysarthria, irritating cough	Brainstem, cerebellum	Pro 0.46 g/L, normal pressure, and cytological test
3	35	M	GC^a CTX	Pyramidal sign, epilepsy, psychological and behavioral change, disturbance of urine, myasthenia	Brainstem, hemicerebrum, spinal cord	ICP 200 mmH2O, WBC ^6/L, Pro 1.19 g/L
4	15	M	GC^b MTX	Epilepsy, headache	Not possible	ICP 235 mmH2O, normal biochemical and cytological test
5	36	M	GC^b MTX AZA	Psychological and behavioral change, disturbance of urine, myasthenia	Brainstem, hemicerebrum, spinal cord	WBC ^6/L, Pro 0.61 g/L
6	15	F	GC^a CTX CsA	Dysarthria, irritating cough, psychological and behavioral change	Hemicerebrum	ICP 246 mmH2O, normal biochemical and cytological test

(b)

Case	Treatment of biological agents				Follow-up
	Biological agents	Dose	Course	Drug combination	Symptoms	Side effects	Period (months)
1	IFX	3 mg/kg	4 times	GC^c CTX	Improved	None	31
2	IFX	5 mg/kg	5 times	GC^c CTX	Improved and ESR/CRP became normal	None	12
3	IFX	3 mg/kg	4 times	GC^c AZA	Symptoms and follow-up MRI got improved	None	9
4	IFX	3 mg/kg	5 times	MTX	No more seizures	None	72
5	TCZ	8 mg/kg	10 times	GC^c AZA	Symptoms and follow-up MRI got improved	None	11
6	IFN-α2a	3 Mu	53 months	GC^c CsA	Improved and ESR/CRP became normal	None	104

GC: glucocorticoid; CTX: cyclophosphamide; THD: thalidomide; MTX: methotrexate; AZA: azathioprine; CsA: cyclosporine A; Pro: protein; ICP: intracranial pressure; WBC: white blood cell. ^aMethylprednisolone pulse therapy. ^bAs  mg/kg/d or the equivalent dosage of other corticosteroids. ^cAs prednisone 5 mg/d-10 mg/d.