Table 1.

Clinical and demographic features of the NB patients with MLL

Cases	Age/Sex	BD findings	Complaints at admission	Physical examination	MRI and/or CT lesions	Biopsy	Treatment	Time of BD diagnosis (before or after neurologic manifestations)
Appenzeller et al. (1)	43/female	OU GU Bilateral AU	Headache Photophobia Asthenia	Drowsiness, left arm and facial paresis, right eyelid ptosis	Thalamic lesion with mass effect and contrast enhancement	Gliosis with gemistocytic astrocytes	Steroid CYC	Before
Tuzgen et al. (4)	59/female	OU, GU	Left hemiparesis, lethargy, sensations of bad smell, headache	Left hemiparesis, bilateral papilloedema vascular proliferation	Right frontotemporal mass causing shift	Reactive gliosis, panvasculitis, thrombosis, extensive infarction,	Right frontotemporal craniotomy and subtotal excision	After
Tuzgen et al. (4)	45/female	OU, GU, monoarthritis	Headache, right hemiparesis, somnolence	Right hemiparesis	Mass in the left mesodiencephalic junction	Biopsy was not performed	Steroid	After
Heo et al. (5)	47/male	OU, GU, uveitis	Headache Right side Weakness Vomiting	Right hemiparesis, dysarthria	GdT1I showed ring enhancing lesions with internal hypointensities with subtle perilesional oedema at left side of the pons and parietal cortex	Frequent perivascular lymphocytic cuffing, focal necrotic lesion, microglial nodules, foci of necrosis, foamy hystiocytic collections, reactive gliosis	Steroid	Before
Darmoul et al. (7)	38/Male	Folliculitis	Headache, aphasia, disturbed consciousness	Right hemiplegia	Pseudotumoral lesion in the left capsulo-thalamic region extending to the homolateral peduncle	Biopsy was not performed	Steroid Immunsupresive theraphy	After
Imoto et al. (9)	50/male	OU, GU, Skin eruption	Dystasia dysbasia	Left hemiparesis (+) left Babinski reflex dysarthria	Low density area with significant mass effect in the right basal ganglia and thalamus with ring like enhancement following contrast injection	Large numbers of chronic inflammatory cells mainly in the Virchow robin spaces	Steroid	After
Schmolck et al. (10)	39/male	OU, GU	Recurrent aseptic meningitis, diplopia, dysarthria	Right sensorimotor hemiparesis with hyperreflexia, right sided dysmetria, disdiadochokinesis, ataxic gait	Lesion in the left thalamus with patchy contrast enhancement and mass effect		Steroid CYC	Before
Matsuo et al. (11)	33/male	OU, GU, EN	Nausea, vomiting, headache	Mild right hemiparesis	Mass lesion at the left basal ganglia extending to the ventral side of the midbrain	Mild reactive gliosis	Steroid	Before
Bennett et al. (12)	23/Male	OU, GU, papulopustular rash	Headache, fever, progressive right sided weakness	Right homonymous hemianopia, right spastic hemiparesis	Mass lesion involving the left temporal lobe	Perivascular inflammatory infiltrate, no neoplasia	Steroid, AZA	Before
Yoshimura et al. (13)	41/Female	OU, GU, EN	Mental deterioration, right hemiparesis	Poor mental activity, dysarthria and right hemiparesis	Homogeneous hypodense lesion of the left lenticulothalamic region, mild mass effect	Ruled out a tumour but did not show any specific diagnosis	Steroid	After
Ben Taarit et al.14	26/Female	OU, GU	Left hemiplegia		Pseudotumoral lesion in the protuberance and the right cerebral pedicule		Steroid	After
Park et al.15	52/female	OU, EN like skin lesion and arthritis	Dizziness, nausea, vomiting	Brun’s nystagmus	T2WI showed high signal intensity in the right cerebellar hemisphere, post medulla, pons with an isosignal central mass, GdT1WI showed homogenously enhancing mass like lesion inside the T2 high signal intensity	Biopsy was not performed	Steroid AZA	After
Presented case	27/Female	OU, GU, skin lesions	Headache, vomiting	Left hemiparesis	hyperintense lesion in the brainstem	Biopsy was not performed	Steroid AZA	After

NB: neuro-Behçet; MLL: mass-like lesion; BD: Behçet’s disease; MRI: magnetic resonance imaging; CT: computerised tomography; OU: oral ulcer; GU: genital ulcer; AU: anterior uveitis; EN: erythema nodosum; CYC: cyclophosphamide; AZA: azathiopurin