Table 3 Proportion of isolated intracranial hypertension in patients with NBD

General

Male: Female

9:9

Average age

24.7 (8-38) y

Clinical manifestations

Headache

9/10

90%

Blurred or diplopia

8/10

80%

Nausea/vomiting

3/10

30%

Papilledema

18/18

100%

Abducens nerve palsy

2/10

20%

Eye hemorrhage

3/10

30%

Oral ulcer

18/18

100%

Genital ulcer

16/18

88.9%

Uveitis

1/10

10%

Skin manifestations

3/10

30%

Arthritis/arthralgia

2/10

20%

Superficial thrombophlebitis

3/10

30%

Lumbar CSF pressure ≥ 300 mmH₂O

8/10

80%

Glucocorticoid

18/18

100%

Treatment

Dehydrator

14/18

77.8%

Azathioprine

12/18

66.7%

Remission/recovery

16/18

88.9%

Prognosis

Disease recurrence

2/18

11.1%

Optic atrophy

2/18

11.8%

Parenchyma injury

1/18

5.6%