Diagnosis and management of Neuro-Behçet disease with isolated intracranial hypertension: a case report and literature review

BMC Neurol. 2023 Sep 25;23(1):335. doi: 10.1186/s12883-023-03392-3.


Background: Neuro-Behçet's disease (NBD), characterized by isolated intracranial hypertension, is a rarely encountered condition, especially in children. In this study, we describe the treatment of a pediatric patient with NBD, who exhibited isolated intracranial pressure elevation as indicated by the initial manifestation of diplopia and physical examination finding of papilledema.

Case presentation: An 8-year-old boy was admitted to the hospital with a confirmed diagnosis of Behçet's disease (BD) over eight months. The patient also presented with the symptom of diplopia for three days. The evaluation of the patient's nervous system did not reveal any apparent abnormalities. The measurement of cerebrospinal fluid pressure yielded a reading of 470 mm H2O. The examination of the fundus indicated papilledema, and imaging scans revealed evidence of focal demyelination. The symptoms of the child showed alleviation after the administration of mannitol, methylprednisolone, and azathioprine. Furthermore, this study involved a comprehensive analysis of 18 cases of NBD with isolated intracranial hypertension, comprising one case reported herein and 17 cases from the literature review. Three cases were children, and an equal distribution of males and females 9:9 was noted. The average age at the onset of symptoms was 24.7 years (8-38 years). Headache (90%) was the most commonly reported clinical manifestation, followed by blurred vision or diplopia (80%). The ocular manifestations included papilledema (100%), abducent nerve paralysis (20%) and local eye hemorrhages in the retina (30%). Notably, 88.9% of these ocular manifestations were relieved or cured after treatment.

Conclusion: This study presents the first reported case of NBD with isolated intracranial hypertension in the pediatric population of China. In a child with Bechet's disease presenting with features of raised intracranial pressure, it is important to be aware of neuro Bechet's presenting with intracranial hypertension without other neurological abnormalities. This will help make early diagnosis, institute treatment and prevent sequelae resulting from untreated raised intracranial pressure.

Keywords: Behçet's disease; Intracranial hypertension; Neuro-Behçet’s disease; Papilledema.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Adult
  • Awareness
  • Behcet Syndrome* / complications
  • Behcet Syndrome* / diagnosis
  • Behcet Syndrome* / drug therapy
  • Child
  • Diplopia / diagnosis
  • Diplopia / etiology
  • Female
  • Humans
  • Intracranial Hypertension* / complications
  • Intracranial Hypertension* / diagnosis
  • Male
  • Papilledema* / diagnosis
  • Papilledema* / etiology
  • Young Adult