Sarov-Riviere Mariana, Ancelet Claire, Nasser Ghaidaa, Caroff Jildaz, Aghakhani Nozar, Denier Christian
Department of Neurology, Bicêtre Hôpitaux Universitaires Paris Saclay, Assistance Publique, Hôpitaux de Paris (AP-HP), Le Kremlin Bicêtre, France.
Department of Neuroradiology, Bicêtre Hôpitaux Universitaires Paris Saclay, Assistance Publique, Hôpitaux de Paris (AP-HP), Le Kremlin Bicêtre, France.
Case Rep Neurol. 2025 Apr 7;17(1):57-61. doi: 10.1159/000544077. eCollection 2025 Jan-Dec.
Idiopathic intracranial hypertension (IIH) is a rare cause of chronic headaches. Usually, patients with IIH present headaches and papilledema with no focal neurological signs. Classical MRI findings feature characteristic signs, i.e., dilated perioptic nerves sheath and empty sella turcica. Rarely, cranial nerve palsies existed, especially abducens nerve palsy.
We reported herein another rare clinical feature of IIH: trigeminal neuralgia in association with meningocele. This 35-year-old obese woman initially presented with chronic headaches and papilledema. Cerebral MRI showed classical IIH findings. The CSF opening pressure was increased. A treatment coupling acetazolamide and iterative lumbar punctures led to the regression of papilledema, but headaches were difficult to control. Ten years later, while she was lost to follow up and treated with acetazolamide by her , she developed extremely painful neuropathic pain in the left trigeminal nerve territory, in association with a recurrence of the chronic headaches. A new MRI showed new bilateral cavum trigeminal meningoceles, predominantly on the left side, associated with an atrophy of the cisternal segment of the left trigeminal nerve. Angio-CT showed transverse sinus stenosis, treated by stenting. After this treatment, IIH symptoms disappeared, while trigeminal neuralgia amplified: a surgical procedure led to its complete disappearance.
To our knowledge, this is the first case reported of trigeminal neuralgia associated with meningocele formation in IIH. Our case illustrates the great efficacy of venous stenting in IIH, and one may wonder whether earlier stenting could have avoided the subsequent development of meningoceles and subsequent neuralgia.
特发性颅内高压(IIH)是慢性头痛的罕见病因。通常,IIH患者表现为头痛和视乳头水肿,无局灶性神经体征。经典的MRI表现具有特征性征象,即视神经鞘扩张和蝶鞍空泡。很少有颅神经麻痹,尤其是外展神经麻痹。
我们在此报告IIH的另一种罕见临床特征:三叉神经痛合并脑脊膜膨出。这位35岁的肥胖女性最初表现为慢性头痛和视乳头水肿。脑部MRI显示典型的IIH表现。脑脊液开放压升高。乙酰唑胺联合反复腰椎穿刺治疗使视乳头水肿消退,但头痛难以控制。十年后,在她失访且自行服用乙酰唑胺治疗期间,她在左侧三叉神经分布区域出现极其疼痛的神经性疼痛,并伴有慢性头痛复发。新的MRI显示新的双侧三叉神经脑脊膜膨出,主要在左侧,伴有左侧三叉神经脑池段萎缩。血管CT显示横窦狭窄,通过支架置入治疗。经过该治疗,IIH症状消失,而三叉神经痛加剧:手术治疗使其完全消失。
据我们所知,这是首例报道的IIH合并脑脊膜膨出形成的三叉神经痛病例。我们的病例说明了静脉支架置入术在IIH中的巨大疗效,人们可能会想,早期支架置入是否可以避免脑脊膜膨出及随后神经痛的发生。