Division of Neuroimmunology, Department of Neurology, Emory University School of Medicine, 12 Executive Park Drive NE, Atlanta, GA, 30329, USA.
Neurology Residency Program, Department of Neurology, Emory University School of Medicine, Atlanta, GA, USA.
Neurol Sci. 2024 Dec;45(12):5889-5896. doi: 10.1007/s10072-024-07745-9. Epub 2024 Sep 4.
Cranial neuropathy is a principal disease manifestation of neurosarcoidosis, but many forms remain poorly described, including trigeminal nerve disease despite its frequency in reported cohorts (5-12%). Herein, we characterize the clinical course of patients with neurosarcoidosis involving the trigeminal nerve.
A single-center retrospective cohort analysis of patients with biopsy-proven sarcoidosis involving the trigeminal nerve was conducted between 1/1/2000-3/7/2023.
The trigeminal nerve was affected in 14/245 (5.7%) patients, being clinically symptomatic in 5/245 (2.0%) and asymptomatic with radiographic involvement in 9/245 (3.7%). 14/14 (100.0%) patients had systemic sarcoidosis. In the symptomatic group, trigeminal neuropathy was an inaugural feature in 4/5 (80.0%), unilateral in 5/5 (100.0%) with the V1 subdivision most affected (4/5, 80.0%), and associated with neuralgia in 2/5 (40.0%). On MRI, the cisternal nerve roots (9/14, 64.3%), Meckel's cave (7/14, 50.0%), and cavernous sinus (5/14, 35.7%) were most commonly affected, and 14/14 (100.0%) patients had extra-trigeminal neuroinflammation on cranial MRI. CSF was abnormal in at least one dimension in 11/12 (91.7%) tested. All three treated patients with symptomatic trigeminal neuropathy responded to immunomodulatory treatment, and symptomatic treatments for trigeminal neuralgia were helpful in two patients. After a median follow-up period of 63 months, the median modified Rankin scale score was 1 for both subgroups.
Neurosarcoidosis may involve any portion of the trigeminal apparatus, and when affected, it frequently demonstrates a mismatch in radiographic involvement from its clinical manifestations of facial numbness and pain, and typically occurs in association with other clinical or radiographic manifestations of neurosarcoidosis.
颅神经病变是神经结节病的主要疾病表现，但许多类型仍描述不足，包括三叉神经疾病，尽管其在报告的队列中很常见（5-12%）。在此，我们描述了累及三叉神经的神经结节病患者的临床病程。
对 2000 年 1 月 1 日至 2023 年 3 月 7 日期间经活检证实的累及三叉神经的结节病患者进行了单中心回顾性队列分析。
245 例患者中 14 例（5.7%）累及三叉神经，245 例中有 5 例（2.0%）有临床症状，9 例（3.7%）有影像学累及但无症状。14 例（100.0%）患者有全身结节病。在有症状的组中，三叉神经病变是首发症状的有 4/5（80.0%），单侧的有 5/5（100.0%），受影响的 V1 亚区最多（4/5，80.0%），2/5（40.0%）伴神经痛。磁共振成像（MRI）显示，脑池神经根（9/14，64.3%）、 Meckel 腔（7/14，50.0%）和海绵窦（5/14，35.7%）最常受累，14/14（100.0%）患者颅 MRI 上有其他三叉神经外神经炎症。12 例接受检查的患者中，至少有 11 例脑脊液（CSF）至少有 1 项异常。所有 3 例有症状的三叉神经病变患者均对免疫调节治疗有反应，2 例三叉神经痛的对症治疗有帮助。中位随访 63 个月后，两组的改良 Rankin 量表评分中位数均为 1。
神经结节病可能累及三叉神经装置的任何部位，受累时，其影像学表现与临床面部麻木和疼痛的表现不匹配，且通常与神经结节病的其他临床或影像学表现相关。
