Manoharan Kiruba Shankar, Leoni Agalya Pauline Vasanthi, Samuel Deepa Mary, Padmanabhan R
Deptartment of ENT, Skull Base, Head and Neck Surgery, Apollo Main Hospitals, Chennai, 600006 Tamilnadu India.
Deptepartment of Rheumatology, Apollo Main Hospitals, Chennai, 600006 Tamilnadu India.
Indian J Otolaryngol Head Neck Surg. 2025 Apr;77(4):1916-1921. doi: 10.1007/s12070-025-05416-5. Epub 2025 Mar 31.
Neurosarcoidosis when presents with isolated cranial nerve involvement is a significant diagnostic challenge as it can mimic many other conditions like granulomatous infections, vasculitis, IgG4 disease and malignancy. Our patient is a 59 year old lady with symptoms of left sided facial pain, head ache and diplopia. Clinical examination revealed 5th and 6th cranial nerve involvement. MRI revealed enhancing smooth soft tissue lesion in pterygopalatine fossa, pterygomaxillary fissure with extension along V2 and V3. PET-CT revealed hypermetabolic lesion along left mandibular nerve in infratemporal fossa. Vasculitis panel also failed to aid in diagnosis. Biopsy done from the maxillary and mandibular nerve revealed non caseating granuloma which finally led to the diagnosis of Neurosarcoidosis. Presence of non caseasting granuloma in the histopathological examination with absence of infection and malignancy is diagnostic of Neurosarcoidosis. Patient was started on steroids and she improved clinically and symptomatically.
神经结节病若仅表现为孤立的颅神经受累，是一项重大的诊断挑战，因为它可模仿许多其他病症，如肉芽肿性感染、血管炎、IgG4疾病和恶性肿瘤。我们的患者是一位59岁女性，有左侧面部疼痛、头痛和复视症状。临床检查发现第5和第6颅神经受累。MRI显示翼腭窝、翼上颌裂有强化的光滑软组织病变，并沿V2和V3延伸。PET-CT显示颞下窝沿左下颌神经有高代谢病变。血管炎相关检查也无助于诊断。对上颌神经和下颌神经进行活检，发现非干酪样肉芽肿，最终确诊为神经结节病。组织病理学检查中存在非干酪样肉芽肿且无感染和恶性肿瘤，可诊断为神经结节病。患者开始使用类固醇治疗，临床和症状均有改善。
