Fujino Yuzo, Saito Kozo, Maezono Keiko, Kasai Takashi, Mizuno Toshiki
Department of Neurology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine.
Rinsho Shinkeigaku. 2019 Jul 31;59(7):436-441. doi: 10.5692/clinicalneurol.cn-001275. Epub 2019 Jun 27.
We report a 54-year-old man, who presented with an acute onset of diplopia and ptosis on the left side. On admission, neurological examination showed left oculomotor and abducens nerve palsy. Brain MRI showed thickening of the left parieto-temporal dura mater with gadolinium enhancement. Whole-body CT revealed a mass lesion in the right submandibular gland, diffuse goiter, and bilateral hilar lymph node enlargement. Initially, IgG4-related disease was considered because of an elevated serum IgG4 level (240 mg/dl); however, biopsy of the submandibular gland showed non-caseating epithelioid cell granulomas that suggested sarcoidosis, which could be associated with the intracranial lesions causing his neurological manifestation. In cases of hypertrophic pachymeningitis, especially with increased serum IgG4 including our case, a careful assessment with pathological examination is critical for identifying various underlying conditions.
我们报告了一名54岁男性，他急性起病，出现左侧复视和上睑下垂。入院时，神经系统检查显示左侧动眼神经和外展神经麻痹。脑部磁共振成像（MRI）显示左侧顶颞部硬脑膜增厚，钆增强。全身计算机断层扫描（CT）显示右下颌下腺有肿块病变、弥漫性甲状腺肿和双侧肺门淋巴结肿大。最初，由于血清IgG4水平升高（240mg/dl），考虑为IgG4相关疾病；然而，下颌下腺活检显示非干酪样上皮样细胞肉芽肿，提示结节病，这可能与导致其神经表现的颅内病变有关。在肥厚性硬脑膜炎病例中，尤其是像我们的病例血清IgG4升高的情况下，通过病理检查进行仔细评估对于识别各种潜在疾病至关重要。
