Cerebellar PACNS in an Elderly Patient Present as a Tumor-Like Mass Lesion: A case Report.

Q3 Medicine Acta neurologica Taiwanica Pub Date : 2019-06-15
Nai-Fang Huang, Jyh-Seng Wang, Ling-Ying Lu, Wei-Chuan Liao, Chen-San Su
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Abstract

Purpose: PACNS has a broad spectrum of clinical manifestations without typical features, and its clinical diagnosis is challenging. We report an elderly patient of cerebellar PACNS (Primary angiitis of central nervous system) presented as a brain tumor by MRI, and primary angiitis was proven by pathology.

Case report: We report an 81-year-old female who complained about vertigo for 3 weeks with right arm dysmetria. There were no other neurologic symptoms/signs, and the patient was free from headache. Brain CT showed a space-occupying lesion over the right cerebellum, and a high-grade glioma was suspected by brain MRI and MRS. The pathologic result of brain biopsy showed granulomatous variant of PACNS. The patient received immunosuppressant therapy as long-term therapy, and had favorable response during a 2-year follow up.

Conclusion: Due to variations in clinical presentation and nonspecific findings on imaging studies, PACNS is not easily diagnosed, especially in the aged population. PACNS should be considered as one of the differential diagnoses of any CNS dysfunction. PACNS is also an exclusionary diagnosis, so although brain biopsy is limited for its low sensitivity, its application is still important to exclude the possibility of other diseases. Although there have been reports of fulminant cases, PACNS can be treated successfully with immunosuppressant as maintaining therapy.

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老年患者小脑PACNS表现为肿瘤样肿块:1例报告。
目的:PACNS临床表现广谱,无典型特征,临床诊断具有挑战性。我们报告一例老年小脑性中枢神经系统原发性血管炎(PACNS),经MRI表现为脑肿瘤,病理证实为原发性血管炎。病例报告:我们报告一位81岁女性,主诉眩晕3周并伴有右臂节律障碍。患者无其他神经系统症状/体征,无头痛。脑CT显示右侧小脑占位性病变,MRI及mrs怀疑为高级别胶质瘤,脑活检病理显示为PACNS肉芽肿变型。患者长期接受免疫抑制剂治疗,随访2年,疗效良好。结论:由于临床表现的差异和影像学检查的非特异性发现,PACNS不易诊断,特别是在老年人中。PACNS应被视为任何中枢神经系统功能障碍的鉴别诊断之一。PACNS也是一种排他性诊断,因此尽管脑活检因其敏感性低而受到限制,但其应用对于排除其他疾病的可能性仍然很重要。虽然有暴发性病例的报道,但免疫抑制剂作为维持治疗可以成功治疗PACNS。
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来源期刊
Acta neurologica Taiwanica
Acta neurologica Taiwanica Medicine-Neurology (clinical)
CiteScore
1.30
自引率
0.00%
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0
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