IgG4-Related Neurological Disease: A Single Center Ambispective Study from South India

被引：0

作者：

Reddy, Y. Muralidhar ^{[1
,4
]}

Parida, Subhendu ^{[2
]}

Pidaparthi, Lalitha ^{[1
]}

Jaiswal, Shyam Kumar ^{[1
]}

Tourani, Vijaya ^{[3
]}

Osman, Syed ^{[1
]}

Kumar, B. Santosh ^{[1
]}

Murthy, Jagarlapudi M. K. ^{[1
]}

机构：

[1] CARE Hosp, Dept Neurol, Hyderabad, Telangana, India

[2] CARE Hosp, Dept Neuroradiol, Hyderabad, Telangana, India

[3] CARE Hosp, Dept Pathol, Hyderabad, Telangana, India

[4] CARE Hosp, Hyderabad, Telangana, Pakistan

来源：

NEUROLOGY INDIA | 2024年 / 72卷 / 01期

关键词：

Hypophysitis; IgG4-related disease; IgG4-related neurological disease; pachymeningitis; rituximab; STATEMENT;

D O I：

10.4103/NI.Neurol-India-D-23-00016

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

IgG4-related disease (IgG4-RD) is an immune-mediated multi-system disorder. The nervous system (IgG4-RND) is rarely affected. We describe a short case series. We performed an ambispective analysis of IgG4-RND patients admitted at our centre between January 2016 and December 2022. Eight patients (M: F-2:6) were included with a mean age at presentation of 40.63 +/- 17.88 years and disease duration of 5.16 +/- 4.08 years. The common diseased sites were pachymeninges (7), orbits (4), paranasal sinuses (3), frontal lobe (1), hypophysis (1), leptomeninges (1), and middle ear (1). Common symptoms were headache and cranial neuropathy. The common nerves involved were the optic nerve, followed by the third, fifth, sixth, and seventh. Cerebrospinal fluid showed lymphocytic pleocytosis. Histopathology showed lymphoplasmacytic infiltrate (8), fibrosis (5), >10 IgG4 + cells (7), and IgG4/IgG >40% (6). Six had a relapsing course. The rituximab-based treatment regimen showed a favourable response.

引用

页码：117 / 123

页数：8

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