BRAIN Publishes the Latest Clinical Findings of Prof. Xu Hongwei’s Team Online
2018/10/13 15:50:21
Source: Dept. of Geriatric Neurology    Author:     Clicks: 20

The world’s top neurological journal BRAIN (IF = 10.84) published online this month two cases of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLLIPERS) with rare pathological manifestations, which were reported by the team of Prof. Xu Hongwei from the Dept. of Geriatric Neurology of Xiangya Hospital, Central South University. The title of the paper is "Two cases of CLIPPERS with increased number of perivascular CD20-positive B lymphocytes". Dr. Yi Fang and Dr. Tian Yun of the Dept. of Geriatric Neurology, are the first authors. Prof. Xu Hongwei, Director of the Dept. Geriatric Neurology, and Prof. Li Jing of neuro-immunology and myopathy of the Dept. of Neurology are the corresponding authors. Xiangya Hospital, Central South University is the institute of the first and corresponding authors. The paper is the first one to report that the main pathological manifestation of CLLIPERS could be the increase of perivascular D20-positive B lymphocytes. The pathological manifestation could be the sign for forecasting the conversion of CLIPPERS into B-cell lymphoma of the central nervous system or lymphomatoid granulomatosis, providing a new solution for the study of CLIPPERS pathological manifestation.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a chronic inflammatory disease with lymphocytes infiltration in the periphery of pons, midbrain, and cerebellum blood vessels and sensitivity to steroid hormone. The disease is rare, with less than 100 reported cases around the world. Its clinical manifestations are sub-acute onset and progressive course. In most cases, ataxia or diplopia are the initial symptoms. Dysarthria, dysphagia, ocular motility disorders, dizziness, facial paraesthesia, and spinal cord damage could also be the symptoms. Its typical imaging findings is the "pepper" mottling or curvilinear changes that can be seen with magnetic resonance enhancement. In the past, its pathology was believed to be the inflammatory infiltration mostly by CD3 + or CD4 + lymphocyte in the periphery of arterioles and small veins, without the characteristic manifestations of other diseases (tumor, lymphoma, and so on).

For the first time, Prof. Xu Hongwei’s team found in CLIPPERS patients that the number of perivascular CD20-positive B lymphocytes increased and even exceeded the number of T-lymphocytes, differing from the past opinion of inflammatory infiltration mostly by perivascular CD3+ or CD4+T-lymphocytes. This is helpful for further revealing the pathology of CLIPPERS and pathogenesy. Prof. B. Mark Keegan from Mayo Clinic has given the following remark on the findings: The observed association with haematological malignancies is likely a tantalizing clue to its aetiology.

The geriatric neurology team led by Prof. Xu Hongwei has been focusing on the diagnosis and treatment of clinical intractable cases for years and confirmed the diagnosis of various rare and refractory diseases of neurology for the first time in Hunan and even across China. Guided by Prof. Xu Hongwei, Dr. Yi Fang, the first author of the paper, has taken part in the Jiangxi-Hunan Neurological Case Competition with one of the CLIPPERS cases and won the first prize in the final.



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