Introduction

Idiopathic CD4+ lymphocytopenia (ICL) is a rare syndrome characterised by low CD4+ counts in absence of HIV infection and other immunodeficiency states or immunosuppressive therapy. Most of the patients are asymptomatic and are serendipitously diagnosed during evaluation of other clinical presentations. The aetiology, pathogenesis, and management of ICL remain poorly understood and inadequately defined.

Material and Methods

We report three patients with ICL who presented to us with distinct neurological disorders involving the central and peripheral nervous system as well as neuro-muscular junction. These included a middle-aged male with biopsy proven Progressive Multi-focal Leukoencephalopathy, a middle-aged female with Chronic Inflammatory Demyelinating Polyradiculoneuropathy and a young female with double seronegative myasthenia.

Discussion

Idiopathic CD4+ lymphocytopenia is associated with opportunistic infections akin to HIV infection. However, is not a well-described entity in neurological autoimmunity. The occurrence of autoimmune disorders in patients with ICL can be explained by a “two-hit” theory and dysfunction of the CD4 + 25+T-regulatory cells. In patients with ICL, immune reconstitution with intravenous immunoglobulins or Interleukin therapy may help in better outcomes.

Conclusion

Idiopathic CD4+ lymphocytopenia is an increasingly identified entity and has diagnostic and prognostic implications.

中文翻译：

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神经系统疾病中的特发性CD4淋巴细胞减少。

介绍

特发性CD4 +淋巴细胞减少症（ICL）是一种罕见的综合征，其特征在于在没有HIV感染和其他免疫缺陷状态或免疫抑制疗法的情况下，CD4 +计数低。大多数患者无症状，在评估其他临床表现时被偶然诊断。对ICL的病因，发病机制和治疗仍知之甚少，定义不充分。

材料与方法

我们报告了三名ICL患者，他们向我们展示了涉及中枢和周围神经系统以及神经肌肉连接的独特神经系统疾病。其中包括一名经活检证实为进行性多灶性白质脑病的中年男性，一名患有慢性炎性脱髓鞘性多发性神经根神经病的中年女性和一名患有双重血清阴性肌无力的年轻女性。

讨论区

特发性CD4 +淋巴细胞减少症与类似于HIV感染的机会性感染有关。然而，在神经系统自身免疫中并不是一个很好描述的实体。ICL患者自身免疫性疾病的发生可以通过“两次打击”理论和CD4 + 25 + T调节细胞功能障碍来解释。在ICL患者中，静脉内免疫球蛋白或白介素治疗可实现免疫重建，有助于改善预后。

结论

特发性CD4 +淋巴细胞减少症是一个日益被发现的实体，具有诊断和预后意义。