Venkataramana A, Pardo C A, McArthur J C, Kerr D A, Irani D N, Griffin J W, Burger P, Reich D S, Calabresi P A, Nath A
Department of Neurology, Johns Hopkins University, Baltimore, MD 21287, USA.
Neurology. 2006 Aug 8;67(3):383-8. doi: 10.1212/01.wnl.0000227922.22293.93.
To describe challenges in diagnosis and management of patients with clinical syndromes of immune reconstitution inflammatory syndrome (IRIS) involving the CNS.
The authors describe three patients with clinically distinct neurologic manifestations of IRIS with HIV infection who presented as diagnostic and therapeutic challenges.
One patient with cryptococcal meningitis developed acute cerebellitis with mass effect and brainstem compression. Corticosteroid therapy was associated with complete resolution of the cerebellar lesion but the patient developed VZV encephalitis. Another patient with progressive multifocal leukoencephalopathy developed subacute progression of focal neurologic deficits associated with contrast enhancing lesions on brain MRI. This patient had spontaneous resolution of the lesion but was left with residual deficits. One patient developed a progressive dementing syndrome and deterioration over several months resulting in coma during combination antiretroviral therapy. A brain biopsy in this latter patient showed massive infiltration of T lymphocytes predominantly of the CD8 subtype. This patient had a significant improvement with corticosteroids and change in antiretroviral regimen although she was left with residual cognitive impairment.
Immune reconstitution inflammatory syndrome should be suspected in patients who show clinical or radiologic deterioration following initiation of antiretroviral therapy accompanied with improvement in CD4 cell count and viral load. Some patients may respond to a brief course of treatment with corticosteroids.
描述免疫重建炎症综合征(IRIS)累及中枢神经系统(CNS)患者诊断和管理中的挑战。
作者描述了3例伴有HIV感染的IRIS患者,其具有临床上不同的神经系统表现,这些表现构成了诊断和治疗上的挑战。
1例患有隐球菌性脑膜炎的患者发生急性小脑炎,伴有占位效应和脑干受压。皮质类固醇治疗使小脑病变完全消退,但该患者发生了水痘-带状疱疹病毒(VZV)脑炎。另1例进行性多灶性白质脑病患者出现局灶性神经功能缺损的亚急性进展,脑MRI显示有强化病灶。该患者的病灶自行消退,但遗留有残余缺损。1例患者在联合抗逆转录病毒治疗期间出现进行性痴呆综合征并在数月内病情恶化,最终昏迷。对该例患者进行脑活检显示主要为CD8亚型的T淋巴细胞大量浸润。尽管该患者遗留有残余认知障碍,但使用皮质类固醇并改变抗逆转录病毒治疗方案后有显著改善。
对于在开始抗逆转录病毒治疗后出现临床或影像学恶化、同时CD4细胞计数和病毒载量改善的患者,应怀疑免疫重建炎症综合征。一些患者可能对短期皮质类固醇治疗有反应。