Rigardetto R, Vigliano P, Boffi P, Marotta C, Rainò E, Arfelli P, Bonassi E, Gandione M, Vigna Taglianti M, Tovo P A, Russo R
Department of Pediatric and Adolescence Sciences, University of Turin, Italy.
Panminerva Med. 1999 Sep;41(3):221-6.
This study has been conducted on a series of HIV-1 infected children, with the aim of illustrating the features of encephalopathy onset, its evolution and its influence on life expectancy. The most useful exams for diagnosis are also outlined.
The perspective study lasted from January 1989 to June 1997. Forty six symptomatic patients, out of 142 seropositive children, were followed up in the Department of Paediatric and Adolescence Sciences of the University of Turin. The patients, now between 1 yr 2 mth and 13 yr 9 mth old, were born from HIV-1 seropositive mothers; seroreverters have been excluded. Scheduled neuropsychiatric consultations were used, consisting of a neurologic exam and an interview with parents, cognitive evaluations, EEGs, Evoked Potentials and CT scans. The results have been evaluated with log-rank test for the analysis of the survival curves.
We found a significantly higher mortality rate in encephalopathic versus non encephalopathic patients; encephalopathic patients, in whom neurologic signs began in the first year of life, have a worse prognosis than the other patients, in whom encephalopathy appeared later. We did not find a statistical correlation between clinical course and immunological deficit. The clinical features of encephalopathy are mainly characterized by pyramidal signs and cognitive deterioration. Clinical sign evolution is linked to the age of encephalopathy onset: plateau pattern encephalopathy, characterized by an early onset, severe motor signs and cognitive delay from the very beginning, shows a greater severity and a shorter survival than progressive encephalopathy, characterized by a slowly progressive evolution of pyramidal signs, to which a cognitive deterioration may be added.
Neuropsychological exams can be helpful in the diagnosis and follow-up of encephalopathy.
本研究针对一系列感染HIV-1的儿童开展，旨在阐明脑病发病的特征、其演变过程及其对预期寿命的影响。同时还概述了诊断最有用的检查方法。
这项前瞻性研究从1989年1月持续至1997年6月。都灵大学儿科与青少年科学系对142名血清学阳性儿童中的46名有症状患者进行了随访。这些患者目前年龄在1岁2个月至13岁9个月之间，均为HIV-1血清学阳性母亲所生；血清学转阴者被排除在外。采用定期神经精神会诊，包括神经系统检查、与家长面谈、认知评估、脑电图、诱发电位和CT扫描。结果采用对数秩检验进行生存曲线分析评估。
我们发现脑病患者的死亡率显著高于非脑病患者；出生后第一年出现神经体征的脑病患者预后比其他较晚出现脑病的患者更差。我们未发现临床病程与免疫缺陷之间存在统计学相关性。脑病的临床特征主要表现为锥体束征和认知功能减退。临床体征的演变与脑病发病年龄有关：平台型脑病起病早，一开始就有严重的运动体征和认知延迟，与进展型脑病相比，其病情更严重，生存期更短。进展型脑病的特点是锥体束征缓慢进展，可能伴有认知功能减退。
神经心理学检查有助于脑病的诊断和随访。
