Kija Edward, Ndondo Alvin, Spittal Graeme, Hardie Diana R, Eley Brian, Wilmshurst Jo M
Paediatric Neurology Unit, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital (RCWMCH) and Faculty of Health Sciences, University of Cape Town, South Africa.
S Afr Med J. 2015 Sep 14;105(9):713-8. doi: 10.7196/SAMJnew.7788.
Between 2009 and 2011, there was an outbreak of measles throughout South Africa (SA). The largest age category infected was children<5 years of age. In 2014, four patients, with a median age of 4 years and 5 months (range 4 years 3 months-4.5 years), three males and one female, presented with subacute sclerosing panencephalitis (SSPE). All were infected with measles during the period of the 2009-2011 outbreak in early infancy, at a time when their immune systems were immature and before they were vaccinated against the measles virus. One patient was immunocompromised, with vertically acquired HIV infection. All the children presented with cognitive and behavioural decline, abnormal movements and medically intractable myoclonic and atonic seizures. Outcome was poor in all and no reversibility was evident with standard therapeutic interventions. Optimal seizure control with carbamazepine is reported in patients with SSPE. Three of our patients who received carbamazepine experienced improved seizure control, but their neuroregression continued. Since submission of this case series, patient 1 (see Table 1) has died, and a further child has presented with the same clinical phenotype as described. On the basis of this clustering of patients in the Western Cape Province, SA, it is important to screen children admitted with acute cognitive decline and intractable seizures for SSPE, especially those who were infants during the measles outbreak.
2009年至2011年期间，南非爆发了麻疹疫情。感染的最大年龄组为5岁以下儿童。2014年，4名患者出现亚急性硬化性全脑炎（SSPE），中位年龄为4岁5个月（范围为4岁3个月至4.5岁），3名男性和1名女性。所有患者均在2009 - 2011年疫情期间婴儿期感染麻疹，当时他们的免疫系统尚未成熟，且未接种麻疹病毒疫苗。1名患者免疫功能低下，垂直感染了HIV。所有儿童均出现认知和行为衰退、异常运动以及药物难以控制的肌阵挛和失张力发作。所有患者预后均较差，标准治疗干预措施未见明显可逆性。据报道，卡马西平可使SSPE患者实现最佳癫痫控制。我们的3名接受卡马西平治疗的患者癫痫控制情况有所改善，但神经功能仍继续衰退。自本病例系列报告提交以来，患者1（见表1）已死亡，另有一名儿童出现了与上述相同的临床表型。基于南非西开普省患者的这种聚集情况，对于因急性认知衰退和难治性癫痫入院的儿童进行SSPE筛查非常重要，尤其是那些在麻疹疫情期间为婴儿的儿童。
