Gunasekaran Pradeep Kumar, Saini Arushi Gahlot
Designation: Resident, Department of Pediatrics, Institution: NYC Health + Hospitals/ Harlem, New York, USA.
Designation: Additional Professor, Department of Pediatrics, Institution: Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Semin Pediatr Neurol. 2025 Jul;54:101207. doi: 10.1016/j.spen.2025.101207. Epub 2025 Jun 21.
Subacute sclerosing panencephalitis (SSPE) is a devastating neurodegenerative disorder due to a persistent mutated, wild-measles virus infection of the nervous system. While the acute infection is generally self-limiting, defective viral clearance can lead to the emergence of neurovirulent strains that undergo mutations within the host, evade immune surveillance, establishing chronic central nervous system (CNS) infection. It predominantly affects children and young adults although no age, including infants, is immune to SSPE. The characteristic neurological manifestations include progressive behavioral and cognitive decline, neuromotor impairment, myoclonus, vegetative state and death within 1-3 years of diagnosis, although prolonged stabilization and spontaneous resolution have been reported in a minority of patients. Despite global efforts towards measles elimination, SSPE remains a challenging problem in the low- and middle-income countries (LMICs) due to inadequate vaccine coverage. Limited health infrastructure, suboptimal surveillance, and limited availability of diagnostic tests hinders early diagnosis and management making SSPE a public health crisis. The measles outbreaks in high-income countries with developed vaccination programs are commonly due to international travel, immigration, and vaccine hesitancy, making it a global problem. The lack of effective antiviral therapy makes supportive and palliative care the primary management strategy once SSPE is confirmed. Recent research highlights potential therapeutic strategies, novel molecular approaches targeting mutant measles viruses, and enhanced public health measures to contain the outbreaks. However, these remain largely inaccessible in LMICs with measles endemicity and the high disease burden of SSPE. Urgent action is needed to bridge this gap by strengthening vaccination programs, implementing early diagnostic strategies, and enhancing access to emerging treatments. The current review discusses the various aspects of SSPE and the importance of preventive strategies for SSPE as relevant to LMIC. Without holistic efforts and a multi-pronged approach to eliminate measles and prevent SSPE, the disease shall remain a global threat.
亚急性硬化性全脑炎（SSPE）是一种毁灭性的神经退行性疾病，由持续的变异野生型麻疹病毒感染神经系统所致。虽然急性感染通常具有自限性，但病毒清除缺陷可导致神经毒力菌株的出现，这些菌株在宿主体内发生突变，逃避免疫监视，从而建立慢性中枢神经系统（CNS）感染。它主要影响儿童和年轻人，不过包括婴儿在内的任何年龄段都不能对SSPE免疫。典型的神经学表现包括进行性行为和认知衰退、神经运动障碍、肌阵挛、植物人状态，并在诊断后1至3年内死亡，尽管少数患者有病情长期稳定和自行缓解的报道。尽管全球都在努力消除麻疹，但由于疫苗接种覆盖率不足，SSPE在低收入和中等收入国家（LMICs）仍然是一个具有挑战性的问题。有限的卫生基础设施、欠佳的监测以及诊断检测的可及性有限，阻碍了早期诊断和管理，使SSPE成为一场公共卫生危机。在疫苗接种计划发达的高收入国家，麻疹爆发通常是由于国际旅行、移民和疫苗犹豫，这使其成为一个全球性问题。一旦确诊SSPE，由于缺乏有效的抗病毒治疗，支持性和姑息性护理成为主要的管理策略。最近的研究突出了潜在的治疗策略、针对突变麻疹病毒的新型分子方法以及加强公共卫生措施以控制疫情。然而，在麻疹流行且SSPE疾病负担高的LMICs中，这些措施在很大程度上仍然难以获得。需要采取紧急行动来弥合这一差距，加强疫苗接种计划，实施早期诊断策略，并增加获得新兴治疗方法的机会。本综述讨论了SSPE的各个方面以及与LMIC相关的SSPE预防策略的重要性。如果不采取全面努力和多管齐下的方法来消除麻疹和预防SSPE，该疾病将仍然是一个全球威胁。
