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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Subacute sclerosing panencephalitis
Pradeep Kumar Gunasekaran1, Arushi Gahlot Saini2
1Designation: Resident, Department of Pediatrics, Institution: NYC Health + Hospitals/ Harlem, New York, USA.
Abstract:
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.
Insights
Subacute sclerosing panencephalitis (SSPE) is a severe brain disease caused by persistent measles virus. Inadequate vaccination in low-income countries fuels SSPE, demanding urgent public health action.
Area of Science:
- Neuroscience
- Virology
- Public Health
Background:
- Subacute sclerosing panencephalitis (SSPE) is a fatal neurodegenerative disease resulting from persistent, mutated measles virus infection.
- Defective viral clearance allows neurovirulent strains to establish chronic central nervous system infections, affecting all ages but predominantly children and young adults.
- SSPE presents with progressive cognitive decline, neuromotor impairment, and often leads to death within 1-3 years, though rare cases show stabilization.
Purpose of the Study:
- To review the multifaceted aspects of SSPE, emphasizing preventive strategies.
- To highlight the challenges posed by SSPE in low- and middle-income countries (LMICs) due to low vaccine coverage and limited healthcare infrastructure.
- To underscore the global threat of SSPE and advocate for urgent, multi-pronged public health interventions.
Main Methods:
- Review of existing literature on SSPE pathogenesis, clinical manifestations, and epidemiology.
- Analysis of factors contributing to SSPE prevalence, including vaccination rates and healthcare access in LMICs.
- Examination of current and emerging therapeutic and public health strategies.
Main Results:
- SSPE remains a significant public health crisis in LMICs, exacerbated by inadequate measles vaccination.
- Measles outbreaks in high-income countries also contribute to SSPE incidence, driven by vaccine hesitancy and travel.
- Effective antiviral therapies are lacking, with management primarily focused on supportive and palliative care.
Conclusions:
- Strengthening vaccination programs, improving diagnostic strategies, and enhancing access to treatments are crucial for SSPE prevention.
- Addressing SSPE requires a global, holistic approach involving multi-pronged public health efforts to eliminate measles.
- Failure to act holistically will allow SSPE to persist as a global health threat.
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