Subacute Sclerosing Panencephalitis Patients Research Articles

Cardiac Rhythm Aberrations in Subacute Sclerosing Panencephalitis: Insights From Heart Rate Variability Analysis.

Subacute sclerosing panencephalitis (SSPE) is a fatal neurological disorder resulting from persistent measles virus infection within the brain. Although neurological manifestations have been well-documented, the impact of SSPE on cardiac autonomic function, assessed through heart rate variability (HRV), remains understudied. In this prospective single-center study conducted from January 2022 to March 2023 in Southern India, 30 consecutive SSPE patients and age- and sex-matched controls underwent electrocardiogram recordings for HRV analysis. Various HRV parameters were assessed, including time-domain metrics (SD of normal-to-normal intervals, root mean square of successive differences between normal heartbeats, percentage of successive normal interbeat intervals greater than 50 msec), SD1 and SD2 for Poincaré plot analysis, and frequency-domain metrics (low frequency %, high frequency %, low frequency:high frequency ratio). In the study, SSPE patients exhibited markedly reduced HRV. Specifically, SD of normal-to-normal intervals (P = 0.003), percentage of successive normal interbeat intervals greater than 50 msec (P = 0.03), and SD2 (P = 0.0016) were significantly lower compared with controls. Frequency-domain analysis did not reveal significant distinctions. Correlation analysis demonstrated a negative relationship between percentage of successive normal interbeat intervals greater than 50 msec and SSPE severity (r = -0.37, P = 0.042). Heart rate variability did not significantly differ between SSPE stages or with clinical variables. The interbeat interval range showed a narrower distribution in SSPE subjects. Our study highlights the clinical relevance of HRV analysis in SSPE and autonomic dysfunction throughout the disease course underscoring its importance in SSPE. This investigation provides valuable insights into cardiac autonomic dysfunction probably because of affliction of the central autonomic networks caused by the disease process and may be a contributing factor to mortality in SSPE.

TDP-43 pathology in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a fatal, slowly progressive brain disorder caused by a mutated measles virus. Both subacute inflammatory and neurodegenerative mechanisms appear to play significant roles in the pathogenesis. TAR DNA-binding protein 43 (TDP-43) inclusions are a common co-pathology in several neurodegenerative disorders with diverse pathogenesis. In the present study, we examined brains of 16 autopsied SSPE patients for the presence of TDP-43 pathology and possible associations with tau pathology. Immunohistochemical staining identified TDP-43 inclusions in 31% of SSPE cases. TDP-43 pathology was widely distributed in the brains, most severely in the atrophied cerebral cortex (temporal and parietal), and most frequently as tangle- and thread-like neuronal cytoplasmic inclusions. It was associated with longer disease duration (>4 years) and tau pathology (all TDP-43-positive cases had tau-positive neurofibrillary tangles). This study demonstrates for the first time an association between TDP-43 pathology and SSPE. The co-occurrence of TDP-43 and tau aggregates and correlation with the disease duration suggest that both pathological proteins are involved in the neurodegenerative process induced by viral inflammation.

Subakutni sklerozirajući panencefalitis - promene fenotipa tokom poslednje decenije

Introduction: Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, neurodegenerative disease with poor outcome. Anti-measles vaccination contributed to a decreasing number of SSPE patients, but not to its eradication. The aim of our study is to evaluate the course of the disease in our SSPE patients with a focus on vaccinated children. The main goal is considering possibilities for improving prevention of the disease. Methods: A retrospective study included the patients with SSPE treated in the period from December 2010 to December 2020 at the Pediatric Clinic of the Institute. The inclusion criteria were the patients with the diagnosis of SSPE based on clinical presentation, neuroimaging, electroencephalography and positive IgG anti-measles antibodies, both in serum and CSF. Results: Five children with fulminant course of SSPE were included. All these patients were suffering from measles at an early age. Three of them had been vaccinated against measles and two had not. All of them had previously been healthy, immune-competent children, with normal general development. The course was extremely fulminant with lethal outcome within three months since the initial symptoms in four cases. Progressive motor and cognitive decline, behavior changes, movement disorders, myoclonic jerks and seizures were dominant in clinical presentation. Conclusion: Despite vaccination, SSPE has not been eradicated. An increasing number of vaccinated immune-competent children with fulminant form of SSPE and history of measles infection at an early age were treated at our Clinic. As a measure for improving prevention, we suggest considering weaning of vaccine-derived immunity, and re-vaccination of girls at reproductive period.

Association of NOD1 and NOD2 Polymorphisms With Susceptibility to Subacute Sclerosing Panencephalitis.

Background: Subacute sclerosing panencephalitis is a progressive neurodegenerative disease that is a late complication of measles infection. However, to date, the pathogenesis of subacute sclerosing panencephalitis is still not explained; both viral and host factors seem to be associated. The present study aimed to investigate the relationship between NOD1 and NOD2 gene variants and subacute sclerosing panencephalitis. Methods: The gene variants of NOD1 (rs2075820 and rs2075818) and NOD2 (R334Q and R334W) were explored in 64 subacute sclerosing panencephalitis patients and 70 controls using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Results: The frequencies of the AA genotype and A allele of rs2075820 (NOD1; c.796G>A) polymorphism were lower in patients compared with controls (P = .022 and .014, respectively). The presence of the A allele of rs2075820 may be considered as a protective factor for subacute sclerosing panencephalitis. There was a significant difference between the groups in rs2075818 (NOD1 G/C) polymorphism, and the CC genotype increased the risk of subacute sclerosing panencephalitis by 3.471-fold. The carriers of the C allele of rs2075818 (G/C) had a 1.855-fold susceptibility to subacute sclerosing panencephalitis (P = .018). The GC genotype might be associated with subacute sclerosing panencephalitis susceptibility in the patients compared with patients without having that haplotype (P = .03). Conclusions: Thus, we identified an association between subacute sclerosing panencephalitis and the rs2075820 (NOD1 G/A) and rs2075818 (NOD1 G/C) polymorphisms. These findings implicate a possible effect of this genetic polymorphism in susceptibility to subacute sclerosing panencephalitis, which needs to be confirmed in bigger populations.

Short-term Outcomes of the Use of Intraventricular Ribavirin in Filipino Patients with Subacute Sclerosing Panencephalitis

Background. Subacute sclerosing panencephalitis (SSPE) is a fatal neurodegenerative disease caused by prolonged persistent infection of the central nervous system with a measles virus mutant. Though various treatment modalities have been tried, there is no effective treatment to completely cure SSPE and new therapeutic strategies are needed.
 Objective. This is a prospective uncontrolled observational open label trial to describe the short-term outcomes and safety of intraventricular ribavirin in combination with oral isoprinosine in Filipino SSPE patients.
 Methods. Sixteen (16) unrelated SSPE patients between ages 3-26 years and in various clinical stages were included in this study. Demographic data were described. Intraventricular instillation of ribavirin (1-3 mg/kg/dose) through an Ommaya reservoir was given for a duration of 3-6 months in 13 patients. The duration of follow-up was 48 weeks. The clinical outcome was assessed before, during, and after treatment using the Neurological Disability Index (NDI), Brief Assessment Examination (BAE), and clinical staging using the Jabbour Classification. Adverse side effects from intraventricular ribavirin were enumerated.
 Results. Six of 13 (46.15%) patients mostly in Stage III illness had clinical improvement showing decreasing NDI and BAE scores during treatment and the clinical improvement was maintained or improved further during the 48-week follow-up period. Clinical improvement manifested as improved mental alertness, decrease in spasticity and reduction of seizures. The clinical staging of those who improved remained stable during and after treatment was discontinued. Five (38.46%) patients in Stage II disease worsened and progressed to Stage III despite ribavirin therapy including 1 (7.6%) patient who died after the treatment phase due to pneumonia and brainstem failure. The clinical course of two (15.38%) patients remained unchanged. Minor adverse side effects of ribavirin included transient fever, rash, oral sores, seizure episodes, drowsiness, bladder retention and mild increase in transaminases. Ommaya reservoir infection was a serious adverse event in 5 (31.25%) patients.
 Conclusion. There is still no definitive cure for SSPE. Although ribavirin may help alleviate some of the symptoms of SSPE and prolong life, it may not reverse or halt the progression of the disease. Long term follow-up of these patients and continuous use of intraventricular ribavirin will better clarify its role in modifying the fatal course of SSPE. The role of ribavirin in Stage I patients and a controlled clinical trial in Stage II SSPE needs further studies.

The immunogenetics of subacute sclerosing panencephalitis: A comprehensive review

Background: Subacute sclerosing panencephalitis (SSPE) is a rare devastating complication of measles virus (MV) involving the central nervous system (CNS). SSPE occurs 4–11/100,000 cases of the measles. Main Body: A poor cellular immune response seems to predispose individuals to the development of SSPE. The presence of mutations that may lead to MV persistence has also been demonstrated in samples obtained from SSPE patients. However, no study to date has definitively revealed the pathogenesis of SSPE caused by persistent infection in the CNS of MV. Conclusion: In this review, we provide a brief overview of SSPE from both an immunological and genetic perspective. We will try to focus on the mechanisms underlying the pathogenesis of SSPE that results in MV persistence. Clarifying the pathogenesis of SSPE will enable both the expansion of therapeutic options and the prediction of disease prognosis.

Clinical Profile and Diffusion Tensor Imaging in Patients of Subacute Sclerosing Pan Encephalitis: A Prospective Follow-up Study from Eastern Part of India.

Subacute Sclerosing Pan Encephalitis (SSPE) may present with atypical clinical features and lead to diagnostic dilemma. Conventional magnetic resonance imaging (MRI) may be normal in early stage of SSPE. The aim of this work was to study the demography, clinical profile including atypical features of SSPE patients, and the utility of diffusion tensor imaging (DTI) as an adjunctive diagnostic tool to the anti-measles antibody and conventional MRI. Consecutive 25 patients of SSPE were included. Clinical details were recorded at baseline and 6 months follow-up. Anti-measles antibody in serum and CSF, CSF/Serum Quotient reference (CSQ ref), and radiological details including comparison of DTI between SSPE patients and controls were also noted. Of 25 patients, 17 (68%) were male. The most common presenting feature at onset was myoclonus with or without falls (13, 52%). Atypical features such as seizure, hemiparesis, and visual problems were present in 28% patients. At 6 months, 9 patients had progressive course (6 expired), 10 were static, and 6 lost to follow-up. MRI was normal in 8 (32%) patients (stage 2/3- 7/1). On comparison between SSPE patients (N = 10) and control (N = 10) groups, fractional anisotropy (FA), and apparent diffusion coefficient (ADC) values were reduced and elevated, respectively, at most of the regions of interest with significant difference at many sites. A significant number of patients (28%) had atypical features at onset. DTI is an adjunctive tool which supplements the conventional MRI and increase diagnostic yield. It may be a future option to assess disease progression and treatment response.

Subacute Sclerosing Panencephalitis: Restricted Diffusion and Clinical Evolution.

Subacute sclerosing panencephalitis (SSPE) is a rare entity characterized by a protracted course and progressive neurological deterioration. We present patterns of diffusion restriction in eight cases of SSPE, a seldom described imaging attribute. A retrospective analysis was performed on the clinical and neuroimaging data obtained from records of patients with proven SSPE. Patients whose magnetic resonance imaging (MRI) showed evidence of diffusion restriction were included in the analysis. MRI was performed on 3 T and 1.5-T clinical MR systems. Imaging characteristics were reviewed and tabulated by two neuroradiologists. Eight SSPE patients (seven men, one woman; age range: 5-15 years; mean age: 11 years) diagnosed and managed at our institute were included in the analysis. Restricted diffusion was evident in the basal ganglia (n = 3), corpus callosum (n = 2), white matter (n = 2) and in bilateral middle cerebellar peduncles (MCP) (n = 2). One patient had diffusion restriction in the genu of the corpus callosum and bilateral frontal cortical white matter. None of the diffusion-restricted lesions showed contrast enhancement or susceptibility. Six cases fulfilled the diagnostic criteria for fulminant SSPE (fSSPE). The extent of neuroparenchymal involvement was greater in this subset of patients. Restricted diffusion in SSPE, hitherto infrequently described, can indeed occur in both grey and white matter structures and in both supratentorial and infratentorial compartments. Parenchymal diffusion restriction in SSPE possibly reflects an early time point in the clinical evolution. A greater extent of parenchymal diffusion restriction may portend a rapid downhill course, possibly qualifying for fSSPE.

Progression of myoclonus subtypes in subacute sclerosing panencephalitis

ObjectivesDiagnostic criteria of subacute sclerosing panencephalitis (SSPE) include myoclonus, a well-recognized clinical feature. Here, we studied the electrophysiological features of myoclonus with regards to disease staging in SSPE patients. MethodsWe included 10 patients diagnosed with SSPE between 2010 and 2018, along with 21 healthy subjects. All participants had detailed electrophysiological evaluation including polymyographic analysis, blink reflex after trigeminal stimulation, auditory startle response, startle response after somatosensory stimuli, F-waves, and long-loop reflexes. Clinical findings were retrieved from the medical records. ResultsPatients were categorized into Gascon stage 2B (n = 5, 50%), 2A (n = 2, 20%), 3B (n = 2, 20%) and 4A (n = 1, 10%) at the time of electrophysiological evaluation. Two patients had cortical myoclonus, four had possible cortico-subcortical myoclonus, and four had brainstem myoclonus. Patients were categorized into Gascon stages 2a and 2b had possible cortico-subcortical myoclonus (85.7%). However, none of the patients with stage 3b or 4a had possible cortico-subcortical subtype but all had the brainstem subtype. ConclusionAssociation was seen between subtypes of myoclonus and clinical staging in SSPE. This suggests that myoclonus in SSPE may primarily involve the cortex and cortico-subcortical structures such as the thalamus at earlier stages of disease, and then involve more caudal structures as the disease progresses.

Electroencephalography Patterns of Subacute Sclerosing Panencephalitis.

Introduction and background: This article explores the electroencephalography (EEG) pattern in patients of suspected subacute sclerosing panencephalitis (SSPE) visiting Lady Reading Hospital, Peshawar. Pakistan has a huge deficit of research culture, and limited studies are done on this topic. This study concluded that a typical pattern is the most common EEG pattern, although atypical and normal EEG patterns were also observed. It is worth inclusion into the existing literature and may be used for future literature review of similar studies done elsewhere in Pakistan to give a wider perspective comprised of a larger sample size - integrating all studies.Objective: To determine the frequency of different patterns of EEG (typical, atypical, and normal) in SSPE patients.Methodology: Seventy-seven patients of both male and female genders between ages one to 20 years, who were diagnosed with SSPE, were included in the study. Dyken’s criteria were used to diagnose the patients. A prior history of previous measles infection with signs and symptoms suggestive of SSPE and positive anti-measles IgG antibodies in the cerebrospinal fluid (CSF) was found in all the patients included in the study. Besides this, typical EEG patterns and raised CSF globulin levels were also used for confirmation of the diagnosis. All the patients fulfilling the above criteria and presenting to the neurology department of Lady Reading Hospital, Peshawar, from February 1, 2019, to November 30, 2019, were included in the study. All the patients underwent the EEG monitoring in the same EEG laboratory and were reported by the same consultant with careful exclusion of any artifacts during the study.Result: There were 59 (76.62%) males and 18 (23.37%) females. The mean age was 15 ± 8.6 years, and the mean duration of symptoms was 4.79 ± 1.68 months. EEG was normal in 14 (18.18%) patients, while 63 (81.81%) patients had an abnormal EEG pattern, with a majority of 53 (84.12%) patients showing periodic delta wave complexes. Only 10 (15.87%) patients showed atypical patterns.Conclusion: Almost all the patients of SSPE showed periodic high-amplitude delta waves complexes, which usually occurs in patients with a disease duration of more than four months. However, further studies with a large sample size are needed for the confirmation of this observation.

Advances in Antiviral Therapy for Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a late-onset, intractable, and fatal viral disease caused by persistent infection of the central nervous system by a mutant strain of the measles virus. Ribavirin intracerebroventricular therapy has already been administered to several SSPE patients in Japan based on fundamental and clinical research findings from our group, with positive therapeutic effects reported in some patients. However, the efficacy of this treatment approach has not been unequivocally established. Hence, development of more effective therapeutic methods using new antiviral agents is urgently needed. This review describes the current status of SSPE treatment and research, highlighting promising approaches to the development of more effective therapeutic methods.

Immune alterations in subacute sclerosing panencephalitis reflect an incompetent response to eliminate the measles virus.

In subacute sclerosing panencephalitis (SSPE) the persistence of measles virus (MeV) may be related to the altered immune response. In this study, cytokine responses of lymphocytes and monocytes were evaluated in SSPE compared to controls with non-inflammatory (NICON) and inflammatory (ICON) diseases. Patients with SSPE (n = 120), 78 patients with ICON and 63 patients with NICON were included in this study. Phenotypes of peripheral blood mononuclear cells (PBMC) have been analyzed by flow cytometry. CD3 and CD28, and S. aureus Cowan strain I (SAC) stimulated and unstimulated cells were cultured and IL-2, IL-10, IFN-γ, IL-12p40, IL-12p70 and IL-23 were detected in supernatants by ELISA. MeV peptides were used for MeV-specific stimulation and IFN-γ secretion of PBMC was measured by ELISPOT. Spontaneous and stimulated secretions of IL-10 were lower in SSPE compared to both control groups. T cell stimulation induced lower IFN-γ production than ICON group, but higher IL-2 than NICON group in SSPE. Stimulated PBMC produced lower IL-12p70 in SSPE and had decreased CD46 on the cell surface, suggesting the interaction with the virus. IFN-γ responses against MeV peptides were not prominent and similar to NICON patients. The immune response did not reveal an inflammatory activity to eliminate the virus in SSPE patients. Even IL-10 production was diminished implicating that the response is self-limited in controlling the disease.

Diagnostic Approaches and Management of Subacute Sclerosing Panencephalitis in Children

Abstract: Subacute Sclerosing Panencephalitis (SSPE) is a progressive neurodegenerative disease that attacks the central nervous system, especially in the population of children and early adolescents, due to persistent measles virus infection. The incidence of SSPE is quite rare, and data shows that in developing countries it is still quite high compared to developed countries. Diagnosis is based on clinical, supporting examinations such as EEG, as well as increased antibodies against measles virus in serum and cerebrospinal fluid. Symptoms can include changes in behavior, myoclonus, memory problems, and persistent pyramidal or extrapyramidal movements. Management to date has not provided satisfactory results and is individualized. Most SSPE patients experience a progressive and gradual course, leading to death within 1-3 years. The challenge of SSPE in children is the approach to diagnosis and management. Until now, the approach to treating SSPE in children is still based on the pathophysiological mechanisms from several existing research studies. That why therapy guidelines for children with SSPE are still varied. This paper aims to discuss the topic of SSPE in children with a major focus on diagnostic and therapeutic approaches based on the latest scientific evidence. Keywords: Subacute Sclerosing Panencephalitis (SSPE), children, diagnosis, therapy

Utility of enzyme immunoassays for diagnosis of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a progressive neurologic disorder caused by the measles virus (MV) and is identified by positive MV-specific antibody titers, detected mainly by hemagglutination inhibition (HI) tests in the cerebrospinal fluid (CSF). However, an alternative method, the enzyme immunoassay (EIA), has increasingly become a preferred method for detecting MV antibodies. To establish the index for SSPE diagnosis using EIA, we investigated the correlation between HI and EIA titers of MV antibodies in SSPE patients. Data on MV antibody titers and measurement methods at the time of diagnosis in 89 Japanese SSPE cases diagnosed between 1979 and 2006 were obtained by a survey. We also assessed the serum and CSF MV antibody titers in three patients with SSPE and serum MV antibody titers in 38 healthy adults using immunoglobulin G (IgG)-EIA and HI. In all cases diagnosed as SSPE, IgG-EIA titers in the CSF were ≥0.49IU/mL. There was a positive correlation between serum antibody values in the controls measured by IgG-EIA and HI. In patients with SSPE, both serum and CSF antibody values, measured by IgG-EIA, and HI, were positively correlated, and a positive correlation was found between the serum and CSF MV antibody titers as measured by IgG-EIA. The serum/CSF MV antibody titer ratios determined by IgG-EIA were <20 in most SSPE patients. Immunoglobulin G-EIA may be a suitable alternative method for SSPE diagnosis; however, its potential utility and the cut-off point of ≥0.49IU/mL should be tested with additional patient cohorts.

High risk of subacute sclerosing panencephalitis following measles outbreaks in Georgia

ObjectiveTo describe cases and estimate subacute sclerosing panencephalitis (SSPE) risk following large-sale measles outbreaks in Georgia. A rare, fatal late complication of measles, SSPE is often overlooked in assessments focused on the acute illness. Georgia had 8377 and 11,495 reported measles cases during the 2004–2005 and 2013–2015 outbreaks, respectively, but SSPE burden has not been assessed. MethodsSSPE cases diagnosed during 2008–2017 were identified from hospitalization registries in major neurological departments likely to admit SSPE patients. Information on reported measles cases and deaths was obtained from the national measles surveillance system and published reports. The risk of SSPE (number of measles cases per one SSPE case) was calculated for cases associated with the 2004–2005 outbreak. Crude estimates were adjusted to account for potential under-reporting of measles, using 50%, 25% and 10% estimates of completeness of reporting. ResultsSixteen SSPE cases diagnosed during 2008–2017 were identified. Eleven (92%) of 12 SSPE cases with a known history of measles had infection at ≤2 years and one (8%) at 3 years of age. Crude estimate of SSPE risk for the 2004–2005 outbreak was 1:1396. Adjusted estimates were 1:2792, 1:1:5584 and 1:13 960, assuming 50%, 25% and 10% completeness of reporting measles cases, respectively. ConclusionsThe review demonstrated substantial risk of SSPE in Georgia, supporting recent data suggesting that risk of SSPE following measles infection is higher than previously thought. To prevent SSPE in Georgia, very high timely immunization coverage for measles should be achieved among children, and immunity gap among adults should be closed.

Elevated quinolinic acid levels in cerebrospinal fluid in subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a rare neurodegenerative disorder caused by a persistent infection with aberrant measles virus. Indoleamine-2, 3-dioxygenase (IDO) initiates the increased production of kynurenine pathway (KP) metabolites quinolinic acid (QUIN), which has an excitotoxic effect for neurons. We measured serum IDO activity and cerebrospinal fluid (CSF) levels of QUIN. The CSF QUIN levels were significantly higher in SSPE patients than in controls, and increased according as neurological disability in a patient studied. Elevation of CSF QUIN and progression of SSPE indicate a pathological role of KP metabolism in the inflammatory neurodestruction.

Clinico-demographic, Investigation and Outcomes Profiles of Subacute Sclerosing Panencephalitis (SSPE) Patients at A Referral Neurology Hospital in Bangladesh

Background: SSPE is a late complication of measles and is fatal in most cases.&#x0D; Objectives: The purpose of the study was to observe the clinico-demographic, investigation and outcome profiles of SSPE patients.&#x0D; Methodology: This prospective cohort study was conducted in the Department of Neurophysiology at National Institute of Neurosciences and Hospital, Dhaka, Bangladesh from September 2014 to August 2017. Patients of SSPE who were diagnosed by Dyken's Criteria were selected as study population. Details socio-demographic, clinical profiles, investigations and outcome were recorded. The outcome was assessed from the time of first onset of the symptoms till closing of the study.&#x0D; Results: The mean age of the study population was 14.3±4.209 years with the male predominance (73.5%). Majority were from rural area (70.6%) and were of low socio-economic condition (52.9%). The mean age of onset of disease was 13.6±4.30 years. History of previous measles infection was reported in 13(38.2%) cases among which 12(92.3%) cases were vaccinated. The mean age of measles infection was 17.0±15.44 months. The time of first symptom of SSPE from primary measles infection was 11.50±4.80 years. Recurrent fall (47.1%) and myoclonic jerks (17.6%) were the most frequent initial symptoms of the patient. Cognitive decline was observed in 28(82.4%) cases. EEG background was slow in 23(67.6%) patients and periodic burst was observed in all (100.0%) cases. Anti-measles antibody in CSF was positive in all cases. MRI was abnormal in 7(25.9%) cases out of 27 cases. Twelve (35.3%) patients died, 18(52.9%) were alive and 4(11.8%) were lost to follow up when the study was closed.&#x0D; Conclusion: Early aged male from lower socioeconomic condition are found to be suffering from SSPE. Recurrent fall is the commonest initial presentation. Spontaneous recovery can occur in very few patients.&#x0D; Journal of National Institute of Neurosciences Bangladesh, 2019;5(2): 97-100

Role of the Th1 and Th17 Pathway in Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a progressive and fatal disease caused by reactivation of a mutated measles virus in brain tissue. The process of reactivation is yet to be elucidated. In this study, the possible roles of the Th1 (interleukin [IL]-12, interferon [IFN]-γ) and the Th17 axis (IL-23, IL-17, IL-22), particularly of IL-17, in the pathogenesis of SSPE were investigated. Briefly, mononuclear cells from SSPE patients were stimulated using measles virus peptide, and the release of IL-12, IL-23, IL-22, IFN-γ, and IL-17 cytokines was measured using enzyme-linked immunosorbent assay and/or enzyme-linked immunosorbent spot assay (ELISpot). We found that in comparison to the mononuclear cells obtained from healthy donors, cells from SSPE patients exhibited increased levels of IL-12, IL-23, IL-17, IL-22, and IFN-γ cytokines in response to measles virus stimulation. However, the same result was not obtained with cytomegalovirus and phytohemagglutinin. Using flow cytometry, mononuclear cells obtained from SSPE patients and healthy controls were also analyzed for the presence of intracellular IL-17 in response to measles virus stimulation. On stimulation, the number of IL-17-positive cells were found to be higher among mononuclear cells obtained from the patients. In addition, the numbers of IL-17- and IFN-γ-positive cells were significantly increased in SSPE patients. In conclusion, this study demonstrates that both the IL-12/IFN-γ and the IL-23/IL-17/IL-22 pathways are functionally abnormal in SSPE pathogenesis. Targeting these pathways and their specific pro-inflammatory mediator production may provide a new strategy to suppress SSPE development.

Subacute Sclerosing Panencephalltis: Clinical and Laboratory Manifestations

We reviewed clinical and laboratory findings of 12 cases of Subacute sclerosing panencephalitis (SSPE) hospitalized at our department from 1985 to 1991. All cases were diagnosed and hospitalized at the 2nd stage. The principal clinical manifestations were mental changes, myoclonus, and frequent falls. Other clinical manifestations were ocular changes, involuntary movements, loss of social contact, and spasticity. Diagnosis was based on suppression-burst pattern in EEG and positive antibody titer to measles in serum and cerebrospinal fluid. CT scan was not diagnotic, since it was either normal or showed only non-specific cortical atrophy. Eleven patients (91, 7%) recalled a history of measles in the past. Age of onset of SSPE varied among cases and was difficult to specify precisely due to its subtle nature. None of the cases had been vaccinated against measles.&#x0D; SSPE is a rare disease, but is almost always fatal with prolonged suffering of the patient. Based on our experience with SSPE patients, we recommend to broaden the immunization programme against measles.

Fulminant subacute sclerosing panencephalitis in an immunized 20-month-old Indian boy.

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder manifesting in school going children with cognitive decline and myoclonic jerks. It results from persistent infection of immune resistant measles virus infection. Most of the patients of SSPE have a primary measles