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.

PURPOSE: Subacute sclerosing panencephalitis (SSPE) is a neurodegerative disease due to persistent measles virus infection. We investigated the role of programmed death-1 (PD-1) molecule which is related with chronic viral infection in developing SSPE in mouse. METHODS: We adopt the PD-1-/-, PD-1-/+, and wild type BALB/c 3 week old mice to make an animal model of SSPE by injecting measles virus (SSPE strain) intraventricularly. Three months after infusion of virus, the mice were sacrificed and examined if the typical pathologic lesions had been progressed. The sera were collected from each group of mice and the serum level of IL-21 was measured with ELISA kit. RESULTS: The necrotic lesions on white matter and gliosis were found in focal areas in wild type BALB/c. The extent of lesion was smaller in heterotype BALB/c. Scanty lesions were found in PD-1-/- mice. The sera level of IL-21 was not elevated in all three groups. CONCLUSION: Our data suggest that the PD-1 molecule may play a role in persistent viral infection.
Animals ; Enzyme-Linked Immunosorbent Assay ; Gene Knockout Techniques* ; Gliosis ; Measles virus ; Measles* ; Mice* ; Models, Animal ; Subacute Sclerosing Panencephalitis ; Viruses

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, and fatal central nervous system disorder resulting from persistent measles virus infection. Long-term data are scarce, with a maximum follow-up period of 10 years. Interferon-alpha (IFN-α) is a protein that exerts its antiviral activity via enhancement of cellular immune response and is reported to be an effective drug for the treatment of SSPE. However, there is currently no consensus regarding the optimal duration of IFN-α therapy. Here, we present a case report of a patient with SSPE treated with long-term intraventricular IFN-α therapy, which facilitated clinical improvement and neurological stabilization without causing serious adverse effects. To the best of our knowledge, this is one of the longest follow-up studies investigating a patient with SSPE receiving intraventricular INF-α treatment. Further studies are necessary to validate the benefits and safety of long-term intraventricular IFN-α treatment in patients with SSPE.
Central Nervous System ; Consensus ; Follow-Up Studies ; Humans ; Immunity, Cellular ; Interferon-alpha ; Measles ; Measles virus ; Subacute Sclerosing Panencephalitis ; Survivors

No abstract available.
Measles*

No abstract available.
Measles*

Measles

Immunization is the most cost-effective preventive health measure in this century. Mass Immunization Programs worldwide have eradicated small pox and significantly reduced the incidence of vaccine preventable diseases like diphtheria, tetanus, pertussis, polio, hepatitis B, Haemophilus influenzae type B disease, measles, mumps and rubella. Measles, being a vaccine preventable illness may be eliminated. We, the Philippine Pediatric Society and the Pediatric Infectious Disease Society of the Philippines, are seriously concerned by the increasing cases of measles which is a highly preventable disease affecting the most vulnerable and the ones most in need of our protection. As reported by the measles Reference Laboratory of RITM, there were 500 and 482 laboratory- confirmed cases in November and December respectively. As Pediatricians and Pediatric Infectious Disease Specialist, who are in the front lines of the battle against childhood diseases, especially vaccine-preventable diseases, this situation is unacceptable.
Measles

Subacute sclerosing panencephalitis (SSPE) is a slowly progressing, chronic persistent fatal central nervous system disease, involving gray and white matter, especially white matter caused by measles virus that affecting children and young adult. 45 to 68% of affected individuals had measles before the age of 2. Current knowledge of the pathogenesis of SSPE involves mutation of the measles virus, resulting in lack of production of the M(Matrix)-protein. No therapeutic maneuvour gas been proven conclusively to be of value. But recently intraventricular alpha-interferon (a-IFN) injection combined with oral inosiplex increase the length of survival and may bring remission or stabilization in SSPE. We report a case of SSPE which was diagnosed by history, clinical manifestation, typical EEG findings, high titer of measles antibodies in cerebrospinal fluid and serum by hemagglutinin inhibition method. We tried intraventricular a-IFN injection via Ommaya reservoir and oral inosiplex.
Antibodies ; Central Nervous System ; Cerebrospinal Fluid ; Child ; Electroencephalography ; Hemagglutinins ; Humans ; Inosine Pranobex ; Interferon-alpha* ; Measles ; Measles virus ; Subacute Sclerosing Panencephalitis* ; Young Adult

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system (CNS) caused by a persistent, aberrant measles virus infection. The outcome is usually fatal. After a latent period of 6 to 7 years, there is subtle and slow cognitive decline and visuospatial disorientation develops followed by myoclonic jerks, extrapyramidal symptoms, ataxia, and seizures, progressing to coma or vegetative state. The diagnosis is based on at least three of the following criteria 1) clinical manifestations 2) abnormal EEG 3) hyperglobulinorrachia, elevated serum or spinal fluid measles antibody 4) histologic features. No therapeutic maneuver has been proven conclusively to be of value. We have diagnosed and experienced a case of subacute sclerosing panencephalitis (SSPE) in a 5-year-old child with the chief complaint of myoclonic seizure and mental deterioration. We report a case and the brief review of related literature.
Ataxia ; Central Nervous System ; Child ; Child, Preschool ; Coma ; Diagnosis ; Electroencephalography ; Humans ; Measles ; Measles virus ; Myoclonus ; Persistent Vegetative State ; Seizures ; Subacute Sclerosing Panencephalitis*

BACKGROUND: Subacute sclerosing panencephalitis (SSPE) is a delayed and fatal manifestation of measles infection. Fulminant SSPE is a rare presentation in which the disease progresses to death over a period of 6 months. The clinical features are atypical and can be misleading. CASE REPORT: We report herein a teenage boy who presented with acute-onset gait ataxia followed by right hemiparesis that evolved over 1 month, with left-hemispheric, delta-range slowing on the electroencephalogram (EEG). Magnetic resonance imaging disclosed multiple white-matter hyperintensities, suggesting a diagnosis of acute disseminated encephalomyelitis. He received intravenous steroids, and within 4 days of hospital admission he developed unilateral slow myoclonic jerks. Repeat EEG revealed Rademecker complexes, pathognomonic of SSPE, and an elevated titer of IgG antimeasles antibodies was detected in his cerebrospinal fluid. The disease progressed rapidly and the patient succumbed within 15 days of hospitalization. The diagnosis of SSPE was confirmed by autopsy. CONCLUSIONS: This case illustrates the difficulty of recognizing fulminant SSPE when it manifests with asymmetric clinical and EEG abnormalities.
Adolescent* ; Antibodies ; Ataxia* ; Autopsy ; Cerebrospinal Fluid ; Diagnosis ; Electroencephalography ; Encephalomyelitis, Acute Disseminated ; Gait Ataxia ; Hospitalization ; Humans ; Immunoglobulin G ; Magnetic Resonance Imaging ; Male ; Measles ; Myoclonus ; Paresis* ; Steroids ; Subacute Sclerosing Panencephalitis*