inosine-pranobex and Measles

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring after infection with measles virus. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. The physiopathology of the disease is not fully understood; however, there is evidence that it involves factors that favour humoral over cellular immune response against the virus. As a result, the virus is able to infect the neurons and to survive in a latent form for years. The clinical manifestations occur, on average, 6 years after measles virus infection. The onset of SSPE is insidious, and psychiatric manifestations are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. The diagnosis is clinical, supported by periodic complexes on electroencephalography, brain imaging suggestive of demyelination, and immunological evidence of measles infection. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis.

Topics: Antibodies, Viral; Antiviral Agents; Apoptosis; Brain; Demyelinating Diseases; Disease Progression; Drug Therapy, Combination; Electroencephalography; Epilepsies, Myoclonic; Female; Gliosis; Humans; Inosine Pranobex; Interferon-alpha; Magnetic Resonance Imaging; Male; Measles; Measles Vaccine; Measles virus; Ribavirin; Severity of Illness Index; Sex Factors; Subacute Sclerosing Panencephalitis; Time Factors; Virion

Topics: Adolescent; Adrenal Cortex Hormones; Adrenocorticotropic Hormone; Chickenpox; Child; Encephalitis; Enterovirus Infections; Female; Herpesviridae Infections; Humans; Hypnotics and Sedatives; Influenza, Human; Inosine Pranobex; Male; Measles; Mumps; Rubella; Virus Diseases

Topics: Amantadine; Animals; Benzimidazoles; Chickenpox; Cytarabine; gamma-Globulins; Hepatitis, Viral, Human; Herpes Simplex; Herpes Zoster; Humans; Idoxuridine; Inosine Pranobex; Interferons; Measles; Mumps; Phosphonoacetic Acid; Ribavirin; Rifampin; Rubella; Vaccination; Vaccinia virus; Vidarabine; Virus Diseases

During the past five years, 15 patients with subacute sclerosing panencephalitis (SSPE) were treated with inosiplex. Using a disability index specifically designed for the disease, this study monitored the course of SSPE in each patient before and during inosiplex therapy. Posttreatment follow-up ranged from 2 to 144 months. Inosiplex had an apparently beneficial effect on morbidity and mortality in 10 of the 15 patients tested. Eight improved immediately after treatment, 2 had a delayed improvement, and 1 patient stabilized. Four patients followed a typical course for SSPE and died a mean 9 months after onset. Treatment was not associated with adverse reactions. Due to its low risk-benefit ratio, inosiplex is recommended for continuous use in SSPE even after extended remissions.

Topics: Adolescent; Child; Child, Preschool; Clinical Trials as Topic; Female; Follow-Up Studies; Humans; Inosine; Inosine Pranobex; Male; Measles; Neurologic Examination; Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence caused by persistent defective measles virus. Clinical manifestations appear many years after the acute measles infection. The incidence of SSPE has substantially declined after the introduction of an effective vaccine. We report a case of a child with SSPE that began with atonia, dysarthria, and intellectual deterioration without the presence of any particular EEG anomalies. We have reported this girl who was affected by this severe affliction in the hope that, because of the rarity of SSPE, it would not go undiagnosed.

Topics: Antiviral Agents; Child; Disease Progression; Drug Therapy, Combination; Dysarthria; Electroencephalography; Female; Humans; Inosine Pranobex; Interferon-beta; Measles; Measles Vaccine; Prognosis; Severity of Illness Index; Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection of immune-resistant measles virus. Diagnostic hallmarks include widespread cortical dysfunction on EEG, myoclonus, white matter abnormalities on neuroradiological examination and the presence of IgG anti-measles antibodies in the cerebrospinal fluid. We present the first case of SSPE with signs of peripheral nerve hyperexcitability, observed as extra discharges following the compound motor action potential at motor nerve stimulation. In addition we demonstrate the importance of SSPE in the differential diagnosis of adult patients with psychiatric and neurological symptoms.

Topics: Action Potentials; Adult; Antiviral Agents; Brain; Catatonia; Diagnosis, Differential; Disease Progression; Electrodiagnosis; Fatal Outcome; Female; Humans; Inosine Pranobex; Magnetic Resonance Imaging; Measles; Neural Conduction; Peripheral Nerves; Peripheral Nervous System Diseases; Persistent Vegetative State; Subacute Sclerosing Panencephalitis; Treatment Failure

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system with poor prognosis and high mortality. No effective treatment has a proven role; oral isoprinosine and intrathecal administration of alpha-interferon may prolong survival. We report an unusual case of adult onset SSPE patient on treatment with significant clinical improvement, even in the absence of conversion to seronegativity in either CSF or serum, on follow-up serological examination.

Topics: Adult; Antibodies, Viral; Antiviral Agents; Female; Humans; Inosine Pranobex; Interferon-alpha; Measles; Measles virus; Subacute Sclerosing Panencephalitis; Treatment Outcome

Subacute sclerosing panencephalitis (SSPE) is a rare entity with an invariably fatal course that progressively affects the central nervous system. It is caused by persistent infection with the wild-type measles virus. While rare in industrial countries, it is not infrequent in developing countries, where there are still areas of endemic measles infection and immunization is not yet generalized. We describe an eight-year-old Spanish girl who presented rhythmic and symmetric myoclonus. She contracted measles at 13 months and required hospitalization. No cognitive deterioration was found. Neuroimaging and the initial electroencephalogram were normal. Oligoclonal bands and high titers of measles antibodies were found in serum and cerebrospinal fluid. She was treated with oral metisoprinol and intraventricular alpha-interferon (IFN-) and showed no further progression of her symptoms. The importance of including SSPE in the differential diagnosis of patients consulting for school failure, neurological deterioration or movement disorders is highlighted. Special attention should be paid to the immigrant population from countries where the incidence of SSPE is greater than in Spain.

Topics: Antibodies, Viral; Antiviral Agents; Child; Diagnosis, Differential; Electroencephalography; Epilepsies, Myoclonic; Female; Humans; Inosine Pranobex; Interferon-alpha; Measles; Movement Disorders; Subacute Sclerosing Panencephalitis

We report an 18-month-old girl with rapidly progressive subacute sclerosing panencephalitis, whose non immunised mother had measles at the time of delivery. The patient presented with repetitive episodes of myoclonic jerks of the head and arms, followed by a drop of head and trunk with frequent falls. EEG, CSF studies and MRI confirmed the diagnosis. Despite therapy with isoprinosine and valproate, seizure activity continued and she became vegetative within 2 months, with severe spasticity and swallowing difficulties, and died at the age of 28 months. Early age of onset and rapid progression were most likely related to haematogenous in utero acquisition of the measles virus prior to delivery, as well as immaturity of neuronal and immune systems.. this case emphasises the importance of a high measles vaccine coverage in the population in order to prevent the risk of disease in general and, in particular, gestational measles.

Topics: Age of Onset; Anticonvulsants; Antiviral Agents; Disease Progression; Electroencephalography; Fatal Outcome; Female; Humans; Infant; Infectious Disease Transmission, Vertical; Inosine Pranobex; Magnetic Resonance Imaging; Measles; Myoclonus; Risk Factors; Subacute Sclerosing Panencephalitis; Valproic Acid

Incorporation of thymidine by measles infected PHA-activated lymphocytes was found to be depressed although production of interleukin 2 (IL-2) and expression of IL-2 receptors on these cells was similar to that of non-infected cells. The decrease in incorporation of 3H-thymidine by infected cells was not due to cell death and could be restored by treating the cells with isoprinosine or 5-fluoro-2-deoxyuridine. These results suggest that the depressed incorporation of 3H-thymidine by measles-infected cells is not due to inhibition of early events required for lymphocyte proliferation, but is rather due to interference in the thymidine pathway required for the synthesis of DNA.

Topics: Antigens, Surface; Cell Survival; DNA; Floxuridine; Humans; Inosine; Inosine Pranobex; Lymphocyte Activation; Lymphocytes; Measles; Receptors, Immunologic; Receptors, Interleukin-2; Thymidine; Tumor Necrosis Factor Receptor Superfamily, Member 7

Topics: Adolescent; Antibodies; Electroencephalography; Humans; Inosine; Inosine Pranobex; Male; Measles; Subacute Sclerosing Panencephalitis

Topics: Child, Preschool; Electroencephalography; Encephalitis; Humans; Inosine Pranobex; Male; Measles; Measles virus

Lymphocyte subpopulations in peripheral blood (PB) and cerebrospinal fluid (CSF) from 26 children affected with subacute sclerosing panencephalitis (SSPE) and from 13 controls with various neurological diseases without any immunological implication were examined for surface markers. SSPE patients were found to have significantly lower %s of E-rosette forming cells (RFC) (T lymphocytes) and of EA-RFC (TG lymphocytes, suppressor T cells) in both CSF and PB. No difference was found in EAC-RFC (B lymphocytes) either in CSF or PB. The low EA-RFC values can be explained by genetic factors, immune complexes or virus infection, and they could account for the hypersynthesis of oligoclonal immunoglobulins in the central nervous system. Longitudinal studies performed in 6 SSPE patients during isoprinosine therapy revealed a time-dependent decrement of the %s of E- and EA-RFC in CSF. It cannot be affirmed whether this is related to the disease or to the therapy.

Topics: Antibodies, Viral; B-Lymphocytes; Child; Female; Humans; Immunity, Cellular; Immunoglobulin G; Inosine Pranobex; Lymphocyte Activation; Lymphocytes; Male; Measles; Rosette Formation; Subacute Sclerosing Panencephalitis; T-Lymphocytes; T-Lymphocytes, Regulatory

An open therapeutic trial of isoprinosine was conducted in 15 patients with subacute sclerosing panencephalitis (SSPE). Long-term remissions occurred in 5 (33 percent), with documented improvement sustained for 2 or more years. Another patient was in remission 9 months after starting treatment, and three patients had transient remissions or stabilization. The disease was unaltered in five patients who had rapidly progressive SSPE when treatment started. These results compare with an average remission rate of about 5 percent in several series of untreated cases of SSPE or in cases treated with other antiviral agents. Patients in remission continued to have elevated cerebrospinal fluid (CSF) IgG and measles antibody titers, with one exception. Isoprinosine was tolerated for several years without side effects, except for mild hyperuricemia.

Topics: Adolescent; Adult; Antibodies, Viral; Child; Electroencephalography; Female; Humans; Immunoglobulin G; Inosine; Inosine Pranobex; Male; Measles; Reading; Subacute Sclerosing Panencephalitis; Wechsler Scales; Writing

Topics: Animals; Child; Cricetinae; Female; Humans; Inosine Pranobex; Male; Measles; Measles Vaccine; Subacute Sclerosing Panencephalitis