Subacute Sclerosing Panencephalitis

Department of Neurology, King George's Medical College, Lucknow, India.
Postgraduate Medical Journal (Impact Factor: 1.45). 03/2002; 78(916):63-70. DOI: 10.1136/pmj.78.916.63
Source: PubMed


Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Patients usually have behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The disease has a gradual progressive course leading to death within 1-3 years. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease.

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Available from: Ravindra Kumar Garg, May 10, 2014
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    • "While neuroimaging could appear normal in stages 1 and 2, magnetic resonance imaging (MRI) findings characteristically show cerebral atrophy, white matter abnormalities, and signal change in the basal ganglia [6]. The most common finding on electroencephalography (EEG) is a generalized periodic spike and waves occurring every 5–10 seconds in conjunction with myoclonic episodes [2]. "
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    ABSTRACT: We describe the clinical presentation and clinical course of subacute sclerosing panencephalitis in a 13-year-old previously healthy boy who recently immigrated to the United States from Iraq. He presented with macular retinopathy, followed by progressive myoclonus and encephalopathy. After extensive workup, a diagnosis of subacute sclerosing panencephalitis was suspected by the presence of period epileptiform discharges on electroencephalogram and confirmed by elevated measles titers in the cerebrospinal fluid. Combination immunomodulatory therapy with isoprinosine, ribavirin, and intra-Ommaya interferon alpha did not result in clinical improvement. Within days following the administration of carbamazepine, there was remarkable improvement in the myoclonus and he was able to ambulate independently for a period of 4 months at which time he unfortunately progressed to a vegetative state. This case highlights the importance of carbamazepine as a potential first line symptomatic treatment of subacute sclerosing panencephalitis and provides a review of the literature on the subject.
    Full-text · Article · Mar 2013
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    • "Neuronal loss has been reported in human cases of RV (Juntrakul et al, 2005) Hendra virus (Wong et al, 2009), and measles virus (Garg et al, 2008; McQuaid et al, 1997) infection. However, at least one study has indicated that rhabdoviruses do not have an intrinsic ability to induce apoptosis in isolated neuron-like cells (Baloul and Lafon, 2003) suggesting that these viruses elicit neuronal cell death in an indirect manner. "
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    ABSTRACT: While astrocytes produce key inflammatory mediators following exposure to neurotropic nonsegmented negative-sense RNA viruses such as rabies virus and measles virus, the mechanisms by which resident central nervous system (CNS) cells perceive such viral challenges have not been defined. Recently, several cytosolic DExD/H box RNA helicases including retinoic acid-inducible gene I (RIG-I) have been described that function as intracellular sensors of replicative RNA viruses. Here, we demonstrate that primary human astrocytes constitutively express RIG-I and show that such expression is elevated following exposure to a model neurotropic RNA virus, vesicular stomatitis virus (VSV). Evidence for the functional nature of RIG-I expression in these cells comes from the observation that this molecule associates with its downstream effector molecule, interferon promoter stimulator-1, following VSV infection and from the finding that a specific ligand for RIG-I elicits astrocyte immune responses. Importantly, RIG-I knockdown significantly reduces inflammatory cytokine production by VSV-infected astrocytes and inhibits the production of soluble neurotoxic mediators by these cells. These findings directly implicate RIG-I in the initiation of inflammatory immune responses by human glial cells and provide a potential mechanism underlying the neuronal cell death associated with acute viral CNS infections.
    Full-text · Article · Oct 2010 · Glia
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    ABSTRACT: Subacute sclerosing panencephalitis (SSPE) usually begins insidiously and follows a subacute course with relentless but slow progression to death. In recent years, however, patients with acute or fulminant course were reported. In this article, we report on three patients (2 girls, 1 boy) with SSPE who developed an acute and fulminant course. Subacute sclerosing panencephalitis may be seen with more atypical symptoms and more acute and fulminant courses due to various undetermined reasons. Early diagnosis is very important for the effectiveness of treatment. Children presenting with acute or subacute neurologic symptoms should be examined for SSPE, especially if they have no risk factors for hereditary neurodegenerative/ neurometabolic diseases, and it is more important if those children were not vaccinated or were infected with measles.
    Full-text · Article · Nov 2006 · The Turkish journal of pediatrics
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