Intraventricular interferon and oral inosiplex in the treatment of subacute sclerosing panencephalitis

Yalaz, Kalbiye; Anlar, Banu; Öktem, Ferhunde; Aysun, Sabiha; Ustaçelebi, S; O, Gürçay; Gücüyener, Kıvılcım; Renda, Yavuz

doi:10.1212/wnl.42.3.488

Cited by 117 publications

(64 citation statements)

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“…This can be partly explained by the delay on the diagnosis, as it seems in some clinical trials that if the treatment can be effective, it is only in early stages 12,13 . We had two cases of spontaneous remission, the first one was followed for 9 years and had an absolutely normal neuropsychological development, he was treated with isoprinosine for 12 months and valproate.…”

Section: Discussionmentioning

confidence: 99%

Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry

Nunes

Costa

Stancher

et al. 1999

Arq. Neuro-Psiquiatr.

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-Subacute sclerosing panencephalitis (SSPE) is an inflammatory neurodegenerative disease related to the persistence of measles virus. Although its frequency is declining because of measles eradication, we still have some cases being diagnosed. With the aim to describe epidemiological aspects of SSPE in Brazil, we sent a protocol to Child Neurologists around the country, 48 patients were registered, 27 (56 %) were from the southeast region, 34 (71%) were male and 35 (73%) white, 27 (56%) had measles, 9 (19%) had measles and were also immunized, 7 (14%) received only immunization, 1 patient had a probable neonatal form. Mean time between first symptoms and diagnosis was 12 months (22 started with myoclonus or tonic-clonic seizures, 7 (14%) with behavioral disturbances); 36 patients (75%) had EEG with pseudoperiodic complexes. Follow up performed in 28 (58 %) patients showed: 12 died, 2 had complete remission and the others had variable neurological disability Our data shows endemic regions in the country, a high incidence of post-immunization SSPE and a delay between first symptom and diagnosis.KEY WORDS: immunization, measles, seizures, subacute sclerosing panencephalitis Panencefalite esclerosante subaguda, aspectos clínicos e prognóstico: Registro Brasileiro RESUMO -A panencefalite esclerosante subaguda é doença neurodegenerativa inflamatória relacionada à persistência do vírus do sarampo no organismo. Sua incidência vem diminuindo significativamente com a erradicação do sarampo, mas eventualmente alguns casos ainda têm sido diagnosticados. Com o objetivo de descrever aspectos epidemiológicos da panencefalite no Brasil contactamos Neurologistas Infantis de todo país. Foram registrados 48 pacientes, 27% da região sudeste, 34 (70%) do sexo masculino, 35 (73%) brancos, 9 (19%) apresentaram sarampo e receberam imunização, 7 (14%) somente imunizados, um paciente apresentou provável forma neonatal. Intervalo médio entre primeiro sintoma e diagnóstico de 12 meses, 22 pacientes (45%) iniciaram o quadro com mioclonus ou convulsões tônico-clônicas, 7 (14%) com distúrbios comportamentais; 36 (75%) apresentaram EEG com complexos pseudoperiódicos. Seguimento de 28 pacientes (57%) demonstrou 12 óbitos, 2 remissões completas, demais com sequelas neurológicas. Nossos resultados evidenciam regiões endêmicas no país, alta incidência de casos pós-imunização e demora na confirmação do diagnóstico. PALAVRAS-CHAVES: imunização, sarampo, convulsões, panencefalite esclerosante subaguda.Subacute sclerosing panencephalitis (SSPE) is an inflammatory neurodegenerative disease related to the persistence of an aborted form of the wild measles virus. Clinical presentation of the disease is more fully documented at this time but the major etiological and pathological bases of SSPE remain only partially understood 1,2 . The speed of progression has been designated as acute (development of at least 66% neurologic disability within 3 months of the first neurologic symptoms,

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Section: Discussionmentioning

confidence: 99%

Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry

Nunes

Costa

Stancher

et al. 1999

Arq. Neuro-Psiquiatr.

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“…Recent therapeutic successes for SSPE with interferon, and interferon-isoprinosine [9], have been reported, providing impetus for early diagnosis, particularly in cases with atypical history, course, and laboratory findings. This infant had illness onset in PIV season, 4 months after measles immunization.…”

Section: Discussionmentioning

confidence: 99%

“…Cases associated with parainfluenza virus (PIV), relatively common in eastern Europe and the Middle East [4][5][6], have recently been reported in this country [7]. New therapies, e.g., interferon, hold hope for affected individuals, making timely diagnosis important [8,9]. We report an infant who presented in PIV season with no cerebrospinal fluid (CSF) measles antibodies and document the diagnostic utility of molecular analysis of the virus.…”

mentioning

confidence: 98%

Subacute sclerosing panencephalitis in an infant: Diagnostic role of viral genome analysis

Baram

González-Gómez

Xie

et al. 1994

Annals of Neurology

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Subacute sclerosing panencephalitis (SSPE) is related to "defective" measles virus or vaccination, though an association with parainfluenza viruses has been reported. SSPE is characterized by a slow, erratic course and elevated cerebrospinal fluid measles titers. An immunocompetent, vaccinated infant, with onset of symptoms in parainfiuenza virus season and a catastrophic course is described. Cerebrospinal fluid titers were negative, but postmortem brain had typical SSPE lesions. Patient brain-derived RNA, subjected to reverse transcription followed by polymerase chain reaction yielded polymerase chain reaction products with measles virus but not parainfluenza virus genes. The sequenced fragment revealed multiple mutations, typical for SSPE. SSPE can thus present in infants, with short latency and no cerebrospinal fluid antibodies. Viral genomic analysis may be diagnostic, permitting early therapy.The incidence of subacute sclerosing panencephalitis (SSPE), a fatal neurodegenerative disease most commonly associated with prior measles infection [1,2], has significantly decreased in the United States [3]. Since the introduction of measles vaccines, age of onset and vaccine-associated cases have increased [3]. Cases associated with parainfluenza virus (PIV), relatively common in eastern Europe and the Middle East [4][5][6], have recently been reported in this country [7]. New therapies, e.g., interferon, hold hope for affected individuals, making timely diagnosis important [8,9]. We report an infant who presented in PIV season with no cerebrospinal fluid (CSF) measles antibodies and document the diagnostic utility of molecular analysis of the virus.Copyright © 1994 by the American Neurological Association. All rights reserved. RNA Preparation. Reverse Transcription-Polymerase Chain Reaction and SequencingFrozen brain tissue specimens were ground in liquid nitrogen, and total cellular RNA was extracted. RNA samples from measles virus (MV), or PIV type 1-, 2-, or 3-infected Vero cells, were used as positive controls. Reverse transcription-polymerase chain reaction (RT-PCR) was performed as described [10], using published primers specific for measles matrix and fusion protein [11] genes. Three pairs of measles nucleocapsid protein genespecific nested primers, as follows, were developed: MN7/MN8 (5 -CAATCTGGCCTTACCTTCGCAT-3 / 5 -CTCCTTACCATCTCTTGCCCTA-3 ); MV1/ MV2(5 -GCTAAGAAGGTGGATAAA-3 / 5 -TATGCTGGATCAAAGTAAG-3 ), and MV3/MV4 (5 -TTGGAGAGAAAATGGTTGGA-3 / 5 -CAGAGCAGAGGGTATGAT-3 ). Also developed was a primer pair, as follows, recognizing conserved PIV-1, 2, and 3 nucleocapsid gene: PIN1/PIN2 (5 -AATGCTGATGTCAAGTATGT-3 / 5 -ATCCTGTCTGAATGCTTCTAA-3 )-A 410-bp fragment of the matrix gene PCR product derived from two brain specimens was sequenced along with a wild-type isolate. Other ProceduresMagnetic resonance (MR) images (5 mm) were obtained on a 0.5-T magnet. CSF MV IgG and IgM antibody titers were performed by MRL (Cypress, CA); aliquots were cultured for viruses, including PIV. Brain specimens were stain...

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“…Other agents such as Ribavirin, which blocks RNA polymerase without and in combination with INF, Anti CD2O antibodies, THF, Amantadine, Steroids and plasmapehresis showed some beneficial effect in selected non-randomized trials. Several patients have been treated with intraventricular INF-α alone or in combination with oral Isoprinosine but with no advantage over the intrathecal route (Yalaz et al, 1992).…”

Section: Treatmentmentioning

confidence: 99%

Subacute Sclerosing Pan-Encephalitis (SSPE) – Past and Present

Gadoth¹

2011

Pathogenesis of Encephalitis

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Intraventricular interferon and oral inosiplex in the treatment of subacute sclerosing panencephalitis

Cited by 117 publications

References 0 publications

Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry

Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry

Subacute sclerosing panencephalitis in an infant: Diagnostic role of viral genome analysis

Subacute Sclerosing Pan-Encephalitis (SSPE) – Past and Present

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