Combined Interferon-α and Inosiplex Treatment of Subacute Sclerosing Panencephalitis: A Case Report (original) (raw)
Related papers
Combined treatment with interferon-alpha and ribavirin for subacute sclerosing panencephalitis
Pediatric Neurology, 2001
Two patients with subacute sclerosing panencephalitis (SSPE) are described. They were diagnosed on admission to the hospital with SSPE, as judged on cerebrospinal fluid examination involving reverse transcription followed by polymerase chain reaction, at the second stage of Jabbour's classification. They first were treated with intraventricular interferon-alpha monotherapy; however, the combination of interferonalpha and IV ribavirin was started at 8 and 5 months after beginning the interferon-alpha monotherapy, respectively. Although slow progressive brain atrophy was observed in Patient 1 on brain magnetic resonance imaging before the ribavirin therapy, no further progression was noted 11 months after starting combination therapy with ribavirin. The event-related potential study results and audiography of the right ear improved in Patient 1 after the combination therapy was initiated. In Patient 2 the hypertonicity, neurobladder incontinence, and dysphagia improved 3 months after starting the combination treatment. Although this group of patients is small, these results suggest treatment with intrathecal high-dose interferon-alpha and IV ribavirin is effective in the treatment of SSPE. Early administration of intrathecal high-dose interferon-alpha and IV ribavirin should be considered as a possible therapy for SSPE patients, especially interferon-nonresponding ones. © 2001 by Elsevier Science Inc. All rights reserved.
Brain and Development, 1993
Eighteen patients, 16 boys and 2 girls, aged 5-14 years, with subacute sclerosing panencephalitis (SSPE) were treated with oral isoprinosine (100 mg/kg/day) and intraventricular alpha-interferon 2b (Intron A, Schering Corp.), starting at 500,000 U twice a week and later increasing to 3 million U biweekly. Minimal follow-up of living patients is 12 months; maximal 40 months. On the basis of the Neurological Disability Index (NDI) scores and staging, 8 have treatment-induced remissions (3 improved, 5 arrested), 4 are worse and 6 died. This 44% (8/18) rate of remission/improvement compares well with the 9% (1/11) remission in historical controls in the same institution (p = < 0.05) and 5% spontaneous remission in the literature. Combined oral isoprinosine-intraventricular alpha-interferon appears to be an effective treatment for SSPE.
A Multinational Survey on Actual Diagnostics and Treatment of Subacute Sclerosing Panencephalitis
Neuropediatrics, 2015
Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system caused by the measles virus (MV). Its prevalence remains high in resource poor countries and is likely to increase in the Northern Europe as vaccination rates decrease. Clinical knowledge of this devastating condition, however, is limited. We therefore conducted this multinational survey summarizing experience obtained from more than 500 patients treated by 24 physicians in seven countries. SSPE should be considered in all patients presenting with otherwise unexplained acquired neurological symptoms. In most patients, the diagnosis will be established by the combination of typical clinical symptoms (characteristic repetitive myoclonic jerks), a strong intrathecal synthesis of antibodies to MV and typical electroencephalogram findings (Radermecker complexes). Whereas the therapeutic use of different antiviral (amantadine, ribavirin) and immunomodulatory drugs (isoprinosine, interferons) an...
Subacute sclerosing panencephalitis: case based review
International Journal of Research in Medical Sciences
Subacute sclerosing panencephalitis (SSPE) is a chronic debilitating condition that occurs in children affected with measles. SSPE is broadly distinguished as typical SSPE, the more rampant form, occurring over a period of years following primary measles infection, while the atypical has a more rapidly progressive course over weeks to months. SSPE can present with cognitive, epileptic, autonomic, pyramidal and ophthalmologic manifestations with scholastic decline being the primary feature. The management of SSPE focuses on improvement of quality of life and prolongation of survival which can be achieved with the use of supportive care modalities and immunomodulators respectively. This is a comprehensive review which discusses several parameters of SSPE such as epidemiology, pathophysiology, clinical presentations, and detailed management protocol for this condition. As part of this review, we also discuss a case of rapidly progressive, fulminant and atypical SSPE in a five-year-old ...
Subacute sclerosing panencephalitis--the continuing threat
Collegium antropologicum, 2006
Clinical, epidemiological and laboratory findings of four patients with subacute sclerosing panencephalitis (SSPE), diagnosed in Croatia in 2002, were examined. Patient age at disease onset ranged from 5-11 years. All patients were vaccinated regularly with MMR-vaccine. Two patients had a history of measles infection at the age of six and seven months, respectively. In the other two patients, the disease started immediately after the varicella infection. Complement fixing antibody titre to the measles virus (MV) ranged from 1:1024 to 1:65536 in serum, and from 1:16 to 1:128 in cerebrospinal fluid (CSF). In CSF, no antibodies to varicella-zoster virus were found. Brain tissue samples were obtained at autopsy from two patients. In one patient, electron microscopy demonstrated intranuclear viral inclusions (MV nucleocapsids). MV antigen was detected in brain imprints using IFA in both of them. Viral RNA was found in brain tissue samples only, while plasma, serum and CSF were negative. ...
Subacute sclerosing panencephalitis: A clinical appraisal
Annals of Indian Academy of Neurology, 2013
SSPE generally follows the prevalence of measles in a population. The incidence rate in the USA in 1960 was 0.61 cases/million persons younger than 20 year. By 1980, the rate had fallen to 0.06 cases/million. [4] Incidence is still high in developing countries though variable, in India estimated incidence by Saha et al. [5] was 21/million population while an annual incidence of 1.5/million was reported in the Middle East. [6] Measles is primarily disease of childhood with age of onset before 2 years. After a latent period of 6-8 years, it is followed by the onset of progressive neurological symptoms suggestive of SSPE. The measles vaccine has changed the epidemiology of measles dramatically from the erstwhile worldwide distribution to a rare disorder in developed countries. As a result of subclinical measles infection before the age of 1 year, occasionally, it can be seen in vaccinated children. There is no evidence to suggest that attenuated vaccine virus is responsible for sporadic cases of SSPE. [6,7] Individuals with acquired immunodeficiency syndrome (AIDS) or children whose mothers have AIDS might be at higher risk of a fulminant course and earlier onset of SSPE. [8] The aim of the present study is to describe the clinical profile and natural history of patients with SSPE after improvement in measles vaccination and to see any change in clinical characteristics.
Fulminant subacute sclerosing panencephalitis:two cases with atypical presentation
Pediatric Neurology, 2003
From 1972 to 2002, we diagnosed and treated 22 cases of subacute sclerosing panencephalitis. We report on two pediatric patients with fulminant subacute sclerosing panencephalitis who had atypical clinical manifestations. In both patients diagnosis was confirmed by elevated titers of CSF and serum antimeasles antibodies. Patient 1 presented with behavioral disorder, dysarthria, and drop attacks, while Patient 2 presented with partial complex seizures. Mental difficulties, personality changes, or myoclonus were not noticed in Patient 2. In both our patients stage I was not prominent, and stage II was of shortened duration. In spite of treatment with isoprinosine and interferonalpha, both our patients deteriorated rapidly and died 2.5 and 4 months, respectively, after the onset of neurologic symptoms. Both atypical presentation and rapid clinical course observed in our patients could cause problems in making final diagnosis of subacute sclerosing panencephalitis. Therefore, subacute sclerosing panencephalitis should be included in differential diagnosis of acute unexplained encephalopathic diseases.
Subacute sclerosing panencephalitis: clinical and demographic characteristics
Journal of the College of Physicians and Surgeons--Pakistan : JCPSP, 2014
OBJECTIVE To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE). STUDY DESIGN Case series. PLACE AND DURATION OF STUDY The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. METHODOLOGY A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. RESULTS were expressed as percentages. RESULTS Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients (39.5%). All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 (51%) patients. CSF for antimeasles antibodies was ...