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C Paolozzi - One of the best experts on this subject based on the ideXlab platform.
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progressive rubella Panencephalitis follow up eeg study of a case
Acta neurologica, 1992Co-Authors: A Guizzaro, E Volpe, F Bravaccio, R Cotrufo, C PaolozziAbstract:: Progressive rubella Panencephalitis is a very rare slow virus disease of the nervous system. The authors present a case, concerning a young man, aged 20 years, died 11 months after the onset of the disease. The following peculiarities of the case are emphasized: 1) the clinical symptomatology and the evolution (myoclonus, lack of cerebellar impairment) could suggest the diagnosis of SSPE; 2) the EEG recordings showed epileptiform abnormalities, long latency diffuse periodic complexes and--during interferon therapy and simultaneously with a temporary clinical improvement--the appearance of short latency anterior periodic complexes.
Banu Anlar - One of the best experts on this subject based on the ideXlab platform.
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The Turkish Journal of Pediatrics 2007; 49: 295-300 Case Atypical presentations of SSPE: a clinical study in four cases
2015Co-Authors: Ercan Demir, Banu Anlar, Ayşe Aksoy, Fatma Müjgan SönmezAbstract:Subacute sclerosing Panencephalitis (SSPE) is a progressive, fatal disease of the central nervous system caused by a persistent measles virus. It is clinically characterized by insidious onset of intellectual deterioration and behavioral changes followed by myoclonias and eventually complete neurologic deterioration. The diagnosis is based on characteristic clinical features, periodic electroencephalography (EEG) complexes of high slow waves and increased antibody titer against measles in cerebrospinal fluid. Here, we report four SSPE cases, two of whom manifested with hemiparesis; in the third and fourth cases, cerebellar ataxia and acute encephalopathy with focal seizures were the presenting symptoms at the onset of disease, respectively. The typical periodic EEG complexes in our patients led to the diagnosis of SSPE. Our findings show that SSPE should be considered in the differential diagnosis of hemiparesis, cerebellar ataxia and acute encephalopathy, and highlight the diagnostic significance of EEG in unidentified cases. Key words: subacute sclerosing Panencephalitis, hemiparesis, ataxia, encephalopathy
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subacute sclerosing Panencephalitis and chronic viral encephalitis
Handbook of Clinical Neurology, 2013Co-Authors: Banu AnlarAbstract:Subacute sclerosing Panencephalitis (SSPE) is a chronic infection of the central nervous system associated with the presence of mutant measles virus in the brain. It presents as a progressive, usually fatal disease. The diagnosis is based on clinical criteria and an elevated titer of measles antibodies in the cerebrospinal fluid (CSF). Electroencephalography and imaging studies provide supportive laboratory data. A brain biopsy is indicated only when CSF serology is negative or equivocal in a suspected case to assess the presence of inclusion bodies, measles virus antigens, or viral RNA. Among many drugs and methods tried in the treatment, the highest rate of stabilization or improvement was obtained with intraventricular human lymphoblastoid interferon-α and oral inosiplex. Further research for more available and efficient therapeutic regimens is warranted. Measles and SSPE are preventable by maintenance of high rates of immunization in the population.
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Changing epidemiological features of subacute sclerosing Panencephalitis
Infection, 2001Co-Authors: Banu Anlar, Yavuz Gurer, G. Kose, Senay Haspolat, Sakir Altunbasak, Melih OkanAbstract:BACKGROUND: Subacute sclerosing Panencephalitis (SSPE) is a chronic central nervous (CNS) system infection caused by measles virus. Because changing immunization practices affect the epidemiology of measles and consequently SSPE, we examined the epidemiological data of our SSPE registry. MATERIALS AND METHODS: Age of onset, age at onset of measles, duration of Latent period and immunization status were examined in cases recorded at the SSPE Registry Center in Turkey between 1975 and 1999. RESULTS: Age of onset diminished from 13 years before 1994 to 7.6 years after 1995; age at onset of measles declined from 29 months to 20 months and the Latent interval from 9.9 years to 5.9 years. Age at onset of measles and immunization status did not directly affect the duration of the Latent period. CONCLUSION: Although its incidence has decreased in Turkey, SSPE has been seen at younger ages in recent years. This change cannot be attributed solely to younger age at onset of measles. Factors affecting the duration of the Latent period should be investigated further.
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mri findings in subacute sclerosing Panencephalitis
Neurology, 1996Co-Authors: Banu Anlar, G. Kose, Isil Saatci, Kalbiye YalazAbstract:Thirty-four MRI studies of 26 patients with subacute sclerosing Panencephalitis are reported. Lesions of high signal intensity on T 2-weighted images are the most common finding; they frequently involve the periventricular or subcortical white matter. Lesions tend to start in the cortex-subcortical white matter and progress with periventricular white matter involvement and diffuse cerebral atrophy. Pial and parenchymal contrast enhancement, local mass effect of parenchymal lesions, and involvement of the splenic portion of the corpus callosum are not infrequent. Basal ganglia and brainstem lesions were rare in this series. Although cortical and subcortical lesions have some correlation with clinical findings, the extent and location of the periventricular white matter lesions and cerebral atrophy did not reflect the neurologic status in many patients. NEUROLOGY 1996;47: 1278-1283
Pratibha Singhi - One of the best experts on this subject based on the ideXlab platform.
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Nonconvulsive Status Epilepticus on Electroencephalography: An Atypical Presentation of Subacute Sclerosing Panencephalitis in Two Children
2016Co-Authors: Pratibha Singhi, Arushi Gahlot Saini, Jitendra Kumar SahuAbstract:Copyright © 2012 Pratibha Singhi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Subacute sclerosing Panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography. We report two pediatric cases who presented with periodic myoclonic jerks and cognitive decline. In both cases, the electroencephalogram showed continuous nonconvulsive status epilepticus activity. Both had elevated measles antibodies in cerebrospinal fluid and blood. Pediatricians need to be aware of this atypical presentation of subacute sclerosing Panencephalitis. 1
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subacute sclerosing Panencephalitis presenting as acute cerebellar ataxia and brain stem hyperintensities
European Journal of Paediatric Neurology, 2016Co-Authors: Arushi Gahlot Saini, Sameer Vyas, Naveen Sankhyan, Jitendra Kumar Sahu, Hansashree Padmanabh, Pratibha SinghiAbstract:Abstract Background Subacute sclerosing Panencephalitis is a devastating neurodegenerative disease with a characteristic clinical course. Atypical presentations may be seen in 10% of the cases. Aims To describe the atypical clinical and radiological features of SSPE in a child form endemic country. Methods A 5-year-old boy presented with acute-onset cerebellar ataxia without associated encephalopathy, focal motor deficits, seizures or cognitive decline. He had varicella-like illness with vesicular, itchy truncal rash erupting one month prior to the onset of these symptoms. He underwent detailed neurological assessment, relevant laboratory and radiological investigations. Results Neuroimaging revealed peculiar brain stem lesions involving the pons and cerebellum suggestive of demyelination. With a presumptive diagnosis of clinically isolated syndrome of demyelination, he was administered pulse methylprednisolone (30 mg/kg/day for 5 days). Four weeks later he developed myoclonic jerks. Electroencephalogram showed characteristic periodic complexes time-locked with myoclonus. CSF and serum anti-measles antibody titres were elevated (1:625). Conclusion Our report highlights that subacute sclerosing Panencephalitis can present atypically as isolated acute cerebellar ataxia and peculiar involvement of longitudinal and sparing of transverse pontine fibres. The predominant brainstem abnormalities in the clinical setting may mimick acute demyelinating syndrome. Hence, it is important to recognize these features of subacute sclerosing Panencephalitis in children, especially in the endemic countries.
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Blindness, Dancing Extremities, and Corpus Callosum and Brain Stem Involvement: An Unusual Presentation of Fulminant Subacute Sclerosing Panencephalitis
Journal of Child Neurology, 2014Co-Authors: Pratibha Singhi, Pankaj Gupta, Arushi Gahlot Saini, Naveen Sankhyan, Sameer VyasAbstract:A 4-year-old girl presented with acute visual loss followed 2 weeks later with loss of speech and audition, fulminant neuroregression, and choreo-athetoid movements of extremities. Fundus showed bilateral chorioretinitis. Electroencephalography showed periodic complexes. Measles antibody titers were elevated in both serum and cerebrospinal fluid, consistent with subacute sclerosing Panencephalitis. Neuroimaging showed discontiguous involvement of splenium of the corpus callosum and ventral pons with sparing of cortical white matter. Our case highlights the atypical clinical and radiologic presentations of subacute sclerosing Panencephalitis. Pediatricians need to be aware that necrotizing chorioretinitis in a child and/or atypical brain stem changes could be the heralding feature of this condition in endemic countries.
A Guizzaro - One of the best experts on this subject based on the ideXlab platform.
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progressive rubella Panencephalitis follow up eeg study of a case
Acta neurologica, 1992Co-Authors: A Guizzaro, E Volpe, F Bravaccio, R Cotrufo, C PaolozziAbstract:: Progressive rubella Panencephalitis is a very rare slow virus disease of the nervous system. The authors present a case, concerning a young man, aged 20 years, died 11 months after the onset of the disease. The following peculiarities of the case are emphasized: 1) the clinical symptomatology and the evolution (myoclonus, lack of cerebellar impairment) could suggest the diagnosis of SSPE; 2) the EEG recordings showed epileptiform abnormalities, long latency diffuse periodic complexes and--during interferon therapy and simultaneously with a temporary clinical improvement--the appearance of short latency anterior periodic complexes.
Y Yavuz K Gurer - One of the best experts on this subject based on the ideXlab platform.
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ocular findings in subacute sclerosing Panencephalitis
Ocular Immunology and Inflammation, 2011Co-Authors: Deniz Yuksel, Nesrin şenbil, Pelin Atmaca Sonmez, Deniz Yilmaz, Y Yavuz K GurerAbstract:Purpose: The aim of this study was to evaluate the ocular manifestations of subacute sclerosing Panencephalitis (SSPE) and to investigate possible risk factors in a relatively large series of patients.Methods: In this prospective study, patients who were diagnosed with SSPE and had undergone ophthalmic examination were included.Results: Of the 59 patients with SSPE, 25 (42.4%) had ocular findings. Ocular involvement was bilateral in 22 (88%) patients. The most characteristic fundus findings in our patients were chorioretinitis. No significant difference was found between the two groups in terms of age, gender, clinical stage, and ocular findings.Conclusions: Although ophthalmological findings do not constitute a part of the diagnostic criteria, and the correlation between the clinical stage of SSPE and the ocular findings is unclear, patients with SSPE should be screened and followed up for ocular involvement.
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nonconvulsive status epilepticus on electroencephalography in a case with subacute sclerosing Panencephalitis
Journal of Child Neurology, 2006Co-Authors: Omer Faruk Aydin, Nesrin şenbil, Y Yavuz K GurerAbstract:Subacute sclerosing Panencephalitis is a neurodegenerative disease with a poor prognosis. We report a case of a 5 1/2-year-old boy who had emotional lability, cognitive difficulties, and myoclonia after a mild closed head injury. The magnetic resonance image of the brain and computed tomographic scan of the head were normal. His electroencephalogram (EEG) showed continuous nonconvulsive status epilepticus activity, which could not be suppressed with intravenous diazepam. After treatment with phenytoin for 2 days, an EEG showed periodic high-amplitude sharp-and-slow-wave complexes, which were also not suppressed with intravenous diazepam. Since the patient had measles at 5 months of age, subacute sclerosing Panencephalitis was considered, and the diagnosis was confirmed by the presence of measles antibodies in cerebrospinal fluid.
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combined treatment with subcutaneous interferon α oral isoprinosine and lamivudine for subacute sclerosing Panencephalitis
Journal of Child Neurology, 2003Co-Authors: Omer Faruk Aydin, Nesrin şenbil, Necdet Kuyucu, Y Yavuz K GurerAbstract:We compared patients with subacute sclerosing Panencephalitis who received treatment according to our protocol for at least 6 months (19 patients) with the patients who could not receive any treatment (13 patients). The treatment protocol consisted of oral isoprinosine (100 mg/kg/day), subcutaneous interferon alpha-2a (10 mU/m2/three times a week), and oral lamivudine (10 mg/kg/day). There were no statistical differences between the two groups according to Neurological Deficit Index, clinical stage, and average age on admission and also on the final evaluation after treatment. The mortality rates of both groups were similar: 3 (15.7%) for the treatment group and 6 (46%) for controls. The remission rates for the treatment and control groups were 7 of 19 (36.8%) and 0 of 13 (0%), respectively, and the difference was statistically significant (P = .036). The mean survival period of the treatment group was significantly longer than that of the control group (P = .01). In conclusion, this combination treatment protocol resulted in higher remission rates and longer survival periods when compared with controls, as well as a remission rate that was better than the spontaneous remission rate of 5%. For this reason, and as well as because interferon-alpha therapy has an easier route of application and a higher family compliance, we have considered this an alternative protocol for patients with subacute sclerosing Panencephalitis.
