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L Tokay

Bio: L Tokay is an academic researcher from Harran University. The author has contributed to research in topics: Magnetic resonance imaging & Subacute sclerosing panencephalitis. The author has an hindex of 1, co-authored 1 publications receiving 21 citations.

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Journal ArticleDOI
Hasan Cece1, L Tokay1, Sema Yildiz1, Omer Karakas, Ekrem Karakas, Akin Iscan1 
TL;DR: A normal initial cranial MRI does not exclude subacute sclerosing panencephalitis, which should be kept in mind in childhood demyelinating diseases even when the presentation is unusual, according to a retrospective review of 76 cases.
Abstract: Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, inflammatory neurodegenerative disease. This study investigated the relationships of clinical stage with epidemiological and magnetic resonance imaging (MRI) findings in SSPE by retrospective review of 76 cases (57 male) diagnosed by typical periodic electroencephalographic features, clinical symptoms and elevated measles antibody titre in cerebrospinal fluid. Clinical stage at diagnosis was I or II in 48 patients, III in 25 and IV in three. Prominent findings at presentation were atonic/myoclonic seizures (57.9%) and mental deterioration with behaviour alteration (30.3%). Frequent MRI findings (13 – 32 patients) were subcortical, periventricular and cortical involvement and brain atrophy; the corpus callosum, basal ganglia, cerebellum and brainstem were less frequently involved. Five patients had pseudotumour cerebri. Cranial MRI at initial diagnosis was normal in 21 patients (19 stage I/II, two stage III/IV). Abnormal MRI findings were s...

25 citations


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Journal ArticleDOI
TL;DR: Subacute sclerosing panencephalitis (SSPE) is a slowly progressive brain disorder caused by mutant measles virus that affects younger age groups and a universal vaccination against measles is the only proven way to tackle this menace currently.
Abstract: Subacute sclerosing panencephalitis (SSPE) is a slowly progressive brain disorder caused by mutant measles virus. SSPE affects younger age groups. SSPE incidence is proportional to that of measles. High-income countries have seen substantial decline in SSPE incidence following universal vaccination against measles. SSPE virus differs from wild measles virus. Measles virus genome recovered from the autopsied brain tissues demonstrates clustered mutations in virus genome particularly in the M gene. These mutations destroy the structure and functioning of the encoded proteins. Complete infectious virus particle has rarely been recovered from the brain. Human neurons lack required receptor for entry of measles virus inside the neurons. Recent in vitro studies suggest that mutations in F protein confer hyperfusogenic properties to measles virus facilitating transneuronal viral spread. The inflammatory response in the brain leads to extensive tissue damage. Clinically, SSPE is characterized by florid panencephalitis. Clinically, SSPE is characterized by cognitive decline, periodic myoclonus, gait abnormalities, vision loss, and ultimately to a vegetative state. Chorioretinitis is a common ocular abnormality. Electroencephalography (EEG) shows characteristic periodic discharges. Neuroimaging demonstrates periventricular white matter signal abnormalities. In advanced stages, there is marked cerebral atrophy. Definitive diagnosis requires demonstration of elevated measles antibody titers in cerebrospinal fluid (CSF). Many drugs have been used to stabilize the course of the disease but without evidence from randomized clinical trials. Six percent of patients may experience prolonged spontaneous remission. Fusion inhibitor peptide may, in the future, be exploited to treat SSPE. A universal vaccination against measles is the only proven way to tackle this menace currently.

47 citations

Journal ArticleDOI
TL;DR: Magnetic resonance imaging (MRI) findings may be nonspecific or specific and plays a major role in the diagnosis of encephalitis and predicting the possible cause.
Abstract: Viral encephalitis by definition is the result of human virus affecting the brain and sparing the meninges. The other nervous system manifestations are meningitis, meningoencephalitis, encephalomyelitis, and encephalomyeloradiculitis. Encephalitis can involve any age group from children to old people. The severity of the disease depends on the viral agent and the host immune system. The patient can present with fever, headache, seizure, neurological deficit, or altered sensorium. Laboratory investigations, imaging, and cerebrospinal fluid analysis are crucial in the diagnosis of encephalitis. Magnetic resonance imaging (MRI) findings may be nonspecific or specific and plays a major role in the diagnosis of encephalitis and predicting the possible cause. This pictorial essay reviews the MRI findings of common types of viral encephalitis.

36 citations

Journal ArticleDOI
TL;DR: This report documents current data relating to the clinical phenotype, epidemiology, and understanding of SSPE, inclusive of preventive interventions, and indicates children residing in areas with poor vaccination coverage and high prevalence of human immunodeficiency virus are at increased risk of developing S SPE.
Abstract: Subacute sclerosing panencephalitis (SSPE) is a preventable condition reported in 6.5 to 11 per 100 000 cases of measles, and highest in children who contracted measles infection when they were less than 5 years of age. Children residing in areas with poor vaccination coverage and high prevalence of human immunodeficiency virus are at increased risk of developing SSPE. SSPE is life-threatening in most affected children. This report documents current data relating to the clinical phenotype, epidemiology, and understanding of SSPE, inclusive of preventive interventions. While improvements in disease progression with immunomodulation may occur, overall there is no cure. Most therapies focus on supportive needs. Seizures and abnormal movements may respond to carbamazepine. Many countries advocate policies to enhance vaccination coverage. Effective preventive health care programmes, assurance of parental perceptions, and crisis support for unprecedented events obstructing effective primary health care are needed. Until measles is eradicated worldwide, children in all regions remain at risk. WHAT THIS PAPER ADDS: Measles contracted under 5 years of age has highest risk of developing subacute sclerosing panencephalitis (SSPE). Children with, or exposed to, human immunodeficiency virus infection, who contract measles may be at increased risk of SSPE.

31 citations

Journal ArticleDOI
TL;DR: A consensus on essential laboratory and clinical parameters that should facilitate collaborative studies are established that are urgently needed to improve outcome of subacute sclerosing panencephalitis.
Abstract: 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) and of immunoglobulins has been reported repeatedly, optimum application regimen of these drugs has not been established. This is partly due to the absence of common diagnostic and clinical standards focusing on neurological and psychosocial aspects. Carbamazepine, levetiracetam, and clobazam are the drugs most frequently used to control myoclonic jerks. We have established a consensus on essential laboratory and clinical parameters that should facilitate collaborative studies. Those are urgently needed to improve outcome.

11 citations

Journal Article
TL;DR: Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis and can be prevented by timely immunization against measles.
Abstract: 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 positive in approximately 86% of the 40 (93%) children. EEG showed burst suppression pattern in 36 (83.7%) cases. Forty-two patients (97.6%) were discharged home in a vegetative state. Conclusion SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions.

9 citations