scholarly journals Subacute Sclerosing Panencephalitis in a Child with Recurrent Febrile Seizures

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Ayşe Kartal ◽  
Ayşegül Neşe Çıtak Kurt ◽  
Tuğba Hirfanoğlu ◽  
Kürşad Aydın ◽  
Ayşe Serdaroğlu
Keyword(s):  
Cerebrospinal Fluid ◽  
Measles Virus ◽  
Unusual Case ◽  
Subacute Sclerosing Panencephalitis ◽  
Febrile Seizures ◽  
Final Diagnosis ◽  
High Amplitude ◽  
Measles Antibodies ◽  
Antibody Titres ◽  
The Central Nervous System

Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis.

Immune electron microscopic study of interactions between measles virus nucleocapsids and antibodies from sera of patients with SSPE

1978 ◽  
Vol 36 (2) ◽  
pp. 368-369
Author(s):  
L. Cohen-Forterre ◽  
S. Rousset ◽  
P. Lebon
Keyword(s):  
Genetic Information ◽  
Chronic Infection ◽  
Measles Virus ◽  
Canine Distemper Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Canine Distemper ◽  
Electron Microscopic ◽  
Sspe Virus ◽  
Measles Antibodies ◽  
The Central Nervous System

Subacute sclerosing panencephalitis(SSPE)is a progressive degenerative disease of the central nervous system. This disease is generally associated with a chronic infection with measles virus. If measles virus is clearly involved however additional factors must determine the onset of SSPE. Hall and Ter Meulen reported that if “all the genetic information of measles virus is contained in SSPE virus, the latter apparently contain an additional 10% information”. They thought that “if a recombination occurs with another second virus, then it is with the intact genome of measles virus and a defective genome of the second virus ”. Previously Lebon et al. report ed the presence in sera/from patients with SSPE of antibodies able to precipitate an antigen of canine distemper virus. Antibodies of this type have not been detected inhuman sera with confirmed measles antibodies.

Presence of antibody in virus-infected brain cells of SSPE ferrets

1983 ◽  
Vol 41 ◽  
pp. 804-805
Author(s):  
Hannah R. Brown ◽  
Tammy L. Donato ◽  
Halldor Thormar
Keyword(s):  
Measles Virus ◽  
Plasma Cells ◽  
Subacute Sclerosing Panencephalitis ◽  
Protein A ◽  
Neutralizing Antibody ◽  
Brain Cells ◽  
Antibody Titers ◽  
A Cell ◽  
The Central Nervous System ◽  
The Brain

Measles virus specific immunoglobulin G (IgG) has been found in the brains of patients with subacute sclerosing panencephalitis (SSPE), a slowly progressing disease of the central nervous system (CNS) in children. IgG/albumin ratios indicate that the antibodies are synthesized within the CNS. Using the ferret as an animal model to study the disease, we have been attempting to localize the Ig's in the brains of animals inoculated with a cell associated strain of SSPE. In an earlier report, preliminary results using Protein A conjugated to horseradish peroxidase (PrAPx) (Dynatech Diagnostics Inc., South Windham, ME.) to detect antibodies revealed the presence of immunoglobulin mainly in antibody-producing plasma cells in inflammatory lesions and not in infected brain cells.In the present experiment we studied the brain of an SSPE ferret with neutralizing antibody titers of 1:1024 in serum and 1:512 in CSF at time of sacrifice 7 months after i.c. inoculation with SSPE measles virus-infected cells. The animal was perfused with saline and portions of the brain and spinal cord were immersed in periodate-lysine-paraformaldehyde (P-L-P) fixative. The ferret was not perfused with fixative because parts of the brain were used for virus isolation.

Diagnosis of Subacute Sclerosing Panencephalitis by a Simple Spinal Fluid Gel Precipitation Test for Measles

PEDIATRICS ◽  
1972 ◽  
Vol 49 (1) ◽  
pp. 133-136
Author(s):  
Dale E. Dietzman ◽  
Luiz Horta-Barbosa ◽  
Helen M. Krebs ◽  
David L. Madden ◽  
David A. Fuccillo ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Hemagglutination Inhibition ◽  
Measles Virus ◽  
Complement Fixation ◽  
Subacute Sclerosing Panencephalitis ◽  
Double Diffusion ◽  
Virus Antigen ◽  
Spinal Fluid ◽  
Reliable Procedure ◽  
Precipitation Test

A double-diffusion gel precipitation test is described which provides an easy, rapid, and reliable procedure for assistance in the diagnosis of subacute sclerosing penencephalitis by detecting measles antibody in concentrated cerebrospinal fluid. The test is based on the precipitation of rubeola antibodies with a high titered SSPE measles-virus antigen. The sensitivity of the test is comparable to the sensitivity of rubeola complement-fixation and hemagglutination-inhibition determinations on unconcentrated spiral fluid. The method could be available to hospitals or institutions if the antigen were prepared commercially or by a national center.

scholarly journals Subacute Sclerosing Panencephalitis

2019 ◽  
Vol 37 (4) ◽  
pp. 205-208
Author(s):  
Gobinda Chandra Banik ◽  
Sakib Aman ◽  
Farhana Sultana ◽  
Syed Mohammad Arif
Keyword(s):  
Cerebrospinal Fluid ◽  
Virus Infection ◽  
Antibody Titer ◽  
Measles Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Abnormal Behavior ◽  
Short History ◽  
Altered Consciousness ◽  
History Of ◽  
Metachromatic Leucodystrophy

  Subacute sclerosing panencephalitis (SSPE) is chronic progressive encephalitis of childhood and young adoloscent due to persistent measles virus infection. This case illustrates a 14 year old girl presented with short history of intellectual decline, abnormal behavior, myoclonus and altered consciousness with suggestive neuroimaging mimicking metachromatic leucodystrophy. Subsequently she was diagnosed to be a case of Subacute sclerosing panencephalitis (SSPE) on the basis of Electroencephalography (EEG) and Cerebrospinal fluid(CSF) measles antibody titer. J Bangladesh Coll Phys Surg 2019; 37(4): 205-208

scholarly journals Nonconvulsive Status Epilepticus on Electroencephalography: An Atypical Presentation of Subacute Sclerosing Panencephalitis in Two Children

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Pratibha Singhi ◽  
Arushi Gahlot Saini ◽  
Jitendra Kumar Sahu
Keyword(s):  
Cerebrospinal Fluid ◽  
Status Epilepticus ◽  
Cognitive Decline ◽  
Neurodegenerative Disease ◽  
Slow Wave ◽  
Subacute Sclerosing Panencephalitis ◽  
Nonconvulsive Status Epilepticus ◽  
Atypical Presentation ◽  
Measles Antibodies

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.

scholarly journals A role for dual viral hits in causation of subacute sclerosing panencephalitis

2005 ◽  
Vol 202 (9) ◽  
pp. 1185-1190 ◽  
Author(s):  
Michael B.A. Oldstone ◽  
Samuel Dales ◽  
Antoinette Tishon ◽  
Hanna Lewicki ◽  
Lee Martin
Keyword(s):  
Measles Virus ◽  
B Lymphocyte ◽  
Subacute Sclerosing Panencephalitis ◽  
Small Animal ◽  
Transgenic Mouse Model ◽  
Lymphocytic Choriomeningitis ◽  
Small Animal Model ◽  
M Gene ◽  
The Matrix ◽  
The Central Nervous System

Subacute sclerosing panencephalitis (SSPE) is a progressive fatal neurodegenerative disease associated with persistent infection of the central nervous system (CNS) by measles virus (MV), biased hypermutations of the viral genome affecting primarily the matrix (M) gene with the conversion of U to C and A to G bases, high titers of antibodies to MV, and infiltration of B cells and T cells into the CNS. Neither the precipitating event nor biology underlying the MV infection is understood, nor is their any satisfactory treatment. We report the creation of a transgenic mouse model that mimics the cardinal features of SSPE. This was achieved by initially infecting mice expressing the MV receptor with lymphocytic choriomeningitis virus Cl 13, a virus that transiently suppressed their immune system. Infection by MV 10 days later resulted in persistent MV infection of neurons. Analysis of brains from infected mice showed the biased U to C hypermutations in the MV M gene and T and B lymphocyte infiltration. These sera contained high titers of antibodies to MV. Thus, a small animal model is now available to both molecularly probe the pathogenesis of SSPE and to test a variety of therapies to treat the disease.

scholarly journals Adult Onset Subacute Sclerosing Panencephalitis: A case report

2017 ◽  
Vol 2 (1) ◽  
pp. 40-42
Author(s):  
Mohammad Akter Hossain ◽  
Romal Chowdhury ◽  
Nazmul Islam ◽  
Md Azharul Hoque ◽  
Md Enayet Hussain
Keyword(s):  
Measles Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Accurate Diagnosis ◽  
Adult Onset ◽  
Chronic Encephalitis ◽  
The Central Nervous System ◽  
Atypical Feature ◽  
High Index Of Suspicion ◽  
Electroencephalogram Eeg ◽  
Young Adolescence

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistent measles virus infection of the central nervous system (CNS). In majority of cases, onset occurs between 5-10 years of age. SSPE generally occurs 5-10 years after measles virus infection1. The diagnosis of SSPE is based on characteristic clinical and electroencephalogram (EEG) findings, increase measles antibody titer in cerebrospinal fluid (CSF) and serum. As onset of SSPE in adults is rare and may have atypical feature it requires high index of suspicion for early and accurate diagnosis. Herein, we report a case of SSPE in a male of 26 years with recurrent episodes of myoclonic jerks. Journal of National Institute of Neurosciences Bangladesh, 2016;2(1): 40-42

scholarly journals The involvement of Central Nervous System and sequence variability of Severe Adult Respiratory Syndrome – Coronavirus-2 revealed in autopsy tissue samples: a case report.

2020 ◽  
Author(s):  
Lis Høy Marbjerg ◽  
Christina Jacobsen ◽  
Jannik Fonager ◽  
Claus Bøgelund ◽  
Morten Rasmussen ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Central Nervous System Infection ◽  
Final Diagnosis ◽  
Forensic Autopsy ◽  
Tissue Samples ◽  
Specific Antibodies ◽  
Perivascular Inflammation ◽  
The Central Nervous System

Abstract Background: The case presented here illustrates that interdisciplinary teamwork can be essential for the understanding of the COVID-19 disease presentation and enlightening of the pathophysiology. Case presentation: A 60-years-old overweight woman without any comorbidities was found dead in her apartment after 14 days of home isolation due to suspicion on the Coronavirus disease 2019 (COVID-19). She had reported symptoms of tachycardia, fever, and increasing respiratory difficulty one day before her death. Due to the Danish legal act on sudden deaths a forensic autopsy was performed including a thorough examination and biosampling. The results of the forensic autopsy displayed sever densified, almost airless, firm lungs, and an unspecific reactive minimal focal perivascular inflammation consisting of macrophages of the brain tissue. The final diagnosis, COVID-19 with involvement of the central nervous system was established by use of the RT-RNA analysis on cerebrospinal fluid, as well as by serologic detection of the specific antibodies for SARS-CoV-2 in cerebrospinal fluid and serum. The genetic analysis displayed a 2 % variation between SARS-CoV-2 isolates recovered from the tracheal sample, cerebrospinal fluid, and tissues from both lungs.Conclusion: The combination of all available results revealed that the cause of death was COVID-19 with severe pulmonary disease and neuroinvasion, as well as renal affection resulting in hyponatremia. To our knowledge, it was not shown previously that neuroinvasion could be confirmed by the detection of specific antibodies for SARS-CoV-2 and SARS-CoV-2 specific RNA in cerebrospinal fluid. This case supports hypotheses that SARS-CoV-2 may cause central nervous system infection. The genetic distinction between SARS-CoV-2 isolates was done by whole-genome sequencing, where the isolate recovered from the cerebrospinal fluid was the most different.

scholarly journals Measles Virus Mutants Possessing the Fusion Protein with Enhanced Fusion Activity Spread Effectively in Neuronal Cells, but Not in Other Cells, without Causing Strong Cytopathology

2014 ◽  
Vol 89 (5) ◽  
pp. 2710-2717 ◽  
Author(s):  
Shumpei Watanabe ◽  
Shinji Ohno ◽  
Yuta Shirogane ◽  
Satoshi O. Suzuki ◽  
Ritsuko Koga ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Fusion Protein ◽  
Measles Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Neuronal Cells ◽  
Extracellular Domain ◽  
Fusion Activity ◽  
F Protein ◽  
The Central Nervous System

ABSTRACTSubacute sclerosing panencephalitis (SSPE) is caused by persistent measles virus (MV) infection in the central nervous system (CNS). Since human neurons, its main target cells, do not express known MV receptors (signaling lymphocyte activation molecule [SLAM] and nectin 4), it remains to be understood how MV infects and spreads in them. We have recently reported that fusion-enhancing substitutions in the extracellular domain of the MV fusion (F) protein (T461I and S103I/N462S/N465S), which are found in multiple SSPE virus isolates, promote MV spread in human neuroblastoma cell lines and brains of suckling hamsters. In this study, we show that hyperfusogenic viruses with these substitutions also spread efficiently in human primary neuron cultures without inducing syncytia. These substitutions were found to destabilize the prefusion conformation of the F protein trimer, thereby enhancing fusion activity. However, these hyperfusogenic viruses exhibited stronger cytopathology and produced lower titers at later time points in SLAM- or nectin 4-expressing cells compared to the wild-type MV. Although these viruses spread efficiently in the brains of SLAM knock-in mice, they did not in the spleens. Taken together, the results suggest that enhanced fusion activity is beneficial for MV to spread in neuronal cells where no cytopathology occurs, but detrimental to other types of cells due to strong cytopathology. Acquisition of enhanced fusion activity through substitutions in the extracellular domain of the F protein may be crucial for MV's extensive spread in the CNS and development of SSPE.IMPORTANCESubacute sclerosing panencephalitis (SSPE) is a fatal disease caused by persistent measles virus (MV) infection in the central nervous system (CNS). Its cause is not well understood, and no effective therapy is currently available. Recently, we have reported that enhanced fusion activity of MV through the mutations in its fusion protein is a major determinant of efficient virus spread in human neuronal cells and brains of suckling hamsters. In this study, we show that those mutations render the conformation of the fusion protein less stable, thereby making it hyperfusogenic. Our results also show that enhanced fusion activity is beneficial for MV to spread in the CNS but detrimental to other types of cells in peripheral tissues, which are strongly damaged by the virus. Our findings provide important insight into the mechanism for the development of SSPE after MV infection.

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