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

Adult-onset subacute sclerosing panencephalitis

2021 ◽  
pp. practneurol-2020-002880
Author(s):  
Sruthi S Nair ◽  
K V Vysakha ◽  
Ramshekhar N Menon ◽  
Soumya Sundaram
Keyword(s):  
Measles Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Viral Disease ◽  
Motor Dysfunction ◽  
Adult Onset ◽  
Progressive Dementia ◽  
Long Latency ◽  
Indian Origin ◽  
History Of ◽  
The Central Nervous System

Subacute sclerosing panencephalitis (SSPE) is a lethal slow viral disease of the central nervous system caused by a defective measles virus. The onset is mostly in childhood, manifesting clinically as decline in academic performance, behavioural changes, motor dysfunction and myoclonus. Adult-onset SSPE is rare and can present as rapidly progressive dementia. We present a young man of Indian origin with adult-onset SSPE with rapidly progressive dementia but no localising neurological signs. The diagnostic clues were parieto-occipital white matter changes on MR brain scan and history of childhood fever with rash. High titres of antimeasles antibody in cerebrospinal fluid confirmed the diagnosis. The long latency from primary measles virus infection to symptom onset can be misleading in adults. SSPE should be considered in adults with dementia, especially in tropical countries where vaccination coverage is suboptimal.

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.

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.

Recurrent Drop Attacks as Presenting Manifestation of Subacute Sclerosing Panencephalitis: An Atypical Presentation

2017 ◽  
Vol 16 (01) ◽  
pp. 021-024
Author(s):  
Aniruddha More ◽  
Rajesh Verma
Keyword(s):  
Cognitive Abilities ◽  
Measles Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Atypical Presentation ◽  
Progressive Decline ◽  
Drop Attacks ◽  
Atypical Manifestations ◽  
Dreaded Disease ◽  
High Index Of Suspicion ◽  
The Brain

AbstractSubacute sclerosing panencephalitis (SSPE) is a potentially fatal progressive complication of measles virus infection of the brain occurring in children and adolescents. It typically presents with a progressive decline in cognitive abilities along with periodic myoclonic jerks. We report a case of SSPE with atypical presentation. Our patient presented with intermittent drop attacks without any obvious myoclonic jerks or cognitive decline making his diagnosis difficult. Atypical manifestations of SSPE occur in 10% of patients, and a high index of suspicion is necessary for early diagnosis and management of this dreaded disease.

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.

scholarly journals A mouse model of persistent brain infection with recombinant Measles virus

2006 ◽  
Vol 87 (7) ◽  
pp. 2011-2019 ◽  
Author(s):  
S. Schubert ◽  
K. Möller-Ehrlich ◽  
K. Singethan ◽  
S. Wiese ◽  
W. P. Duprex ◽  
...  
Keyword(s):  
Immune System ◽  
Measles Virus ◽  
Fluorescent Protein ◽  
Subacute Sclerosing Panencephalitis ◽  
Cns Infection ◽  
Brain Infection ◽  
The Central Nervous System ◽  
Immunocompetent Mice ◽  
Green Fluorescent ◽  
Histological Staining

Measles virus (MV) nucleocapsids are present abundantly in brain cells of patients with subacute sclerosing panencephalitis (SSPE). This invariably lethal brain disease develops years after acute measles as result of a persistent MV infection. Various rodent models for MV infection of the central nervous system (CNS) have been described in the past, in which the detection of viral antigens is based on histological staining procedures of paraffin embedded brains. Here, the usage of a recombinant MV (MV-EGFP-CAMH) expressing the haemagglutinin (H) of the rodent-adapted MV-strain CAM/RB and the enhanced green fluorescent protein (EGFP) is described. In newborn rodents the virus infects neurons and causes an acute lethal encephalitis. From 2 weeks on, when the immune system of the genetically unmodified animal is maturating, intracerebral (i.c.) infection is overcome subclinically, however, a focal persistent infection in groups of neurons remains. The complete brain can be analysed in 50 or 100 μm slices, and infected autofluorescent cells are readily detected. Seven and 28 days post-infection (p.i.) 86 and 81 % of mice are infected, respectively, and virus persists for more than 50 days p.i. Intraperitoneal immunization with MV 1 week before infection, but not after infection, protects and prevents persistence. The high percentage of persistence demonstrates that this is a reliable and useful model of a persistent CNS infection in fully immunocompetent mice, which allows the investigation of determinants of the immune system.

scholarly journals Interesting MRI finding in SSPE – a case report

2019 ◽  
Author(s):  
Anantha Guruswamy ◽  
Krishna Prasad Kurpad
Keyword(s):  
Measles Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Mri Findings ◽  
Chronic Encephalitis ◽  
Signal Abnormality ◽  
Behavioral Abnormality ◽  
Diffuse Atrophy ◽  
Extrapyramidal Features ◽  
Occipital Lobes

Introduction: 10% of the subacute sclerosing panencephalitis (SSPE) presents with atypical features. Aim: SSPE is rare chronic encephalitis caused by persistent infection with defective measles virus. Characteristic MRI findings include signal changes (T2W and FLAIR hyperintensities) in bilateral occipital and parietal regions involving both gray and white matter. Early involvement of cerebellum and brainstem is not common. Case study: A 17-year-old male presented with complaints of recurrent seizures, slow walking, and behavioral abnormality. Neurological examination revealed cogwheel rigidity in all four limbs. MRI of the brain revealed asymmetrical cortical and subcortical altered signal intensities (T2W and FLAIR hyperintensity and T1W hypointensity) involving temporal and occipital lobes bilaterally (left more than right) with diffuse atrophy of cerebrum and cerebellum. Results and discussion: Early onset extrapyramidal features, seizures without myoclonus with MRI finding of posterior predominant asymmetrical cortical and subcortical signal abnormality is uncommon in SSPE. Conclusions: A high index of clinical suspicion for SSPE has to be maintained in patients haling from endemic areas, unvaccinated individuals presenting with seizures, behavioral abnormality, and extrapyramidal features.

Acute Measles Encephalitis in Children With Immunosuppression

PEDIATRICS ◽  
1977 ◽  
Vol 59 (2) ◽  
pp. 232-239
Author(s):  
Jean Aicardi ◽  
Francoise Goutieres ◽  
Maria-Leonor Arsenio-Nunes ◽  
Pierre Lebon
Keyword(s):  
Measles Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Immunosuppressive Drugs ◽  
Prominent Feature ◽  
Epilepsia Partialis Continua ◽  
Fatal Course ◽  
Immunosuppressed Patients ◽  
History Of ◽  
The Central Nervous System ◽  
The Brain

Four cases of encephalitis occurring in children treated for lymphatic malignancies by immunosuppressive drugs are reported. Measles virus was isolated from the brain in one case and identified immunologically in another. Nucleocapsids identical to those seen in subacute sclerosing panencephalitis were demonstrated in three cases. Severe immunosuppression was evidenced in two patients by failure of rosette formation and low phytohemagglutinin tests. Pathologically, the inflammatory reaction was absent in one brain and moderate in two. Clinically, epilepsia partialis continua was a prominent feature in three patients. A history of measles or of contact was elicited in three cases, five weeks to three months before onset. All cases ran an acute fatal course. Measles virus can behave as an opportunistic invader of the central nervous system in children and the diagnosis of measles encephalitis should be considered in immunosuppressed patients.

scholarly journals Viruses and Multiple Sclerosis

2011 ◽  
Vol 17 (6) ◽  
pp. 659-676 ◽  
Author(s):  
Gregory P. Owens ◽  
Don Gilden ◽  
Mark P. Burgoon ◽  
Xiaoli Yu ◽  
Jeffrey L. Bennett
Keyword(s):  
Multiple Sclerosis ◽  
Measles Virus ◽  
Plasma Cells ◽  
Demyelinating Disease ◽  
Subacute Sclerosing Panencephalitis ◽  
Water Channel ◽  
Infectious Agent ◽  
Unknown Etiology ◽  
Antigen Specificity ◽  
Chronic Encephalitis

Multiple sclerosis (MS) is a chronic demyelinating disorder of unknown etiology, possibly caused by a virus or virus-triggered immunopathology. The virus might reactivate after years of latency and lyse oligodendrocytes, as in progressive multifocal leukoencephalopathy, or initiate immunopathological demyelination, as in animals infected with Theiler’s murine encephalomyelitis virus or coronaviruses. The argument for a viral cause of MS is supported by epidemiological analyses and studies of MS in identical twins, indicating that disease is acquired. However, the most important evidence is the presence of bands of oligoclonal IgG (OCBs) in MS brain and CSF that persist throughout the lifetime of the patient. OCBs are found almost exclusively in infectious CNS disorders, and antigenic targets of OCBs represent the agent that causes disease. Here, the authors review past attempts to identify an infectious agent in MS brain cells and discuss the promise of using recombinant antibodies generated from clonally expanded plasma cells in brain and CSF to identify disease-relevant antigens. They show how this strategy has been used successfully to analyze antigen specificity in subacute sclerosing panencephalitis, a chronic encephalitis caused by measles virus, and in neuromyelitis optica, a chronic autoimmune demyelinating disease produced by antibodies directed against the aquaporin-4 water channel.

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