scholarly journals Interesting MRI finding in SSPE – a case report

Author(s):  
Anantha Guruswamy ◽  
Krishna Prasad Kurpad

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.

2017 ◽  
Vol 2 (1) ◽  
pp. 40-42
Author(s):  
Mohammad Akter Hossain ◽  
Romal Chowdhury ◽  
Nazmul Islam ◽  
Md Azharul Hoque ◽  
Md Enayet Hussain

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


2011 ◽  
Vol 17 (6) ◽  
pp. 659-676 ◽  
Author(s):  
Gregory P. Owens ◽  
Don Gilden ◽  
Mark P. Burgoon ◽  
Xiaoli Yu ◽  
Jeffrey L. Bennett

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.


Author(s):  
Hannah R. Brown ◽  
Anthony F. Nostro ◽  
Halldor Thormar

Subacute sclerosing panencephalitis (SSPE) is a slowly progressing disease of the CNS in children which is caused by measles virus. Ferrets immunized with measles virus prior to inoculation with the cell associated, syncytiogenic D.R. strain of SSPE virus exhibit characteristics very similar to the human disease. Measles virus nucleocapsids are present, high measles antibody titers are found in the sera and inflammatory lesions are prominent in the brains. Measles virus specific immunoglobulin G (IgG) is present in the brain,and IgG/ albumin ratios indicate that the antibodies are synthesized within the CNS.


Author(s):  
Hannah R. Brown ◽  
Tammy L. Donato ◽  
Halldor Thormar

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.


The Lancet ◽  
1967 ◽  
Vol 289 (7489) ◽  
pp. 542-544 ◽  
Author(s):  
J.H. Connolly ◽  
IngridV. Allen ◽  
L.J. Hurwitz ◽  
J.H.D. Millar

2021 ◽  
Author(s):  
Ryuichi Takemoto ◽  
Tateki Suzuki ◽  
Takao Hashiguchi ◽  
Yusuke Yanagi ◽  
Yuta Shirogane

Measles virus (MeV), an enveloped RNA virus in the family Paramyxoviridae , usually causes acute febrile illness with skin rash, but in rare cases persists in the brain, causing a progressive neurological disorder, subacute sclerosing panencephalitis (SSPE). MeV bears two envelope glycoproteins, the hemagglutinin (H) and fusion (F) proteins. The H protein possesses a head domain that initially mediates receptor binding and a stalk domain that subsequently transmits the fusion-triggering signal to the F protein. We have recently shown that cell adhesion molecule 1 (CADM1, also known as IGSF4A, Necl-2, SynCAM1) and CADM2 (also known as IGSF4D, Necl-3, SynCAM2) are host factors enabling cell-cell membrane fusion mediated by hyperfusogenic F proteins of neuropathogenic MeVs as well as MeV spread between neurons lacking the known receptors. CADM1 and CADM2 interact in cis with the H protein on the same cell membrane, triggering hyperfusogenic F protein-mediated membrane fusion. Multiple isoforms of CADM1 and CADM2 containing various lengths of their stalk regions are generated by alternative splicing. Here we show that only short-stalk isoforms of CADM1 and CADM2 predominantly expressed in the brain induce hyperfusogenic F protein-mediated membrane fusion. While the known receptors interact in trans with the H protein through its head domain, these isoforms can interact in cis even with the H protein lacking the head domain and trigger membrane fusion, presumably through its stalk domain. Thus, our results unveil a new mechanism of viral fusion triggering by host factors. Importance Measles, an acute febrile illness with skin rash, is still an important cause of childhood morbidity and mortality worldwide. Measles virus (MeV), the causative agent of measles, may also cause a progressive neurological disorder, subacute sclerosing panencephalitis (SSPE), several years after acute infection. The disease is fatal, and no effective therapy is available. Recently, we have reported that cell adhesion molecule 1 (CADM1) and CADM2 are host factors enabling MeV cell-to-cell spread in neurons. These molecules interact in cis with the MeV attachment protein on the same cell membrane, triggering the fusion protein and causing membrane fusion. CADM1 and CADM2 are known to exist in multiple splice isoforms. In this study, we report that their short-stalk isoforms can induce membrane fusion by interacting in cis with the viral attachment protein independently of its receptor-binding head domain. This finding may have important implications for cis -acting fusion triggering by host factors.


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 ◽  
...  

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.


BMJ ◽  
1988 ◽  
Vol 296 (6623) ◽  
pp. 684-684 ◽  
Author(s):  
J.-G. Fournier ◽  
J. Gerfaux ◽  
A.-M. Joret ◽  
P. Lebon ◽  
S. Rozenblatt

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