Neuronal loss is an early component of subacute sclerosing panencephalitis

Neurology ◽  
2014 ◽  
Vol 83 (10) ◽  
pp. 938-944 ◽  
Author(s):  
D. Yuksel ◽  
B. Diren ◽  
H. Ulubay ◽  
 akir Altunba ak ◽  
B. Anlar
2004 ◽  
Vol 19 (3) ◽  
pp. 233-233 ◽  

The fulminating form of subacute sclerosing panencephalitis is an extremely rare condition. Imaging findings are usually not correlated with clinical staging. We describe a 4-year-old girl with severe neuronal loss, demyelination, and gliosis in subcortical white matter by magnetic resonance spectroscopic examination even though she was diagnosed as clinical stage II. In 2 months’ time, her clinical status worsened significantly. Follow-up magnetic resonance spectroscopy revealed findings that were consistent with clinical status. It is our opinion that magnetic resonance spectroscopy could demonstrate a rapidly progressive fulminating course of subacute sclerosing panencephalitis even in the early clinical stages.


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.


Author(s):  
W.L. Steffens ◽  
M.B. Ard ◽  
C.E. Greene ◽  
A. Jaggy

Canine distemper is a multisystemic contagious viral disease having a worldwide distribution, a high mortality rate, and significant central neurologic system (CNS) complications. In its systemic manifestations, it is often presumptively diagnosed on the basis of clinical signs and history. Few definitive antemortem diagnostic tests exist, and most are limited to the detection of viral antigen by immunofluorescence techniques on tissues or cytologic specimens or high immunoglobulin levels in CSF (cerebrospinal fluid). Diagnosis of CNS distemper is often unreliable due to the relatively low cell count in CSF (<50 cells/μl) and the binding of blocking immunoglobulins in CSF to cell surfaces. A more reliable and definitive test might be possible utilizing direct morphologic detection of the etiologic agent. Distemper is the canine equivalent of human measles, in that both involve a closely related member of the Paramyxoviridae, both produce mucosal inflammation, and may produce CNS complications. In humans, diagnosis of measles-induced subacute sclerosing panencephalitis is through negative stain identification of whole or incomplete viral particles in patient CSF.


2019 ◽  
Author(s):  
Yong-Gang Fan ◽  
Tian Guo ◽  
Xiao-Ran Han ◽  
Jun-Lin Liu ◽  
Yu-Ting Cai ◽  
...  

2013 ◽  
Vol 10 (4) ◽  
pp. 390-405 ◽  
Author(s):  
Mar Cuadrado-Tejedor ◽  
Jesus Felipe Cabodevilla ◽  
Marta Zamarbide ◽  
Teresa Gomez-Isla ◽  
Rafael Franco ◽  
...  

2018 ◽  
Vol 15 (2) ◽  
pp. 230-236 ◽  
Author(s):  
Joana Ferreira da Costa ◽  
Xerardo Garcia-Mera ◽  
David Silva Poceiro ◽  
Olga Caamano

Backiground: Alzheimer's disease is a fatal, complex, neurodegenerative disease over 46 million people live with dementia in the world characterized by the presence of plaques containing β-amyloid and neuronal loss. The GPE acts as a survival factor against β-amyloid insult in brain and suggests a possible new therapeutic strategy for the treatment of Central Nervous System injuries and neurodegenerative disorders. The structural simplicity of GPE makes it a suitable lead molecule for the development of new drugs that to cross the blood-brain barrier. Objective: With these aims in mind, we embarked on a synthetic program focused on the modification of the Lproline residue of GPE in order to investigate its importance on the neuroprotective activities. Method: The general synthetic strategy involved the preparation of several modified proline residues, which were subsequently coupled to N-Boc-glycine-OH and glutamic dimethyl ester hydrochloride. Results: the mixture of compounds 11 was obtained in good yields (72%) under these conditions, and this was readily separated by column chromatography and the components were identified by 1H and 13C NMR spectral, as well as by its EI HRMS. Conclusion: Compound (±)-8 was coupled with L-glutamic dimethyl ester hydrochloride gave a mixture of dipeptides 9a and 9b in a satisfactory yield. The use of T3P as coupling agent of the mixture 10a and 10b with Boc-glycine provided a new analogue of GPE, tripeptide 11, obtained with an overall yield of 65% from (±)-1.


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