scholarly journals Rapidly progressive dementia with generalized myoclonus in an adult: Do not forget subacute sclerosing panencephalitis

Neurología ◽  
2021 ◽  
Author(s):  
R. Ghosh ◽  
S. Dubey ◽  
A. Mukherjee ◽  
J. Benito-León
2015 ◽  
Vol 21 (4) ◽  
pp. 468-471 ◽  
Author(s):  
Adalberto Studart Neto ◽  
Paulo Ribeiro Nóbrega ◽  
Maria Irma Seixas Duarte ◽  
Leandro Tavares Lucato ◽  
Luiz Henrique Martins Castro ◽  
...  

2012 ◽  
Vol 6 (4) ◽  
pp. 286-289 ◽  
Author(s):  
Kelson James Almeida ◽  
Sonia Maria Dozzi Brucki ◽  
Maria Irma Seixas Duarte ◽  
Carlos Augusto Gonçalves Pasqualucci ◽  
Sérgio Rosemberg ◽  
...  

ABSTRACT The parieto-occipital region of the brain is the most frequently and severely affected in subacute sclerosing panencephalitis (SSPE). The basal ganglia, cerebellum and corpus callosum are less commonly involved. We describe a patient with SSPE confirmed by neuropathology based on brain magnetic resonance imaging showing extensive basal ganglia involvement and no significant involvement of other cortical structures. Though rarely described in SSPE, clinicians should be aware of this involvement. SSPE should be kept in mind when changes in basal ganglia signal are seen on brain magnetic resonance imaging with or without involvement of other regions of the human brain to avoid erroneous etiological diagnosis of other pathologies causing rapidly progressive dementia.


2015 ◽  
Vol 16 (1) ◽  
pp. 51-52
Author(s):  
Mohammad Rafiqul Islam ◽  
Mamunur Rashid Bhuiyan ◽  
Sudip Ranjan Deb ◽  
Ahmedul Kabir

Subacutesclerosingpanencephalitis(SSPE) is a late complication of measles virus infection. It presents with psychiatric manifestation, progressive dementia, myoclonic jerk and other focal neurological signs. The diagnosis is based upon characteristics clinical manifestation, the presence of characteristic periodic EEG discharge, and demonstration of raised antibody titer against measles in plasma and cerebrospinal fluid. In our case patient presented with behavioral change, myoclonic jerk, characteristic EEG and elevated level of antibody both in serum and CSF.DOI: http://dx.doi.org/10.3329/jom.v16i1.22403 J MEDICINE 2015; 16 : 51-52


2021 ◽  
pp. practneurol-2020-002880
Author(s):  
Sruthi S Nair ◽  
K V Vysakha ◽  
Ramshekhar N Menon ◽  
Soumya Sundaram

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.


2019 ◽  
Vol 9 (0) ◽  
Author(s):  
Antonio Jose Reyes ◽  
Kanterpersad Ramcharan ◽  
Sean Perot ◽  
Stanley Lawrence Giddings ◽  
Fidel Rampersad ◽  
...  

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.


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