Conclusion: SSPE should be considered and necessary tests should be performed in the differential diagnosis of encephalitis patients. Treatment is supportive and primarily based on the signs and symptoms present in each person. SSPE is a unique slow viral disease in which the measles virus has been identified as the pathogen. Found inside â Page 242SSPE is diagnosed by the measurement of elevated blood and CSF titers of antibodies against measles virus (Connolly et al. 1967) and by EEG abnormalities (Cobb 1966). The differential diagnosis includes diseases of childhood which ... Conditions with similar signs and symptoms from Orphanet. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis. Found inside â Page 962It remains to be seen whether this will be useful in the differential diagnosis with MS , in which a reduction in ... white matter perilesional ( differential diagnosis with MS ) Subacute Sclerosing Panencephalitis posterior reversible ... Complications are mainly associated with the intrathecal reservoir used for the INF administration in up to 23% of the participants.46 Most authors recommend the combination of INF-α and isoprinosine as the most effective therapy to start with.37, 49, 52, 53, Isoprinosine, a derivative of inosine and the p-acetamidobenzoic acid salt of N,N-dimethylamido-2-propanol, is thought to disrupt viral replication and is probably an immunomodulator1 with a relatively safe profile. SSPE is a unique slow viral disease in which the measles virus has been identified as the pathogen. Usually two major criteria plus one minor criterion are required; the more atypical the SSPE, the more criteria 5 and/or 6 are needed. Found insidedParentheses around a differential feature indicate an uncommon feature that is very useful in differential diagnosis when it is found. α-ACT, α-antichymotrypsin macrophage marker; CNS, central nervous system; GFAP, glial fibrillary ... Early in the disease, oedema appears to be the predominant finding.6 Evidence of DNA and ribonucleic acid oxidative damage has been found in infected cells during the first few years of the disease, with lipid peroxidation found in areas of early demyelination.34, During the acute inflammatory phase, nucleocapsids are found in oligodendrocytes, and neurons and nuclear bodies with granulofilamentous inclusions are found in astrocytes.5, 36 Cortical and subcortical perivascular infiltration of inflammatory cells, spongiosis, and demyelination are also reported in the acute phase, followed by neuronal loss as the disease evolves.5-7 The inflammatory changes initially tend to be more pronounced in the posterior areas of the brain, with marked involvement of the medial thalamus and deep structures followed by spread to anterior areas,7 and with relative sparing of the cerebellum.34, The age at presentation is usually 8 to 11 years,19, 37, 38 with onset usually occurring 6 years after measles infection.6, 19 Affected individuals present with poor school performance and progressive intellectual deterioration, personality changes, and behaviour abnormalities; this is followed by steady motor decline, myoclonus, focal paralysis, seizures, autonomic failure, and rigidity, finally leading to death with akinetic mutism.5, 16, 19, 38, 39 These changes are characterized in four stages (see Table I). On the background of this history SSPE should be considered as differential diagnosis in patients with changes of personality. It is thought that an inadequate cellular response plays a pivotal pathogenic role in the development of SSPE. Found insideIncidence estimates range from four to nearly 30 SSPE cases per 100 000 measles cases [27]. ... When myoclonus is present, the differential diagnosis includes prion disorders, corticobasal syndrome, dementia with Lewy bodies, hypoxia, ... 2019 Jul;101(1):260-262. doi: 10.4269/ajtmh.19-0215. Subacute sclerosing panencephalitis in the differential diagnosis of encephalitis. A rare but lethal late complication of measles is. Greater measles virus loads, as demonstrated by polymerase chain reaction, have been associated with the acute phase of SSPE;33 the immune response is not directed against myelin or any other CNS antigen but against infected cells.24 B-cell lymphoma-2-induced apoptosis and DNA fragmentation have been suggested as early causes of neuron and oligodendrocyte death, and lipid peroxidation and disturbed glutamate transport have been implicated in subsequent neurodegeneration (Fig. The authors describe five cases of subacute sclerosing panencephalitis (SSPE) identified through the California Encephalitis Project that emphasize the importance of considering SSPE in the differential diagnosis of encephalitis, particularly among pediatric patients. Trials have shown that, at best, 30 to 35% of individuals benefit from therapy, depending on the study design.37, 41 Benefit is defined as either slower progression, temporal stabilization of disease progression, prolonged survival, or, less likely, clinical improvement.4, 37 The benefit afforded, although relatively modest, is much better than the 5% spontaneous remission rate reported in the literature.41, 51 So far, the best results have been achieved with a combination of weekly intrathecal INF-α and daily oral isoprinosine, which was found to be of benefit in 35% of participants; however, this treatment was found to offer no clear advantage compared with isoprinosine monotherapy.37, 41, Despite this, the combination is still recommended because of theoretical synergistic effects.
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