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Subacute sclerosing panencephalitis: evaluation with CT and MR

J Brismar, +3 more
- 01 Apr 1996 - 
- Vol. 17, Iss: 4, pp 761-772
TLDR
The progress of MR abnormalities seen in patients with SSPE seems to follow a constant pattern, but the severity of MR changes does not always correlate well with the clinical findings, so caution must be used when evaluating the effects of therapy.
Abstract
PURPOSE: To evaluate the progression of CT and MR changes of the brain in subacute sclerosing panencephalitis (SSPE) as a basis for assessing the effects of different types of therapy. METHODS:Fifty-two patients with SSPE were examined, 44 with MR imaging and 42 with CT of the brain on one or more occasions. A total of 92 MR and 67 CT studies were performed. RESULTS: Correlation between the clinical status and the MR findings on admission was poor. Of 20 patients with clinically advanced disease, only 8 had marked MR abnormalities; 6 had normal or almost normal findings on MR examinations. Two of 4 patients with clinically mild disease had advanced MR changes. The progression of the MR findings appeared to follow a constant pattern. The earliest pathologic finding was focal, high-T2-intensity white matter changes; later atrophic changes followed. The atrophy lagged behind the white matter changes and was thus mild when white matter changes were moderate or severe. In the most advanced stage, when the patient was in a neurovegetative state, an almost total loss of white matter had usually taken place. At this stage, the corpus callosum was also thin. Basal ganglia changes, usually involving the putamina, were seen in one third of patients and cortical gray matter changes were seen in one fourth of patients examined with MR imaging. In 2 of 20 patients, MR changes regressed in parallel with clinical improvement following therapy, but in 5 patients clinical improvement was accompanied by progression of MR changes.CONCLUSION:The progress of MR abnormalities seen in patients with SSPE seems to follow a constant pattern, but the severity of MR changes does not always correlate well with the clinical findings. Caution must therefore be used when evaluating the effects of therapy.

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Citations
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Subacute sclerosing panencephalitis

TL;DR: A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment for subacute sclerosing panencephalitis.
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Subacute sclerosing panencephalitis.

TL;DR: There is no effective treatment to completely cure SSPE, but Oral isoprinosine and intrathecal or intraventricular alpha-interferon may prolong survival to some extent.
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Randomized Treatment Study of Inosiplex Versus Combined Inosiplex and Intraventricular Interferon-α in Subacute Sclerosing Panencephalitis (SSPE): International Multicenter Study

TL;DR: The observed rates of satisfactory outcome were higher than the spontaneous remission rates of 5 to 10% reported in the literature, suggesting that treatment was superior to no treatment.
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Subacute sclerosing panencephalitis: clinical and magnetic resonance imaging evaluation of 36 patients.

TL;DR: It is concluded that there seems to be no correlation between the clinical stages and either the duration from the onset of subacute sclerosing panencephalitis or the MRI findings.
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Subacute sclerosing panencephalitis

TL;DR: This report reviews the pathogenesis, clinical and laboratory diagnosis, and future perspectives in treatment and prevention of subacute sclerosing panencephalitis in the USA.
References
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Journal ArticleDOI

Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases

TL;DR: The delineation of four clinical stages of the disease, markedly elevated measles antibody titers in serum and cerebrospinal fluid, ultrastructural observation of intranuclear and intracytoplasmic inclusions in neurons and neuroglia in three patients, and alteration of serum and CSF immunoglobulins indicate that the measles myxovirus may be the etiological agent in these patients.
Journal ArticleDOI

The Variable Natural History of Subacute Sclerosing Panencephalitis: A Study of 118 Cases From the Middle East

TL;DR: A variable natural history was found in a standardized follow-up study of 118 patients with subacute sclerosing panencephalitis (SSPE) and only 20% followed the sequence:behavior change, mental deterioration, periodic attacks, severe debility, and death within a year.
Journal ArticleDOI

Subacute Sclerosing Panencephalitis: Current Status

Paul R. Dyken
- 01 Feb 1985 - 
TL;DR: Subacute sclerosing panencephalitis is a neurodegenerative disease of childhood that is due to a persistent measles infection that is coexistent with the decline in natural measles infection.
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