Subacute sclerosing panencephalitis – current perspectives

Abstract

Subacute sclerosing panencephalitis is a progressive neurodegenerative disease. It usually occurs 7–10 years after measles infection. The clinical course is characterized by progressive cognitive decline and behavior changes followed by focal or generalized seizures as well as myoclonus, ataxia, visual disturbance, and later vegetative state, eventually leading to death. It is diagnosed on the basis of Dyken’s criteria. There is no known cure for subacute sclerosing panencephalitis to date, but it is preventable by ensuring that an effective vaccine program for measles is made compulsory for all children younger than 5 years in endemic countries.

Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report

“…Spontaneous remission has been reported in stage 4 of the Risk and Haddad scale in between 53% to 10% of cases [10]. Spontaneous remission may occur during any stage of the disease and last for a variable period before an eventual relapse occurs [11].…”

“…Antiviral drugs and immunomodulators are commonly used in the treatment of SSPE [11]. Antiviral drugs interfere with the viral replication by preventing or inhibiting one of the key components necessary for viral assembly [11].…”

“…Antiviral drugs and immunomodulators are commonly used in the treatment of SSPE [11]. Antiviral drugs interfere with the viral replication by preventing or inhibiting one of the key components necessary for viral assembly [11]. The altered characteristics of the mutant MV in SSPE may by itself hamper the effectiveness of these antiviral drugs [11].…”

“…Antiviral drugs interfere with the viral replication by preventing or inhibiting one of the key components necessary for viral assembly [11]. The altered characteristics of the mutant MV in SSPE may by itself hamper the effectiveness of these antiviral drugs [11]. The combined use of both isoprinosine and interferon alpha was found to have some initial effectiveness but not in the long term [12].…”

“…Neuronal fibrillary tangles (NFTs), which are associated with altered levels of tau and related proteins in CSF, have been observed in certain SSPE cases [11]. NFTs act by inducing glucose hypometabolism and reducing mitochondrial function due to increased superoxide production with oxidative damage and thus alter brain metabolic activity [11].…”