Role of Carbamazepine in the Symptomatic Treatment of Subacute Sclerosing Panencephalitis: A Case Report and Review of the Literature

Abstract

We describe the clinical presentation and clinical course of subacute sclerosing panencephalitis in a 13-year-old previously healthy boy who recently immigrated to the United States from Iraq. He presented with macular retinopathy, followed by progressive myoclonus and encephalopathy. After extensive workup, a diagnosis of subacute sclerosing panencephalitis was suspected by the presence of period epileptiform discharges on electroencephalogram and confirmed by elevated measles titers in the cerebrospinal fluid. Combination immunomodulatory therapy with isoprinosine, ribavirin, and intra-Ommaya interferon alpha did not result in clinical improvement. Within days following the administration of carbamazepine, there was remarkable improvement in the myoclonus and he was able to ambulate independently for a period of 4 months at which time he unfortunately progressed to a vegetative state. This case highlights the importance of carbamazepine as a potential first line symptomatic treatment of subacute sclerosing panencephalitis and provides a review of the literature on the subject.

Subacute sclerosing panencephalitis: clinical phenotype, epidemiology, and preventive interventions

“…Benzodiazepines such as intravenous diazepam and midazolam are also used. 47 Children coinfected with HIV and SSPE are challenging to manage, as the cross-drug interactions between carbamazepine and antiretroviral agents such as protease inhibitors or non-nucleoside reverse transcriptase inhibitors may result in virological failure, thus requiring careful monitoring of HIV viral titres and carbamazepine levels. 45 Improvement in myoclonus with carbamazepine is also reported.…”

“…The aetiology for the myoclonus is thought to be due to basal ganglia involvement of the virus, whose pathways are not governed by alterations in sodium channels. 47 Children coinfected with HIV and SSPE are challenging to manage, as the cross-drug interactions between carbamazepine and antiretroviral agents such as protease inhibitors or non-nucleoside reverse transcriptase inhibitors may result in virological failure, thus requiring careful monitoring of HIV viral titres and carbamazepine levels. 48 This situation is largely relevant to sub-Saharan Africa where 83% of children with HIV infection reside and data to support development of recommendations to optimize care in this setting are limited.…”

Subacute sclerosing panencephalitis in pregnancy

“…Cortical metabolism was preserved and neurological prognosis was good at 3 years after diagnosis. 64 One report 65 noted an improvement of myoclonus with carbamazepine use, although this treatment had no eff ect on mortality.…”

A Multinational Survey on Actual Diagnostics and Treatment of Subacute Sclerosing Panencephalitis

“…In addition to antiviral agents, most patients need supportive treatment to suppress myoclonic jerks and/or epileptic seizures. 30,31 Carbamazepine, levetiracetam, and clobazam are the drugs applied by most centers, whereas topiramate, lamotrigine, phenobarbital, and oxcarbazepine are applied less frequently.…”

“…Most frequently, treatment of these jerks is based on carbamazepine, levetiracetam, and clobazam application. 30,31 The question for preventive intervention before the onset of SSPE may also arise in a child presenting with acute primary measles infection. Although several drugs have been discussed for secondary prophylaxis, most contributors remained skeptical about their ability to prevent the development of SSPE at an older age.…”