with intractable epilepsy for which functional hemispherectomy remains the only effective cure. Indications Rasmussen encephalitis with concurrent steroid therapy The indication has been agreed by Specialist unless contra-indicated Working consensus after consulting the literature. Rasmussen syndrome is a chronic, Rasmussen encephalitis is a chronic, progressive, focal Description The description and diagnostic criteria section progressive, focal encephalitis encephalitis that is commonly accompanied by focal and has been updated to reflect the findings of that is commonly accompanied seizures, hemiparesis and cognitive decline. It is generally Diagnostic more recently published evidence on disease by focal seizures, hemiparesis and considered to be a disease of childhood, with most cases Criteria presentation, progression and management. cognitive decline. It is generally occurring in children younger than 10 years, although adult considered to be a disease of onset cases do occur. Conventional anticonvulsant therapy childhood, with most cases is usually ineffective and hemispherectomy may be helpful occurring in children younger in the correct setting. than 10 years, although adult onset cases do occur. Generally there is an active progressive phase of the Conventional anticonvulsant disease, often lasting some years, followed by quiescence therapy is usually ineffective and and no further progression of disease. (Varadkar et al, hemispherectomy may be helpful 2014). Although historical data has generally reported in the correct setting. seizure freedom only after functional hemispherectomy, some centres practise more aggressive immune therapies Immunomodulatory therapy may and report improved outcomes. Functional be useful and, of the different hemispherectomy provides the best opportunity of seizure therapies, intravenous freedom, however there will inevitably be post-surgical immunoglobulin (IVIg) may be motor deficits, and often cognitive impairments most useful. Other therapies to Therefore it can be argued that consider include aggressive early immune therapy may reduce the surgical methylprednisolone and sequelae of motor and cognitive deficits (The decision rituximab. regarding when and if to perform hemispherectomy is challenging and discussed in Figure 4 of Varadkar et al Ongoing supply of IVIg would be (2014). based on evidence of stabilisation
with intractable epilepsy for which functional hemispherectomy remains the only effective cure. Indications Rasmussen encephalitis with concurrent steroid therapy unless contra-indicated The indication has been agreed by Specialist Working consensus after consulting the literature. Description and Diagnostic Criteria Rasmussen syndrome is a chronic, progressive, focal encephalitis that is commonly accompanied by focal seizures, hemiparesis and cognitive decline. It is generally considered to be a disease of childhood, with most cases occurring in children younger than 10 years, although adult onset cases do occur. Conventional anticonvulsant therapy is usually ineffective and hemispherectomy may be helpful in the correct setting. Immunomodulatory therapy may be useful and, of the different therapies, intravenous immunoglobulin (IVIg) may be most useful. Other therapies to consider include methylprednisolone and rituximab. Ongoing supply of IVIg would be based on evidence of stabilisation Rasmussen encephalitis is a chronic, progressive, focal encephalitis that is commonly accompanied by focal seizures, hemiparesis and cognitive decline. It is generally considered to be a disease of childhood, with most cases occurring in children younger than 10 years, although adult onset cases do occur. Conventional anticonvulsant therapy is usually ineffective and hemispherectomy may be helpful in the correct setting. Generally there is an active progressive phase of the disease, often lasting some years, followed by quiescence and no further progression of disease. (Varadkar et al, 2014). Although historical data has generally reported seizure freedom only after functional hemispherectomy, some centres practise more aggressive immune therapies and report improved outcomes. Functional hemispherectomy provides the best opportunity of seizure freedom, however there will inevitably be post-surgical motor deficits, and often cognitive impairments Therefore it can be argued that aggressive early immune therapy may reduce the surgical sequelae of motor and cognitive deficits (The decision regarding when and if to perform hemispherectomy is challenging and discussed in Figure 4 of Varadkar et al (2014). The description and diagnostic criteria section has been updated to reflect the findings of more recently published evidence on disease presentation, progression and management.
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