First line treatment for autoimmune encephalitis Indications mediated by antibodies targeting neuronal cell surface antigens Potassium channel antibody-associated Clinical features consistent with antibody Given that the symptoms vary with the encephalopathy Qualifying Criteria mediated autoimmune encephalitis antibody target, a description of the relevant including seizures, cognitive impairment, symptoms for each patient will be captured Potassium channel antibody-associated psychiatric symptoms, dyskinesias, together with a baseline assessment using the neurologic syndromes include limbic autonomic instability, encephalopathy, Modified Rankin Score: encephalitis/subacute amnesic catatonia, central hypoventilation 0 - No symptoms at all encephalopathy, Morvan syndrome, AND 1 - No significant disability despite symptoms; peripheral nerve hyperexcitability and able to out all usual duties and activities A baseline assessment of function is autonomic ganglionopathy. 2 - Slight disability; unable to carry out all measured by the Modified Rankin Score previous activities, but able to look after own Potassium channel antibody-associated AND affairs without assistance encephalopathy is considered to be an 3 - Moderate disability; requiring some help, autoimmune, nonparaneoplastic, Neuronal antibodies to cell surface but able to walk without assistance potentially treatable syndrome, but may antigens have been demonstrated, unless 4 - Moderately severe disability; unable to tests results are unavailable. walk without assistance and unable to attend respond to a variety of to own bodily immunomodulatory agents, including Note anti-GAD or thyroid antibody associated needs without assistance IVIg. syndromes and the classical intracellular 5 - Severe disability; bedridden, incontinent antineuronal antibodies are not considered trial and requiring constant nursing care and this condition. attention Limbic encephalitis — 6 Dead nonparaneoplastic IVIg is approved for one induction cycle (2g/kg over 5 days) in conjunction with systemic steroids
Indications First line treatment for autoimmune encephalitis mediated by antibodies targeting neuronal cell surface antigens Qualifying Criteria Potassium channel antibody-associated encephalopathy Potassium channel antibody-associated neurologic syndromes include limbic encephalitis/subacute amnesic encephalopathy, Morvan syndrome, peripheral nerve hyperexcitability and autonomic ganglionopathy. Potassium channel antibody-associated encephalopathy is considered to be an autoimmune, nonparaneoplastic, potentially treatable syndrome, but may respond to a variety of immunomodulatory agents, including IVIg. Limbic encephalitis — nonparaneoplastic Clinical features consistent with antibody mediated autoimmune encephalitis including seizures, cognitive impairment, psychiatric symptoms, dyskinesias, autonomic instability, encephalopathy, catatonia, central hypoventilation AND A baseline assessment of function is measured by the Modified Rankin Score AND Neuronal antibodies to cell surface antigens have been demonstrated, unless tests results are unavailable. Note anti-GAD or thyroid antibody associated syndromes and the classical intracellular antineuronal antibodies are not considered this condition. IVIg is approved for one induction cycle (2g/kg over 5 days) in conjunction with systemic steroids Given that the symptoms vary with the antibody target, a description of the relevant symptoms for each patient will be captured together with a baseline assessment using the Modified Rankin Score: 0 - No symptoms at all 1 - No significant disability despite symptoms; able to out all usual duties and activities 2 - Slight disability; unable to carry out all previous activities, but able to look after own affairs without assistance 3 - Moderate disability; requiring some help, but able to walk without assistance 4 - Moderately severe disability; unable to walk without assistance and unable to attend to own bodily needs without assistance 5 - Severe disability; bedridden, incontinent and requiring constant nursing care and attention 6 Dead trial
autoimmune-encephalitis-mediated-by-antibodies-targeting-cell-surface-antigens_0.DOCX