[P3-47] Effects of steroid pulse therapy in refractory epilepsy with anti-glutamate receptor antibodies
[Background] Since there are few reports of steroid pulse therapy (SPT) for refractory epilepsy, we have chosen here to evaluate the effects of SPT on epilepsy with anti-glutamate receptor (GluR) antibodies.
[Methods] We retrospectively analyzed eight refractory epilepsy patients with anti-GluR antibodies in their cerebrospinal fluid who received SPT between 2010 and 2014.
[Results] Eight children (4 boys and 4 girls) aged 4 to 12 years were studied. Two of them had Symptomatic epilepsy, three frontal lobe epilepsy, and the others partial epilepsy, Panayiotopoulos syndrome, or Lennox-Gastaut syndrome. All patients were given SPT after showing increased a seizure frequency or non-convulsive status epilepticus to which anti-epileptic drugs had been ineffective. Four of the patients had autism spectrum disorder, three mental retardation. EEG showed a high-amplitude slow-wave in 4 cases, spike-and-slow-wave complex in 5, sharp wave and sharp-and-slow-wave complex in 3 and small spikes in 1. All cases revealed localizations. Four showed paroxysmal discharge with burst and periodicity after EEG status in sleep. EEG findings showed clinical improvement and reduction of paroxysmal discharge after SPT. Two patients showed an increase in paroxysmal discharges two months after SPT; in five the seizures recurred after one year, and in one, symptoms of paroxysmal discharge disappeared.
[Conclusions] Our findings indicate that SPT might be effective for refractory epilepsy patients whose immunological mechanisms have been damaged. Also, they show that paroxysmal discharge with burst and periodicity after EEG status in sleep, developmental problems, and anti-GluR antibodies benefit from SPT.
[Methods] We retrospectively analyzed eight refractory epilepsy patients with anti-GluR antibodies in their cerebrospinal fluid who received SPT between 2010 and 2014.
[Results] Eight children (4 boys and 4 girls) aged 4 to 12 years were studied. Two of them had Symptomatic epilepsy, three frontal lobe epilepsy, and the others partial epilepsy, Panayiotopoulos syndrome, or Lennox-Gastaut syndrome. All patients were given SPT after showing increased a seizure frequency or non-convulsive status epilepticus to which anti-epileptic drugs had been ineffective. Four of the patients had autism spectrum disorder, three mental retardation. EEG showed a high-amplitude slow-wave in 4 cases, spike-and-slow-wave complex in 5, sharp wave and sharp-and-slow-wave complex in 3 and small spikes in 1. All cases revealed localizations. Four showed paroxysmal discharge with burst and periodicity after EEG status in sleep. EEG findings showed clinical improvement and reduction of paroxysmal discharge after SPT. Two patients showed an increase in paroxysmal discharges two months after SPT; in five the seizures recurred after one year, and in one, symptoms of paroxysmal discharge disappeared.
[Conclusions] Our findings indicate that SPT might be effective for refractory epilepsy patients whose immunological mechanisms have been damaged. Also, they show that paroxysmal discharge with burst and periodicity after EEG status in sleep, developmental problems, and anti-GluR antibodies benefit from SPT.