In 2001, a 44-year-old woman began having focal aware seizures characterized by episodic expressive aphasia. MRI at that time did not reveal abnormalities and her seizures were well controlled with AED polytherapy. Repeat MRI 1 year later revealed a small left frontal lobe T2 hyperintensity without enhancement. Her seizures remained well controlled over the next 10 years on a combination of oxcarbazepine, topiramate, and levetiracetam, and she continued working. Approximately 11 years after first lifetime seizure, she experienced an episode of complete expressive aphasia lasting hours and presented to our institution. She was diagnosed with focal status epilepticus, characterized clinically by expressive aphasia. EEG findings were significant for electroclinical seizures with left frontal onset as well as continuous spike/wave activity in the left frontal region (Figure ). MRI at that time showed swelling of the left cortical frontal lobe and operculum gray matter as well as mild left frontal atrophy (Figure ). Initial serum and CSF studies, including cell counts were normal. Further testing was notable for the following negative studies: paraneoplastic and autoimmune encephalitis antibody (NMDA-R, ANNA-1, ANNA-2, ANNA-3, AGNA-1, PCA-1, PCA-2, PCA-Tr, Amphiphysin, CRMP-5, VGKC, GAD), 14-3-3, serum lyme, NYS viral encephalitis panel, ANA, ACE. The patient was acutely treated with phenobarbital, which abolished the status epilepticus. IVIG was also initiated over this hospitalization for the possibility of an autoimmune etiology without specific consideration of RE. She was discharged from the hospital on four AEDs—oxcarbazepine, topiramate, levetiracetam, and phenobarbital. Subsequent clinical improvement over the next several months, initially thought to be related to phenobarbital, waned, leading to the conclusion that IVIG had been the ameliorative therapy. The patient began monthly IVIG infusions to supplement AED pharmacotherapy and experienced improvement in expressive aphasia and a decline in seizure frequency.\nApproximately 6 months later, the semiology of the patient’s seizure again evolved to include right rhythmic facial twitching in addition to expressive aphasia. Less than 1 year from prior episode of status epilepticus, she was re-admitted to the EMU. There was no evidence of electrographic seizures, but the presence of left frontal spike/wave complexes persisted as did episodes of right facial twitching and mutism. Ictal SPECT imaging revealed a focus of increased uptake in the left anterior frontal lobe, relative to the right frontal lobe, correlating with MRI abnormality. Over the following months, patient’s aphasia became nearly constant and semiology again began to involve the right arm. Repeat MR imaging now very clearly showed focal atrophy in the left frontal lobe, particularly involving the frontal pole, as well as the left temporal lobe (Figure ). This finding coupled with the progressive nature of the patient’s focal epilepsy solidified the diagnosis of RE. Monthly IVIG treatments were continued, topiramate does was augmented, and PRN lorazepam was added to the patient’s AED regimen. Unfortunately, the patient continued to have brief focal seizures several times per week and her mild expressive aphasia was static. In September 2016, approximately 3 years after her presentation to our institution and 15 years from first seizure, she began treatment with Rituximab. While her overall seizure frequency appears to have improved, she continues to have weekly focal seizures, a static mild expressive aphasia and remains on four AEDs.
A 5 year old male child presented with complaints of multiple episodes of clonic seizures of the left upper and lower limb since the last 8 months, each episode was lasting for 5–15 min with a prolonged postictal state. He was tre ated with anticonvulsant drugs. He had no history of blood transfusions, neurosurgery, traumatic head injury, proven allergies, and family history of epilepsy. Antenatal, perinatal and postnatal history was normal. The seizures were progressively increasing in frequency and severity since last month. The episodes also involved a sudden loss of consciousness and bladder incontinence. Because of that, he admitted to our neurology department. On examination, the child was disoriented. His vitals were stable. Central nervous system examination showed left-sided hemiparesis with exaggerated tendon reflexes on the left side. Muscle power Medical Research Council (MRC) was grade 4 on the left upper and lower limb, but a full 5 on the right upper and lower limbs. There were no signs of meningeal irritation, and cranial nerves were intact. Cerebrospinal fluid analysis was normal, (PCR) for Herpes Simplex Virus, EBV, measles and CMV, appeared to be negative.\nThe patient had a progression to epilepsia partialis continua within 9 months after first focal seizure and impairment in cortical functioning.\nThe MRI Flair showed high signal intensity area in right temporal lobe ().\nMRI T1 and T2 showed atrophy in right partial and temporal lobes ().\nEEG showed slow activity with focal right frontal polyphasic spikes paroxysmal discharges ().\nOn arrival he was give diazepam and phenytoin infusion. Later treatment was started on oral levetiracetam, clonazepam, lamotrigin, Carbamzepine and valproate sodium.\nAfter 25 days of treatment, patient continued to develop seizures and her right-sided weakness persisted, progressed and became worsen. During these episodes of fits, child was started on intravenous steroids.\nTo slow hemispheric atrophy, anti-inflammatory treatment with tacrolimus was given.\nFollow up MRI revealed effacement of cortical sulci and also asymmetry between two cerebral hemisphere with atrophy of right cerebral hemisphere.\nThe patient was treated with regular dosing of IVIG. A dose interval of 4 weeks was determined. Despite regular IVIG dosing for approximately 6 months as well as concomitant AED therapy, the patient's epilepsy remained refractory. Functional Hemispherectomy was performed by the complete removal of the cortex and white matter, sparing the basal ganglia. After surgery, the child had mild episodes of seizures. Unfortunately, he died because of meningitis.