An 8 year-old girl presented with history of repeated episodes of clonic seizures involving right upper and lower limbs and each episode lasting for 2–5 minutes with prolonged postictal state. The episodes didn't involve a sudden loss of consciousness but she had a bladder incontinence. The first episode started at the age of 7 years with rapid increase in seizure activity followed by decrease in frequency 10–15 episodes in the day with slurring of speech, cognitive delay and abnormal gait. She was born as a second twin and cried immediately after birth with normal APGAR score. No history of neonatal seizures. She had no history of blood transfusions, neurosurgery, traumatic head injury, proven allergies and family history of epilepsy. An initial routine electroencephalogram (EEG) was normal. Magnetic resonance imaging (MRI) was normal. The patient put on Oxcarbazepine and then, Valproate Sodium and Clonazepam were added gradually.\nOne month ago, she suffered from paraesthesia and twitching of right arm.\nShe admitted to our neurology department when the patient experienced an exacerbation of focal status epilepticus and generalized seizures.\nHer seizures could not be controlled with antiepileptic drugs alone.\nNeurologic exam at admission showed constant twitching of the right hand and intermittent stiffening of the right leg and pelvic. She had a right hemiparesis and muscle power Medical Research Council (MRC) grade 3 on the right upper and lower limb, but a full 5 on the left upper and lower limbs.\nShe also had exaggerated tendon reflexes on the right side. She showed severe global developmental delay.\nThere were no signs of meningeal irritation, and cranial nerves were intact. Cerebrospinal fluid analysis was normal, (PCR) for Herpes Simplex Virus, EBV, measles, Rubella and CMV appeared to be negative.\nEEG showed slow activity with focal left frontal biphasic spikes paroxysmal discharges ().\nMRI brain T1 revealed left hemispheric atrophy and in the left insular cortex ().\nMRI T2 axial revealed atrophy in left Caudate and putamen nuclei ().\nA diagnosis of RE was suspected.\nShe was given Immunotherapy with IVIG for one dose followed by IV. methyl prednisolone 30mg/kg for 5 days. Followed by oral prednisolone for 28 days. She slightly improved.\nThe parents however noticed an increase in the frequency of her seizure to three times a day every day. She is currently on outpatient review, and a more aggressive treatment (including surgical intervention) is being considered.
In March 2007, a 47-year-old woman with a history of bilateral optic neuritis at age 35 experienced her first seizure. One month later, she experienced a second seizure and was started on lamotrigine. She presented to our institution as an elective admission to the EMU 6 months after seizure onset. Many right temporal seizures (clinical semiology of paresthesias over left face and arm) were observed and the interictal EEG was notable for right periodic lateralized discharges (PLDs; Figure ). MRI showed abnormal T2 signal in the right frontal cortex, right thalamus as well as a small area of contrast enhancement in the right temporal uncus. MR SPECT showed normal choline:creatinine ratio. Serum studies were notable for the following normal/negative studies: ANA, ACE, anti-TPO, Lyme, paraneoplastic antibody (ANNA-1, ANNA-2, ANNA-3, AGNA-1, PCA-1, PCA-2, PCA-Tr, CRMP-5, VGKC). CSF evaluation was significant for a mild lymphocytic pleocytosis, negative HSV and CMV PCR, and cytology negative for malignant cells. Ultimately, after trials of several AEDs including lamotrigine, levetiracetam, and carbamazepine, oxcarbazepine and phenytoin were utilized for seizure control.\nOver the next 3 years, the patient had several admissions to our institution for breakthrough seizures. Her seizure semiologies over this time frame were isolated left face and arm paresthesias, focal motor seizures involving left face and arm as well as secondarily generalized tonic/clonic seizures. A static mild left hemiparesis, most affecting the left hand, was also noted during this time. Further diagnostic work-up over one of these later admissions was notable for normal CSF cell counts, a CT scan of the chest, abdomen, and pelvis negative for malignancy, and repeat negative paraneoplastic panel evaluation. At this point, the patient was lost to follow-up.\nShe re-presented to our institution 5 years later after she experienced a generalized convulsion. Collateral history indicated that she had become unable to live at home unassisted and often had confusional and agitated episodes. Since last seen at our institution, her AED regimen had been changed to levetiracetam and valproic acid. In addition, a third lumbar puncture was performed and notably NMDA-R antibody testing was negative. The patient was found to be in non-convulsive status epilepticus and treated with benzodiazepines and augmented doses of her AEDs. CT imaging done on this admission revealed marked right hemisphere atrophy. Continuous EEG monitoring showed both right frontal and temporal spikes and left temporal slowing. Clinical exam was significant for perseverative speech, inability to follow complex commands and a moderate left hemiparesis. Given the patient’s marked hemiatrophy, refractory epilepsy, and static neurologic deficits, she was diagnosed with RE and monthly IVIG was initiated.\nMR imaging performed showed marked right hemisphere volume loss, affecting both gray and white mater (Figure ). Despite the marked asymmetry on imaging findings, EEG monitoring during this advanced stage of disease revealed bihemispheric electrographic pathology; in addition to PLDs in the right posterior quadrant, PLDs and spikes were observed over the left temporal region as well as bilateral independent frontal spikes (Figure ). By family and caregiver report, patient’s overall seizure frequency decreased over the 5-month period she was treated with immunotherapy. As there was no significant change in the patient’s overall neurologic functional status, decision was made by family to discontinue IVIG infusions and pursue hospice.\nWritten informed consent was obtained from patients or next of kin for publication of these three case reports.