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.
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.