The patient is a 13-year-old male who initially presented at the age of 4 years with seizures, marked by staring, twitching of the left eyelid and projectile vomiting. He experienced anywhere between 10–60 seizures a day. Starting at the age of 7 years, after multiple failed attempts to control the seizures using various pharmacologic regimens, the patient underwent the first of 7 surgical resections. The first two surgeries were performed outside the United States and consisted of resections of the left hippocampus and neocortex and the left anterior temporal lobe and hippocampus. Nonspecific diagnoses of gliosis were reportedly made in examining the resected tissue from the first two surgeries. A third surgical resection for continued seizures at the age of 10 years included resections of the left frontal lobe and portion of the left temporal lobe; the pathologic diagnosis was focal cortical dysplasia, Palmini type IA []. The patient was seizure-free for 14 months postoperatively but eventually developed seizure recurrence. Three subsequent surgeries within a 3-week period ensued and included resections of the left temporal lobe, orbitofrontal region, and insula. The pathology in all three cases was interpreted as representing Palmini type IA focal cortical dysplasia. Seizures recurred within 2 weeks of the sixth surgery and persisted for the next 5 months. A seventh surgery included a left frontoparietal resection.\nHistopathologic examination of tissue from the final surgical resection showed focal evidence of contusional damage, related to previous surgery. Areas of cortical architectural disorganization marked by an abnormal layering pattern with focal absence of cortical layer two and occasional enlarged and dysmorphic neurons characterized by an atypical distribution of Nissl substance in the cytoplasm were observed (). The findings were interpreted as being consistent with a Palmini type IIA pattern of focal cortical dysplasia. Balloon cells were not identified. Additionally, multiple foci of perivascular meningeal and parenchymal chronic inflammation consisting of benign appearing lymphocytes were observed (). Most of the lymphocytes stained with T-cell antibody to CD3 (prediluted, Ventana, Tucson, AZ, USA) (); only rare CD20 (1 : 25 dilution, Dako, Carpenteria, CA, USA) positive staining B lymphocytes were present. Infiltration of the parenchyma by scattered lymphocytes was highlighted on the CD3 immunostain. Distributed primarily within the cortex were small collections of microglial nodules, highlighted with an CD68 immunostain (1 : 60 dilution, Dako, Carpenteria, CA, USA) (). Viral inclusions were not identified. Neutrophils, eosinophils and granulomatous inflammation were not observed. Focally, prominent cortical atrophy with gliosis was observed (). The histopathologic findings were interpreted as representing Rasmussen's encephalitis associated with focal cortical dysplasia (Palmini et al. type IIA pattern or ILAE focal cortical dysplasia type IIId) [].
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.