"Mirror EPC": epilepsia partialis continua shifting sides after rolandic resection in dysplasia.

BACKGROUND: Epilepsia partialis continua (EPC) is a life-threatening condition often caused by focal cortical dysplasia (FCD). Resection of the motor cortex is contemplated in the hope that the trade-off between a severe motor deficit and complete seizure control justifies the procedure. METHODS: Report of 3 patients with EPC due to histologically confirmed FCD, who underwent resection of the motor cortex under acute electrocorticography. RESULTS: All had re-emergence of medically intractable EPC in the other side of the body after rolandic resection. Two patients died and the third continues with refractory attacks. CONCLUSION: In some instances, EPC due to FCD may shift sides and re-emerge in the contralateral, previously asymptomatic, hemibody. A mechanism of disinhibition by surgery of a suppressed contralateral and homologous epileptogenic zone is speculated.

Long-term outcome after limited cortical resections in two cases of adult-onset Rasmussen encephalitis.

Rasmussen encephalitis (RE) is a progressive inflammatory disorder characterized by brain hemiatrophy, unilateral focal deficits, and drug-refractory focal epilepsy. Epilepsia partialis continua (EPC) is a hallmark of the disease. Several immunomodulatory treatments may slow but not halt the disease progression. The treatment of choice still relies on surgical hemispheric disconnection, which is burdened by heavy neurologic morbidity. More limited cortical resections, although more tolerable, are usually considered to be, at best, only transiently effective in RE. Hemispheric disconnections may be not feasible when neurologic functions are preserved and the dominant hemisphere is affected. Adult patients with a milder RE course that preserves neurologic function for a long period are particularly at risk of developing severe deficits after surgery. In this study we present the histories of two patients with adult-onset RE who have undergone selective cortical resections to control EPC, avoiding, at the same time, the severe postsurgical deficits that may be induced by hemispheric disconnective surgery. The good result obtained on EPC has been stable over a prolonged period; however, this result was not paralleled by the stop of neurologic progression in one of the two cases. A PowerPoint slide summarizing this article is available for download in the Supporting Information section http://dx.doi.org/10.1111/epi.12596/supinfo.

Rasmussen syndrome: an Argentinean experience in 32 patients.

PURPOSE: The aim of this study is to analyze the electroclinical features, treatment, and evolution of patients with Rasmussen syndrome (RS). MATERIALS AND METHODS: We conducted a retrospective, descriptive study in 32 consecutive patients with RS followed between 1990 and 2012. RESULTS: Twenty boys and 12 girls were included in the study. The mean and median ages at onset of the seizures were 6.5 and 7 years, respectively. Twenty-eight cases had epilepsia partialis continua that had started at a mean age of 9.5 years. Fixed hemiparesis occurred within the first two years after seizure onset in 26 patients. The ictal EEG showed a multifocal origin, but confined to the affected hemisphere in all patients. Mild focal atrophy involved the temporo-insular region associated with enlargement of the ipsilateral horn and Sylvian fissure. An abnormal cortical and/or subcortical hyperintense signal was observed in T2 and Flair images in 25 and 17 patients, respectively. T2 hyperintensity and atrophy in the basal ganglia was documented in five patients. Corticosteroids associated with immunoglobulins were used in 25 patients. Surgical treatment was performed in 25 patients. After a mean follow-up of 13 years (range, 2-20) good surgical outcome-- Engel class I--was observed in 23 of 25 patients operated. CONCLUSION: Corticosteroid and intravenous immunoglobulin treatment should be considered in the early stages of the disease. Patients with RS had a good response to surgical excision of the affected hemisphere.

A case of pathological excitability located with navigated-TMS: presurgical evaluation of focal neocortical epilepsy.

PURPOSE: The quality of presurgical evaluation in focal extratemporal epilepsy surgery is highly dependent on precise structural and functional identification of the epileptic focus. Navigated transcranial magnetic stimulation (nTMS) is a tool that combines the spatial information of high-resolution magnetic resonance imaging (MRI) with the functionality of non-invasive cortical stimulation. The non-invasive character of nTMS suggests that it could be a promising tool for presurgical evaluation of cortical excitability. METHODS: Presurgical nTMS evaluation was performed on an 8-year-old boy with left-sided intractable focal epilepsy, somatosensory auras and epilepsia partialis continua. In line with standardized procedures, motor evoked potentials were sought in both hemispheres over perirolandic cortex during simultaneous belly-tendon surface recordings of the first dorsal interosseus muscles. RESULTS: One singular motor-evoked potential (MEP) could be elicited in the unaffected hemisphere. In contrast, in the affected hemisphere MEPs could be elicited over a large area of the cortex even after the stimulation strength was reduced by at least 44%. Latency stratification in the affected hemisphere differentiated a motor from a sensory region of interest. Stimulation over the sensory region induced a sensory aura. The sensory site was concordant with a previous transient diffusion restriction found in an MRI two years prior to nTMS. CONCLUSIONS: NTMS can locate pathological excitability with high spatial precision. Future studies should compare nTMS with direct cortex stimulation, as well as the combination of nTMS with electroencephalography (EEG) in a larger patient-collective.

Cortical resection tailored to awake, intraoperative ictal recordings and motor mapping in the treatment of intractable epilepsia partialis continua: technical case report.

OBJECTIVE: Epilepsia partialis continua (EPC) is a form of status epilepticus that is characterized by continuous simple partial seizures and can occur as a manifestation of a variety of underlying pathological processes. Because these seizures typically take onset within or close to motor cortex, the treatment of refractory EPC with resective surgery risks significant postoperative deficits. CLINICAL PRESENTATION: We describe our experience using ictal recordings obtained intraoperatively during awake craniotomy, in conjunction with direct cortical stimulation mapping, to tailor surgical resections in 2 patients with refractory EPC. Both patients had pan-hemispheric pathologies that made extraoperative recording difficult. INTERVENTION: Awake craniotomy takes advantage of a unique feature of refractory EPC, namely the near-continuous presence of focal seizure activity. It allows the surgeon to record seizures in the operating room and precisely define the anatomic location of epileptic activity, to resect the seizure focus, and to both visually and electrographically confirm successful cessation of EPC after resection, all within a single operation. We used standard methods of awake craniotomy to finely tailor a cortical resection to the epileptogenic cortex while sparing nearby eloquent motor areas. The precision of awake mapping made this approach safe and effective. CONCLUSION: The cases we describe demonstrate the role of focal resection in the treatment of EPC. Standard techniques of awake craniotomy have application in the treatment of this challenging problem.

Periodic lateralized frontal epileptiform discharges with ipsilateral epilepsia partialis continua.

PURPOSE: Lateralized clonic jerks of the extremities during epileptic seizures usually arise from the contralateral rolandic cortex. In our exceptional case one-sided extremity twitches related to epileptiform activity in the ipsilateral frontal lobe. METHODS: Case study with clinical-electroencephalographic correlation. RESULTS: A 47-year-old man suffered destruction of most of his right cerebral hemisphere, including the perirolandic region. Epilepsia partialis continua (EPC) ensued, with clonic jerks of the proximal right upper and lower limbs temporally related to periodic lateralized epileptiform discharges (PLEDs) from the parasagittal region of the right frontal lobe with a variable time interval over 100 ms. Sectioning of the subcortical callosal and projection connections of the frontal lobe abolished the clonic jerks. CONCLUSIONS: We propose the ipsilateral EPC arose from projections from the supplementary motor region to the medullary reticular formation to account for the long and variable latency between PLEDs and jerks.

Lingual epilepsia partialis continua in Rasmussen's encephalitis.

We report an adult male who presented with disabling dysarthria due to epilepsia partialis continua (EPC) of the left half of the tongue. The clinical, brain magnetic resonance imaging and electroencephalographic features were consistent with Rasmussen's encephalitis, although, despite having had the disease for over 15 years, he did not exhibit any hemiparesis. The accompanying video illustrates the lingual EPC and its total resolution following a right frontal opercular focal cortical resection.

[Epilepsia partialis continua (Kojevnikov's syndrome)]. / Epilepsia partialis continua (Kojevnikovs syndrom).

BACKGROUND: Lesions close to the central sulcus may give rise to focal motor seizures of long duration. This condition is called epilepsia partialis continua (Kojevnikov's syndrome). MATERIAL AND METHODS: Over the last two years, the National Centre for Epilepsy in Norway has treated 12 patients with epilepsia partialis continua. We discuss the occurrence, etiologies, semiology, findings from supplementary investigations, and therapeutic options on the basis of relevant literature and our own experience with these patients. RESULTS AND INTERPRETATION: Morphological lesions were found in 10 out of these 12 patients; cortical dysplasia in 3 patients, brain tumour in 2 patients, cerebral infarction in 2 patients, Rasmussen syndrome in 2 patients, and cerebral haemorrhage from an arteriovenous malformation in 1 patient. 9 patients had intermittent periods of jerking lasting from some hours to several days; the remaining 3 had permanent jerks. One of them had had this condition for 44 years. In 11 patients the jerks were localised to the face and/or the hand. The effect of antiepileptic drugs was disappointing; none became seizure-free. Five patients had undergone surgery. Surgical lesionectomy in this brain area is associated with a high risk of damage to eloquent cortex, but multiple subpial transections may have a seizure-blocking effect. One patient with Rasmussen's syndrome became seizure-free after a functional hemispherotomy.

[Rasnmussen syndrome. 7 years' follow-up. Aspects related to cerebral plasticity in epilepsy]. / Síndrome de Rasmussen. Seguimiento de siete años. Aspectos relacionados con plasticidad cerebral en epilepsia.

Present study represents a case follow up for seven years. A case of Epilepsy Partialis Continua (EPC) in a fifteen year old girl was studied with CT, MRI, cerebral SPECT and constantly EEG procedures: The SPECT study showed in right frontal lobe and right temporal lobe hypoperfusion (reduction of regional cerebral blood flow [rCBF]) cerebral zone in the interictal period and hyperperfusion (increase rCBF) in the ictal period in the same hemisphere. The EEG showed abnormal activity consisting in sharp wave and polyphasic sharp wave in frontal and temporal regions. The medical treatment was refractory at all kind of anticonvulsant drugs. In order to improve the refractory epilepsy the team decided an epilepsy surgery with right temporal lobotomy and right frontal corticetomy. This procedure were done at the eight year old and the results were satisfactory immediately after the surgery with absolute control and normal cognitive performance two weeks later the surgery. This patient was follow up for seven years with EEG constantly with normal activity, SPECT and MRI, all studies showed functional hemispherectomy, total absence in the structure and cerebral blood flow in right hemisphere with normal perfusion and structure in left hemisphere. The late control of neuropsychology assessment showed some deficits in the I.Q. cognitive performance, evaluated by the Weschler scale and Machover tests. These findings would be able to demonstrate a phenomenon of brain plasticity and neuronal restoration in the left cerebral hemisphere after to remove the abnormal cerebral epilepsy focus. This procedure might permits the restoration of inadequate neuronal environment and normalization of neural physiological stability. The postulated mechanisms of reorganization of function are unmasking, nonsynaptic diffusion neurotransmission and receptor plasticity, trophic factors, synapsins and neurotransmitters. The neuropsychological mechanism to preserve the functions would be involving in a cortical reorganization with axonal and dendritic development beside sprouting and synaptogenesis. This patient is a good example of neuronal plasticity in epilepsy surgery.

Disconnective hemispherectomy.

Hemispherectomy is a valuable procedure in the management of seizure disorders caused by unilateral hemispheric disease. Modifications to anatomical hemispherectomy have been proposed to reduce the incidence of superficial cerebral hemosiderosis and hydrocephalus while still achieving seizure control. We report on the modification of a previously described disconnective form of hemispherectomy. We used this procedure on 2 children, with the aid of stereotactic navigation in 1 of the 2 cases. This disconnection was achieved via a transventricular route with minimal cortical resection or disruption of the blood supply. Over the 20 months of follow-up, 1 patient achieved complete seizure control, and 1 patient achieved control of previously incapacitating seizures with few minor seizures persisting. Motor function and speech significantly improved in both patients. Blood loss during the two procedures was significantly less than that reported for anatomical hemispherectomy, and so far there have been no signs of postoperative complications. The hospital stay was limited to 7-14 days after surgery.

Isolation of dominant seizure foci by multiple subpial transections.

Dominant seizure foci (DSF) are seizure foci that do not respond to multiple subpial transections (MST). DSF were isolated in 15/31 patients who underwent MST in the past 6 years. These patients had MST over both hemispheres (6 patients) or over multiple lobes of one hemisphere (9 patients). DSF measured 0.5-1 cm in diameter and numbered 1-4 (in each patient). Their excision resulted in dramatic improvement in EEG over wide areas of the cortex. The longest postoperative follow-up is 67 months, with a median of 21 months. Nine of 15 patients are free of seizures, 3/15 have rare seizures, 2/15 have > 90% reduction in seizure frequency, and 1/15 has > 70% reduction in seizure frequency with improvement in motor and speech functions. In summary, DSF are resistant to MST and influence the electrical activity of the surrounding cortex. MST combined with excision of DSF resulted in good seizure control in this series, which included patients with multilobar and bihemispheric seizure foci.

Double pathology in Rasmussen's encephalitis: etiologic considerations.

In a 7-year-old girl with epilepsia partialis continua (EPC) involving the left face, arm, and leg for 1 year, serial neuroimaging studies showed progressive, brain atrophy. Because medical treatment was ineffective, she underwent a large fronto-temporal surgical resection. Neuropathological examination showed loss of lamination and dysplastic neurons, gliosis, microglial nodules, and perivascular cuffing. Such "double pathology" (dysgenesia and a chronic inflammatory process) may have implications for the pathophysiology of Rasmussen's syndrome.

Status epilepticus and acute repetitive seizures in children, adolescents, and young adults: etiology, outcome, and treatment.

Status epilepticus (SE) is one of the most common neurologic emergencies in children, adolescents, and young adults. SE may be due to acute neurologic conditions such as meningitis, encephalitis, or stroke, complicated febrile seizures, intractable epilepsy, degenerative diseases, intoxication, or may be the first manifestation of epilepsy. Initial treatment of convulsive SE is usually with an intravenous benzodiazepine (BZD) [lorazepam (LZP) or diazepam (DZP)], phenobarbital (PB), or phenytoin (PHT). LZP is less likely to cause respiratory depression than DZP and is therefore preferred. Sequelae and risk for recurrence of SE are primarily related to the underlying cause. Refractory SE (RSE) is most often symptomatic of an acute neurologic condition or neurodegenerative disease. Treatment for RSE is difficult, usually requiring intensive support of vital functions. Reported treatments for RSE include very high dose PB, continuous infusions of pentobarbital or BZDs (DZP, midazolam), lidocaine, inhalation anesthesia, and propofol. Outcome is related to underlying cause. Nonconvulsive SE may present as confusion or may mimic psychiatric illness. Response to BZDs is usually rapid but may not be sustained. Rapid initiation of oral or rectal valproate may be useful. Epilepsia partialis continua (EPC) is almost always due to an acute or chronic destructive lesion. Surgical treatment may be the only effective modality in some children with EPC. Acute treatment of breakthrough seizures and clusters of seizures at home with rectal BZDs (usually DZP, 0.2-0.5 mg/kg) may prevent progression to SE in some children and adolescents and reduce the need for visits to emergency facilities.