Marina Casazza

Rasmussen's encephalitis: Early characteristics allow diagnosis

Objective: To identify early manifestations of Rasmussen encephalitis (RE) that can prompt early and reasonably secure diagnosis, allowing medical or surgical therapies at an early stage when they may be more effective in slowing the disease. Methods: The authors studied 12 patients with clinical and neuropathologic diagnosis of RE, followed from disease onset, assessing clinical history, imaging, and EEG and focusing on early characteristics. Anti-GluR3 antibody assays were also considered in 11 patients. Results: By 4 months from first symptoms, all cases had 1) refractory focal seizures with a predominant motor component, 2) slow focal activity on EEG contralateral to the motor manifestations, and 3) focal contralateral white matter hyperintensity with insular cortical atrophy on neuroimaging. Less constant or later findings were epilepsia partialis continua, oligoclonal bands, and serum anti-GluR3 antibodies. Conclusions: The association of partial seizures with focal EEG and neuroimaging changes allows a tentative diagnosis of RE 4 to 6 months after first symptoms.

Cerebral cavernomas and seizures: a retrospective study on 163 patients who underwent pure lesionectomy.

The objective was to evaluate the outcome of microsurgical “pure” lesionectomy in patients with supratentorial cavernous angiomas presenting with seizures. For this retrospective study 163 patients with cavernoma-related epileptic seizures were selected. They all underwent surgery in a single institution between 1988 and 2003. A microsurgical frame/frameless guided minimally invasive transulcal “pure” lesionectomy was performed. The haemosiderin stained gliotic brain parenchyma that was usually found surrounding the lesion was not removed. Among the 99 patients with epilepsy and longer clinical history, 68 (68.7%) were found completely to be seizure-free, 10 (10.1%) presented sporadic and less frequent seizures and 17 (17.1%) remained unchanged. Sixty-three out of 64 (98.4%) patients who experienced only single or sporadic seizures were found to be completely seizure-free after surgery. Five patients were lost at follow-up (mean 48 months, range 0.5–14 years). Long-term morbidity was 1.8%. Mortality was null. No haemorrhagic episodes were observed during follow-up. Pure lesionectomy prevents bleeding and development of epilepsy in patients that receive early surgery after the epileptic onset. In most of the epileptic patients with a good concordance between the electroclinical data and the location of the angioma, good results can be achieved by this kind of surgery so that more invasive and costly studies to find and remove the epileptogenic cerebral parenchyma seem justified only after lesionectomy fails.

Vagal nerve stimulation: Relationship between outcome and electroclinical seizure pattern

In recent years, vagal nerve stimulation (VNS) has been proposed as a possible way to improve the control of refractory (partial and generalized) seizures. To date, however, there is no complete understanding of the underlying mechanism for this action nor are there any available guidelines or criteria for the selection of those candidates that might be most suitable for this kind of neuromodulating surgery. This report presents evidence that should be helpful in defining the clinical criteria for using VNS for the treatment of refractory seizures. We report on 17 patients with severe partial refractory epilepsy and polymorphous seizures, who have been operated on previously or who were excluded from epilepsy surgery and for whom, at least, one seizure type has been electrographically recorded. Sixteen of these patients also had falling seizures. Our objective was to identify responders and to correlate the outcome of their seizures with the EEGraphic onset of their seizure. Follow-up ranged from 4 to 9 years. The results of this study indicate a significant reduction of seizures in only four patients and better outcome in patients where the onset of seizure activity occurred in the temporal area. Patients with frontal or frontocentral seizures resulted in the poorest outcomes. In four patients with Lennox-Gastaut syndrome VNS produced no significant reduction of seizures, while falling seizures decreased significantly in three patients with retropulsive falls. These results of this small series of patients suggest that VNS might be more suitable in patients with temporal rather than frontal or central seizure onset. Further studies are required to support this hypothesis.

Focal epilepsies in adult patients attending two epilepsy centers: classification of drug-resistance, assessment of risk factors, and usefulness of "new" antiepileptic drugs

Purpose:  To classify the grade of antiepileptic drug (AED) resistance in a cohort of patients with focal epilepsies, to recognize the risk factors for AED resistance, and to estimate the helpfulness of “new‐generation” AEDs.

Vagus nerve stimulation in refractory epilepsy: New indications and outcome assessment

Although vagus nerve stimulation (VNS) is an effective alternative option for patients with refractory epilepsy unsuitable for conventional resective surgery, predictors of a better control of seizure frequency and severity are still unavailable. This prospective study reports on 39 patients, including 4 children affected by epilepsia partialis continua (EPC), who underwent VNS for refractory epilepsy. The overall seizure frequency outcome was classified into three groups according to reduction rate: ≥75%, ≥50%, and <50%. Engel and McHugh classifications were also used. The median follow-up period was 36months. A seizure reduction rate ≥50% or EPC improvement was observed in 74% of the patients. Twenty-one out of 35 cases (60%) resulted in Engel classes II and III. Outcome, as defined by the McHugh scale, showed a responder rate of 71%. These results suggest that younger patient age and focal or multifocal epilepsy are related to a better seizure control and cognitive outcome. Vagus nerve stimulation could also be considered as an effective procedure in severe conditions, such as drug-refractory EPC.

Preserved memory in temporal lobe epilepsy patients after surgery for low-grade tumour. A pilot study

The objective was to carry out a pilot study exploring memory outcome in patients with temporal lobe epilepsy (TLE) and low-grade tumour. A prospective study using a competence-related memory assessment was carried out in the Laboratory of Neuropsychology, Epilepsy Center and Neurosurgical Department of the “C. Besta” National Neurological Institute in 24 TLE patients undergoing surgical resection for left (n=12) or right (n=12) low-grade tumours and 36 healthy subjects. Patients underwent mesial or lateral temporal lobe lesionectomy. Neuropsychological tests exploring verbal and visual short-term memory, learning, delayed recall and ability to control interference in memory were applied. Before and after surgery, significant verbal impairment was present in left TLE patients compared to controls and right TLE patients, and visual deficits were present in both groups compared to controls. After surgery, there was no significant decrease in mean verbal or visual memory scores related to the operated side. Some memory abilities subserved by the contralateral temporal lobe improved. Postoperative memory scores were related to preoperative scores, side of operation, age and education. In patients with TLE and low-grade tumour, temporal lobe surgery does not necessarily induce memory deficits. Improvement of memory abilities subserved by the unoperated temporal lobe may be expected.

Self-rated and assessed cognitive functions in epilepsy: impact on quality of life.

AIM OF THE STUDY To compare the effects of perceived and assessed cognitive functions on quality of life (QoL) in patients with epilepsy (PWE). METHODS The study analyzed the data from a series of PWE who compiled the Quality of Life in Epilepsy-89 Inventory (QOLIE-89) and the Multiple Ability Self-Report Questionnaire (MASQ) for QoL and perceived cognitive abilities, respectively. The State-Trait Anxiety and Beck Depression inventories were used to assess mood. Neuropsychological tests evaluated abstract reasoning, attention, conceptual-motor tracking, constructional praxis, language, verbal and non-verbal memory, abstraction, category shifting, verbal fluency, and visual-spatial abilities. RESULTS The QOLIE-89 overall score was predicted by the Mood and Attention and Executive Functions factors and MASQ scores, explaining 38, 6, and 4% of its variance, while disease duration, seizure frequency, and schooling determined 16%. The QOLIE-89 Psychosocial, Cognitive, and Physical Performance sub-domains related to mood. The Cognitive and Physical Performance factors also related to the MASQ and Attention and Executive Functions factor scores, respectively. CONCLUSIONS In PWE, self-rated and assessed cognitive deficits may influence QoL, explaining 10% of its variance irrespective from mood and clinical variables. Treating cognitive deficits and their perception may help improve QoL.

Ictal EEG fast activity in west syndrome : From onset to outcome

Purpose: To characterize the fast EEG activities associated with infantile spasms in West syndrome, and their value in predicting the recurrence and localization of late seizures.

Vagus nerve stimulation for drug-resistant Epilepsia Partialis Continua: Report of four cases

BACKGROUND Vagus nerve stimulation (VNS) represents an adjunctive surgical option for adult and pediatric patients with drug-resistant epilepsy, who are not eligible for surgical resection or disconnection. However, little is known on its efficacy in the treatment of Epilepsia Partialis Continua (EPC), a rare but serious form of motor status epilepticus associated either with progressive or with non-evolving neurological diseases. PURPOSE AND METHODS To evaluate the effect of VNS in a series of four children affected by medically unresponsive EPC secondary to chronic inflammatory encephalopathy (two cases), Rasmussen encephalitis (one case) and poliodystrophy (one case). RESULTS After VNS implantation, the stimulation amplitude was progressively increased and, after a mean interval of 47 days, a partial reduction of EPC and associated focal seizures was observed in all patients. After a mean follow-up of three years, one child stopped EPC, two presented short and rare episodes and in one patient 2-3 residual seizures per day was reported. In all cases, reduction of epileptic activity was associated with mild improvement of motor and cognitive abilities. No serious side effects were reported. CONCLUSION VNS may be considered as an option for EPC when medical treatment fails and other more invasive neurosurgical options are not feasible.

Hemispherotomy in Rasmussen encephalitis: Long-term outcome in an Italian series of 16 patients

Surgical disconnection of the affected hemisphere is considered the treatment of choice for Rasmussen encephalitis (RE), however few data on long-term outcomes after disconnective surgery are available. We report on long-term seizure, cognitive and motor outcomes after disconnective surgery in 16 (8 M, 8 F) RE patients. Pre- and post-operative evaluations included long-term video-EEG monitoring, MRI, assessment of motor function, and cognitive evaluation. Hemispherotomy, by various techniques was used to obtain functional disconnection of the affected hemisphere. The patients, of median current age 23.5 years, range 12-33, were operated on between 1993 and 2009. Median age at disease onset was 5.8 years (range 3-11.4). Median time from seizure onset to surgery was 3.8 years, range 8 months to 21 years. Post-surgical follow-up was a median of 9.5 years, range 3-20. At surgery all patients were receiving two or more antiepileptic drugs (AEDs). All but three patients were seizure-free at latest follow-up. AEDs had been stopped in ten patients; in the remaining six AEDs were markedly reduced. Postural control improved in all patients. Gain in cognitive functioning was significantly (p=0.002) related to disease duration. The long-term outcomes, in terms of seizure control, motor improvement, and cognitive improvement provide important support for disconnective surgery as first choice treatment for RE.