Stereotactic Body Radiotherapy (SBRT) of Pancreatic Cancer-A Critical Review and Practical Consideration.

Pancreatic cancer is the third leading cause of cancer death in the developed world and is predicted to become the second by 2030. A cure may be achieved only with surgical resection of an early diagnosed disease. Surgery for more advanced disease is challenging and can be contraindicated for many reasons. Neoadjuvant therapy may improve the probability of achieving R0 resection. It consists of systemic treatment followed by radiation therapy applied concurrently or sequentially with cytostatics. A novel approach to irradiation, stereotactic body radiotherapy (SBRT), has the potential to improve treatment results. SBRT can deliver higher doses of radiation to the tumor in only a few treatment fractions. It has attracted significant interest for pancreatic cancer patients, as it is completed quickly, requires less time away from full-dose chemotherapy, and is well-tolerated than conventional radiotherapy. In this review, we aim to provide the reader with a basic overview of current evidence for SBRT indications in the treatment of pancreatic tumors. In the second part of the review, we focus on practical information with respect to SBRT treatment plan preparation the performance of such therapy. Finally, we discuss future directions related to the use of magnetic resonance linear accelerators.

Preoperative Chemoradiotherapy for Gastroesophageal Junction Adenocarcinoma Modified by PET/CT: Results of Virtual Planning Study.

Background and Objectives: The treatment of gastroesophageal junction (GEJ) adenocarcinoma consists of either perioperative chemotherapy or preoperative chemoradiotherapy. Radiotherapy (RT) in the neoadjuvant setting is associated with a higher probability of resections with negative margins (R0) and better tumor regression rate, which might be enhanced by incrementing RT dose with potential impact on treatment results. This virtual planning study demonstrates the feasibility of increasing the dose to GEJ tumor and involved nodes using PET/CT imaging. Materials and Methods: 16 patients from the chemoradiotherapy arm of the phase II GastroPET study were treated by a prescribed dose of 45.0 Gray (Gy) in 25 fractions. PET/CT was performed before treatment. The prescribed dose was virtually boosted on PET/CT-positive areas to 54.0 Gy by 9 Gy in 5 fractions. Dose-volume histograms (DVH) were compared, and normal tissue complication (NTCP) modeling was performed for both dose schedules. Results: DVHs were exceeded in mean heart dose in one case for 45.0 Gy and two cases for 54.0 Gy, peritoneal space volume criterion V45Gy < 195 ccm in three cases for 54.0 Gy and V15Gy < 825 ccm in one case for both dose schedules. The left lung volume of 25 Gy isodose exceeded 10% in most cases for both schedules. The NTCP values for the heart, spine, liver, kidneys and intestines were zero for both schemes. An increase in NTCP value was for lungs (median 3.15% vs. 4.05% for 25 × 1.8 Gy and 25 + 5 × 1.8 Gy, respectively, p = 0.013) and peritoneal space (median values for 25 × 1.8 Gy and 25 + 5 × 1.8 Gy were 3.3% and 14.25%, respectively, p < 0.001). Conclusion: Boosting PET/CT-positive areas in RT of GEJ tumors is feasible, but prospective trials are needed.

Targeted Radiotherapy of the Tumor Cavity after Surgical Resection of Aggressive Recurrent Brain Metastasis: A Case Report.

Postoperative management of patients with brain metastases is controversial. Besides local control, cognitive function and quality of life are the most important outcomes of postoperative radiotherapy. In this case report, we introduce a patient with aggressive recurred solid metastasis treated with repeated surgery and an individual radiotherapy approach in order to highlight that close mutual collaboration leads to a clear benefit for our patients. The local targeted radiotherapy with 35 Gy in 10 fractions was performed with the volumetric modulated arc technique, leading to more than 2.5 years of local control and survival without any of the side effects usually attributed to whole brain radiotherapy.

Stereotactic Body Radiotherapy of Lymph Node Oligometastases.

BACKGROUND: The aim of this retrospective study is to evaluate the efficacy and toxicity of extracranial stereotactic radiotherapy for the treatment of oligometastatic lymph node involvement in the mediastinum, retroperitoneum, and pelvis in a consecutive group of patients from real clinical practice. MATERIAL AND METHODS: Of a total of 50 patients treated between 2011 and 2017, 29 were men and 21 were women, and the mean age was 62 years (median 66 years, range 25-81 years). Patients were most often irradiated in five fractions; the dose was selected according to dose-volume histograms of organs-at-risk in proximity to the planning target volume. The primary objectives were local control (LC), progression-free survival (PFS), time to multiple dissemination not allowing the use of local treatment methods (freedom from widepread dissemination - FFWD), and overall survival (OS). Acute and delayed toxicity were evaluated as well. RESULTS: The median dose equivalent at α/β = 10 (BED10) was 54 Gy (range 48-80 Gy). The median follow-up period was 40.4 months. LC after irradiation was 90% in 1 year and 75% in 3 years. Median time to local progression was not achieved. Patients irradiated with a high dose had significantly better LC than patients irradiated with a low dose; the cut-off was the median of the applied dose (ie BED10 = 54 Gy). Pathological node localization had no significant effect on LC. The median PFS was 8.2 months (95% CI 7.4-11.6 months). PFS in 1 year was 38.5% and 17% in 3 years. The median OS was 37.3 months (95% CI 23.2-51.4 months). One-year OS was 83% and 3-year OS was 51%. The median FFWD was 13.6 months (range 8.7-18.5 months). The one-year FFWD was 55% and the 3-year FFWD was 24%. None of these parameters (PFS, OS, FFWD) was dose or localization dependent. No grade III or IV toxicity was reported. CONCLUSION: Our study shows that targeted stereotactic radiotherapy is a very effective low toxic treatment for oligometastatic lymph node involvement. It can delay cytotoxic chemotherapy and thus improve/maintain the quality of life of patients. Approximately one fifth of patients treated with extracranial stereotactic radiotherapy for oligometastatic lymph node involvement survived without signs of disease for prolonged periods. Future studies should aim at identifying patients who would benefit most from this treatment, adjusting the timing of extracranial stereotactic radiotherapy depending on the treatment strategy, and optimizing the dose prescription. This work was supported by grant of the Ministry of Health of the Czech Republic AZV 19-00354 and by grant of the Ministry of Health of the Czech Republic - Conceptual development of a research organization (MMCI 00209805). The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.

Stereotactic Body Radiotherapy for Lymph Node Oligometastases: Real-World Evidence From 90 Consecutive Patients.

AIMS: To evaluate the efficacy and toxicity of extracranial stereotactic body radiotherapy (SBRT) in the treatment of oligometastatic lymph node involvement in the mediastinum, retroperitoneum, or pelvis, in a consecutive group of patients from real clinical practice outside clinical trials. METHODS: A retrospective analysis of 90 patients with a maximum of four oligometastases and various primary tumors (the most common being colorectal cancers). The endpoints were local control of treated metastases (LC), freedom from widespread dissemination (FFWD), progression-free survival (PFS), overall survival (OS), and freedom from systemic treatment (FFST). Acute and delayed toxicities were also evaluated. RESULTS: The median follow-up after SBRT was 34.9 months. The LC rate at three and five years was 68.4 and 56.3%, respectively. The observed median FFWD was 14.6 months, with a five-year FFWD rate of 33.7%. The median PFS was 9.4 months; the three-year PFS rate was 19.8%. The median FFST was 14.0 months; the five-year FFST rate was 23.5%. The OS rate at three and five years was 61.8 and 39.3%, respectively. Median OS was 53.1 months. The initial dissemination significantly shortened the time to relapse, death, or activation of systemic treatment-LC (HR 4.8, p < 0.001), FFWD (HR 2.8, p = 0.001), PFS (HR 2.1, p = 0.011), FFST (HR 2.4, p = 0.005), OS (HR 2.2, p = 0.034). Patients classified as having radioresistant tumors noticed significantly higher risk in terms of LC (HR 13.8, p = 0.010), FFWD (HR 3.1, p = 0.006), PFS (HR 3.5, p < 0.001), FFST (HR 3.2, p = 0.003). The multivariable analysis detected statistically significantly worse survival outcomes for initially disseminated patients as well as separately in groups divided according to radiosensitivity. No grade III or IV toxicity was reported. CONCLUSION: Our study shows that targeted SBRT is a very effective and low toxic treatment for oligometastatic lymph node involvement. It can delay the indication of cytotoxic chemotherapy and thus improve and maintain patient quality of life. The aim of further studies should focus on identifying patients who benefit most from SBRT, as well as the correct timing and dosage of SBRT in treatment strategy.

In silico study of pseudoprogression in glioblastoma: collaboration of radiologists and radiation oncologists in the estimation of extent of high dose RT region.

BACKGROUND AND AIM: Oncologists play a vital role in the interpretation of radiographic results in glioblastoma patients. Molecular pathology and information on radiation treatment protocols among others are all important for accurate interpretation of radiology images. One important issue that may arise in interpreting such images is the phenomenon of tumor "pseudoprogression"; oncologists need to be able to distinguish this effect from true disease progression.Exact knowledge about the location of high-dose radiotherapy region is needed for valid determination of pseudoprogression according to RANO (Response Assessment in Neuro-Oncology) criteria in neurooncology. The aim of the present study was to evaluate the radiologists' understanding of a radiotherapy high-dose region in routine clinical practice since radiation oncologists do not always report 3-dimensional isodoses when ordering follow up imaging. METHODS: Eight glioblastoma patients who underwent postresection radiotherapy were included in this study. Four radiologists worked with their pre-radiotherapy planning MR, however, they were blinded to RT target volumes which were defined by radiation oncologists according to current guidelines. The aim was to draw target volume for high dose RT fields (that is the region, where they would consider that there may be a pseudoprogression in future MRI scans). Many different indices describing structure differences were analyzed in comparison with original per-protocol RT target volumes. RESULTS: The median volume for RT high dose field was 277 ccm (range 218 to 401 ccm) as defined per protocol by radiation oncologist and 87 ccm (range 32-338) as defined by radiologists (median difference of paired difference 31%, range 15-112%). The Median Dice index of similarity was 0.46 (range 0.14 - 0.78), the median Hausdorff distance 25 mm. CONCLUSION: Continuing effort to improve education on specific procedures in RT and in radiology as well as automatic tools for exporting RT targets is needed in order to increase specificity and sensitivity in response evaluation.

Physiological and fitness differences between cytotypes vary with stress in a grassland perennial herb.

BACKGROUND AND AIMS: Understanding the consequences of polyploidization is a major step towards assessing the importance of this mode of speciation. Most previous studies comparing different cytotypes, however, did so only within a single environment and considered only one group of traits. To take a step further, we need to explore multiple environments and a wide range of traits. The aim of this study was to assess response of diploid and autotetraploid individuals of Knautia arvensis (Dipsacaceae) to two stress conditions, shade or drought. METHODS: We studied eleven photosynthetic, morphological and fitness parameters of the plants over three years in a common garden under ambient conditions and two types of stress. KEY RESULTS: The results indicate strong differences in performance and physiology between cytotypes in ambient conditions. Interestingly, higher fitness in diploids contrasted with more efficient photosynthesis in tetraploids in ambient conditions. However, stress, especially drought, strongly reduced fitness and disrupted function of the photosystems in both cytotypes reducing the between cytotype differences. The results indicate that drought stress reduced function of the photosynthetic processes in both cytotypes but particularly in tetraploids, while fitness reduction was stronger in diploids. CONCLUSIONS: The photosynthesis related traits show higher plasticity in polyploids as theoretically expected, while the fitness related traits show higher plasticity in diploids especially in response to drought. This suggests that between cytotype comparisons need to consider multiple traits and multiple environments to understand the breath of possible responses of different cytotypes to stress. They also show that integrating results based on different traits is not straightforward and call for better mechanistic understanding of the relationships between species photosynthetic activity and fitness. Still, considering multiple environments and multiple species traits is crucial for understanding the drivers of niche differentiation between cytotypes in future studies.

Left hippocampus sparing whole brain radiotherapy (WBRT): A planning study.

AIMS: Unilateral sparing of the dominant (left) hippocampus during whole brain radiotherapy (WBRT) could mitigate cognitive decline, especially verbal memory, similar to the widely investigated bilateral hippocampus avoidance (HA-WBRT). The aim of this planning study is dosimetrical comparison of HA-WBRT with only left hippocampus sparing (LHA-WBRT) plans. METHODS: HA-WBRT plans for 10 patients were prepared in accordance with RTOG 0933 trial and served as baseline for comparisons with several LHA-WBRT plans prepared with an effort: 1) to maintain the same left hippocampus dosimetry ("BEST PTV") and 2) to maintain same dosimetry in planning target volume as in HA-WBRT ("BEST LH"). RESULTS: All HA-WBRT plans met RTOG 0933 protocol criteria with a mean Conformity index 1.09 and mean Homogeneity index (HI) 0.21. Mean right and left hippocampal D100% was 7.8 Gy and 8.5 Gy and mean Dmax 14.0 Gy and 13.8 Gy, respectively. "BEST PTV" plans reduced HI by 31.2% (P=0.005) which is mirrored by lower PTV_D2% (-0.8 Gy, P=0.005) and higher PTV_D98% (+1.3 Gy, P=0.005) as well as decreased optic pathway's Dmax by 1 Gy. In "BEST LH", mean D100% and Dmax for the left hippocampus were significantly reduced by 11.2% (P=0.005) and 10.9% (P=0.005) respectively. CONCLUSIONS: LHA-WBRT could improve target coverage and/or further decrease in dose to spared hippocampus. Future clinical trials must confirm whether statistically significant reduction in left hippocampal dose is also clinically significant.

Why and how to spare the hippocampus during brain radiotherapy: the developing role of hippocampal avoidance in cranial radiotherapy.

The goal of this review is to summarize the rationale for and feasibility of hippocampal sparing techniques during brain irradiation. Radiotherapy is the most effective non-surgical treatment of brain tumors and with the improvement in overall survival for these patients over the last few decades, there is an effort to minimize potential adverse effects leading to possible worsening in quality of life, especially worsening of neurocognitive function. The hippocampus and associated limbic system have long been known to be important in memory formation and pre-clinical models show loss of hippocampal stem cells with radiation as well as changes in architecture and function of mature neurons. Cognitive outcomes in clinical studies are beginning to provide evidence of cognitive effects associated with hippocampal dose and the cognitive benefits of hippocampal sparing. Numerous feasibility planning studies support the feasibility of using modern radiotherapy systems for hippocampal sparing during brain irradiation. Although results of the ongoing phase II and phase III studies are needed to confirm the benefit of hippocampal sparing brain radiotherapy on neurocognitive function, it is now technically and dosimetrically feasible to create hippocampal sparing treatment plans with appropriate irradiation of target volumes. The purpose of this review is to provide a brief overview of studies that provide a rationale for hippocampal avoidance and provide summary of published feasibility studies in order to help clinicians prepare for clinical usage of these complex and challenging techniques.

Stereotactic radiofrequency amygdalohippocampectomy for the treatment of temporal lobe epilepsy: do good neuropsychological and seizure outcomes correlate with hippocampal volume reduction?

Temporal lobe surgery bears the risk of a decline of neuropsychological functions. Stereotactic radiofrequency amygdalohippocampectomy (SAHE) represents an alternative to mesial temporal lobe epilepsy (MTLE) surgery. This study compared neuropsychological results with MRI volumetry of the residual hippocampus. We included 35 patients with drug-resistant MTLE treated by SAHE. MRI volumetry and neuropsychological examinations were performed before and 1 year after SAHE. Each year after SAHE clinical seizure outcome was assessed. One year after SAHE 77% of patients were assessed as Engel Class I, 14% of patients was classified as Engel II and in 9% of patients treatment failed. Two years after SAHE 76% of subjects were classified as Engel Class I, 15% of patients was assessed as Engel II and in 9% of patients treatment failed. Hippocampal volume reduction was 58±17% on the left and 54 ± 27% on the right side. One year after SAHE, intelligence quotients of treated patients increased. Patients showed significant improvement in verbal memory (p=0.039) and the semantic long-term memory subtest (LTM) (p=0.003). Patients treated on the right side improved in verbal memory, delayed recall and LTM. No changes in memory were found in patients treated on the left side. There was a trend between the larger extent of the hippocampal reduction and improvement in visual memory in speech-side operated.

Cognitive outcome after stereotactic amygdalohippocampectomy.

PURPOSE: We sought to determine the neuropsychological outcome after stereotactic radiofrequency amygdalohippocampectomy performed for intractable mesial temporal lobe epilepsy. METHODS: The article describes the cases of 31 patients who were evaluated using the Wechsler Adult Intelligence Scale-Revised and the Wechsler Memory Scale-Revised prior to, and one year after, surgery. KEY FINDINGS: Patients showed increases in their mean Full Scale, Verbal and Performance IQ scores of 4, 3 and 4 IQ points respectively (p<.05). 5 (17.2%), 4 (13.8%) and 4 (13.3%) patients improved in their Full-scale, Verbal and Performance IQ respectively. No significant changes were found in memory performance - with a mean increase of 1, 3 and 0 MQ points in Global, Verbal and Visual memory respectively (p<.05). Global memory improved in 3 (10.3%) patients, verbal memory in 1 (3.4%) and 1 patient (3.3%) showed deterioration in visual memory. SIGNIFICANCE: Our results provide evidence for unchanged memory in patients with MTLE after the procedure. No verbal memory deterioration was detected in any of our patients, while improvements were found in intellectual performance. The results suggest that stereotactic radiofrequency amygdalahippocampectomy could be superior to open surgery in terms of its neurocognitive outcomes. A larger randomised trial of these approaches is justified.

[Temporal lobe epilepsy in adults and possibilities of neurosurgical treatment: the role of magnetic resonance]. / Temporální epilepsie dospelých a moznosti neurochirurgické lécby: úloha magnetické rezonance.

Temporal lobe epilepsy is the most common type of focal epilepsy diagnosed in adult patients. According to the location of seizure generation it is classified as mesial temporal lobe epilepsy and neocortical lateral lobe epilepsy. Diagnosis of temporal lobe epilepsy can be proved by the combination of the clinical manifestation of partial complex seizures, scalp-video EEG monitoring, results of magnetic resonance imaging (MRI) and imaging of interictal fluoro-deoxy-glucose positron emission tomography. Mesial temporal sclerosis is the most common finding on MRI. Temporal lobe epilepsy is the most surgically amenable diagnosis and results of surgery treatments are clearly superior to the prolonged medical therapy; surgical treatment of the mesial temporal epilepsy with mesial temporal sclerosis has the best clinical results. Except for standard microsurgical approaches such as anterior temporal resection and selective amygdalo-hippocampectomy, stereotactic thermocoagulation amygdalo-hippocampectomy is provided in our epilepsy centre. This alternative approach has comparable clinical outcome to the standard surgery approaches in 2 years clinical follow-ups. MRI is important not only in diagnostic procedures, but also in neuronavigation of surgery approaches, per operation control of the extent of resections and postoperative follow-ups, especially in failed epilepsy surgery.

[Nonconvulsive status epilepticus]. / Nekonvulzivní status epilepticus.

The article summarizes basic facts about classification, clinical presentation, EEG diagnostics and treatment of nonconvulsive status epilepticus.

[Contemporary pharmacotherapy of epilepsy]. / Soucasná farmakoterapie epilepsie.

The epilepsies are a frequent group of neurologic disorders. We have witnessed significant advances in their medical treatment recently. About 70 % of newly diagnosed patients are rendered seizure-free. Antiepileptic drug treatment has become more complex. The choice of antiepileptic drug is based on seizure type. However, special patient's needs (drug tolerability, toxicity, ease of use and cost and age, sex and commorbidities) should be considered. The article deals with these special situations. In case of seizure persistence epilepsy surgery could be an option.

Stereotactic radiofrequency amygdalohippocampectomy: does reduction of entorhinal and perirhinal cortices influence good clinical seizure outcome?

PURPOSE: Stereotactic radiofrequency amygdalohippocampectomy (SAHE) has been modified recently in our center for the therapy of mesial temporal epilepsy (MTLE). It has promising clinical results comparable with microsurgical amygdalohippocampectomy despite smaller volume reduction of the hippocampus. We hypothesized that the extent of perirhinal and entorhinal cortex (PRC, EC) reduction could explain the clinical outcome. Therefore, we performed, retrospectively, volumetric analysis of PRC and EC and compared it with the seizure control. METHODS: Twenty-six consecutive patients with MTLE treated by SAHE were included. PRC and EC volumes were measured from magnetic resonance imaging (MRI) records obtained before and 1 year after SAHE. The clinical outcome was assessed each year after SAHE using Engel's classification. KEY FINDINGS: Twenty-six patients were analyzed. The volume of PRC decreased by 46 ± 17% (p < 10(-12) ); EC volume decreased by 56 ± 20% (p < 10(-10) ). Two years after the procedure, 73% of patients were classified as Engel's I, 19% as Engel's II; in 2 (8%) the treatment failed (were reoperated). Eighteen patients finished 3 years follow-up; 72% of them were classified as Engel's I, 17% as Engel's II, and in 2 (11%) above-mentioned patients the treatment failed. Thirteen patients finished 4 years of follow-up, 11 of them as Engel's I. There was no significant correlation of the clinical outcome to PRC and EC volume reductions. SIGNIFICANCE: The clinical effect of SAHE is not clearly explained by the volume reductions of PRC and EC (nor of the hippocampus and the amygdala). It promotes opinion that the extent of resection/destruction is not important for seizure outcomes.

Stereotactic radiofrequency amygdalohippocampectomy in the treatment of mesial temporal lobe epilepsy.

PURPOSE: Minimally invasive percutaneous single trajectory stereotactic radiofrequency amygdalohippocampectomy was used to treat mesial temporal lobe epilepsy (MTLE). The aim of the study was to evaluate complications and effectiveness of this procedure. MATERIALS AND METHODS: A group of 51 patients with MTLE was treated using stereotactic thermo-lesion of amygdalohippocampal complex under local anaesthesia. The target was reached through the occipital approach with a single trajectory using MRI stereotactic localisation. Thermocoagulation of the amygdalohippocampal complex was planned according to the individual anatomy of each patient. Amygdalohippocampectomy was performed using a string electrode with a 10-mm active tip, and 16-38 lesions (median = 25) were performed in all patients along the 30- to 45-mm trajectory (median = 35) in the amygdalohippocampal complex. RESULTS: The procedure was well tolerated by all patients with no severe permanent morbidity; meningitis was recorded in two patients (4%), hematoma was detected in four patients, clinically insignificant in three of them, and one patient required temporary ventricular drainage (2%). Thirty-two patients were followed up over at least 2 years, and the clinical outcomes were evaluated by Engel's classification; 25 of them (78%) were Engel I, five (16%) were Engel II, and two (6%) were Engel IV. CONCLUSIONS: Stereotactic amygdalohippocampectomy is a minimally invasive procedure with low morbidity and good results that can be the method of choice in selected patients with MTLE.

Microsurgical and stereotactic radiofrequency amygdalohippocampectomy for the treatment of mesial temporal lobe epilepsy: different volume reduction, similar clinical seizure control.

We compared stereotactic radiofrequency amygdalohippocampectomy (SAHE) with microsurgical amygdalohippocampectomy (AHE) in a group of 33 patients with mesial temporal lobe epilepsy in terms of hippocampal and amygdalar volume reductions and clinical outcome. In 23 subjects treated by SAHE, the hippocampal volume decreased by 58.0% (20.0; median, quartile range), with p = 10(-4), and the amygdalar volume decreased by 55.2% (23.8), with p = 10(-4). Two years after SAHE, 74% of patients were classified as class I, 22% as class II and 4% as class III. In 10 subjects treated by AHE, 83.5% (11.2) of the hippocampal and 53.1% (53.9) of the amygdalar volumes were removed (p = 0.05 and p = 0.005, respectively). Two years after the operation, 50% of the subjects were classified as class I, 30% as class II and 10% as class III and IV. To conclude, SAHE leads to a similar reduction of the amygdalar volume but to a significantly lower reduction of the hippocampal volume than AHE. The clinical outcome of SAHE is comparable with that of AHE.

Highly unstable sequence interruptions of the CTG repeat in the myotonic dystrophy gene.

Myotonic dystrophy type 1 is caused by the expansion of a CTG repeat in the 3' UTR of the DMPK gene. A length exceeding 50 CTG triplets is pathogenic. Intermediate alleles with 35-49 triplets are not disease-causing but show instability in intergenerational transmissions. We report on the identification of multiple patients with different patterns of CCG and CTC interruptions in the DMPK CTG repeat tract that display unique intergenerational instability. In patients bearing interrupted expanded alleles, the location of the interruptions changed dramatically between generations and the repeats tended to contract. The phenotype for these patients corresponded to the classical form of the disease, but in some cases without muscular dystrophy and possibly with a later onset than expected. Symptomatic patients bearing interrupted intermediate length repeat tracts were also identified, although the role of the interruptions in their phenotype remains unclear. The identification of interruptions in the DMPK repeat has important consequences for molecular genetic testing where they can lead to false negative conclusions.

Stereotactic radiofrequency amygdalohippocampectomy for the treatment of mesial temporal lobe epilepsy: correlation of MRI with clinical seizure outcome.

SUMMARY: Stereotactic radiofrequency amygdalohippocampectomy (AHE) has been reintroduced as an alternative treatment of mesial temporal lobe epilepsy. The aim of this study was to describe MRI changes after stereotactic AHE and to correlate the hippocampal and amygdalar volumes reduction with the clinical seizure outcome. Eighteen patients after stereotactic AHE were included. Volumetry was calculated from pre-operative MRI and from MRI obtained 1 year after the operation. The clinical outcome was examined 1 and 2 years after the treatment. Hippocampal volume decreased by 54+/-19%, and amygdalar volume decreased by 49+/-18%. One year after the procedure, 13 (72%) patients were classified as Engel's Class I (9 as Class IA), 4 (22%) patients as Class II and 1 (6%) patient as Class III. Two years after the operation, 14 patients (82%) were classified as Class I (7 as Class IA) and 3 patients (18%) as Class II. We found 3 surgical complications after the procedure: one small subdural hematoma, and twice a small electrode tip left in operation field (these patients were excluded from the study). In 3 patients, temporary meningeal syndrome developed. Results of radiofrequency AHE are promising. The volume reduction of target structures after AHE is significantly related to the clinical outcome.

Vagus nerve stimulation: longitudinal follow-up of patients treated for 5 years.

We performed a retrospective, multicenter, open-label study to evaluate the efficacy of vagus nerve stimulation (VNS) in all patients in the Czech Republic who have received this treatment for at least 5 years (n=90). The mean last follow-up was 6.6+/-1.1 years (79+/-13 months). The median number of seizures among all patients decreased from 41.2 seizures/month in the prestimulation period to 14.9 seizures/month at 5 years follow-up visit. The mean percentage of seizure reduction was 55.9%. The responder rate in these patients is in concordance with the decrease of overall seizure frequency. At 1 year after beginning the stimulation, 44.4% of patients were responders; this percentage increased to 58.7% after 2 years. At the 5 years last follow-up 64.4% of patients were responders, 15.5% experienced > or = 90% seizure reduction, and 5.5% were seizure-free. A separate analysis of patients younger than 16 years of age showed lower efficacy rates of VNS in comparison to the whole group. Complications and chronic adverse effects occurred in 13.3% of patients. VNS is an effective and safe method to refractory epilepsy in common clinical practice.