Bilateral Pallidal Stimulation in a Family With Myoclonus Dystonia Syndrome Due to a Mutation in the Sarcoglycan Gene.

OBJECTIVES: The study aimed to present a family with myoclonus dystonia (M-D) syndrome due to a mutation in the epsilon sarcoglycan gene (SGCE). Three members of the family suffered from treatment-refractory severe myoclonic jerks of the neck, trunk, and upper extremities. The mild dystonic symptoms recognized as cervical dystonia or truncal dystonia affected all individuals. The efficacy of pharmacotherapy, including anticholinergic, dopaminergic, and serotoninergic drugs, has failed. One individual developed an alcohol dependency and suffered from alcoholic epilepsy. MATERIALS AND METHODS: The patients were referred for stereotactic surgery. All individuals underwent bilateral implantation of deep brain stimulation (DBS) leads into the posteroventrolateral segment of the globus pallidus internus (GPi). Surgeries were uneventful. The formal preoperative objective assessment included the Unified Myoclonus Rating Scale (UMRS) and the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). The postoperative UMRS and BFMDRS assessments were done only under continuous stimulation at 3, 6, and 12 months after the surgery and at the last available follow-up ranging from 6 to 15 months (mean, 10 months follow-up). RESULTS: At the last follow-up visit, the rest and action parts of UMRS were improved by 93.3% and 88.2%, respectively, when compared to the baseline scores. The motor and disability scales of BFMDRS were improved by 77% and 43% at the last follow-up visit compared to the baseline BFMDRS scores. There were no hardware or stimulation-induced complications over the follow-up period. Positive social adjustment allowed two patients to regain jobs and one patient continued his education and hobbies. CONCLUSION: Our experience gathered in three individuals in the family with a mutation in SGCE indicates that bilateral GPi DBS can be an effective and safe treatment for disabling pharmacological resistant, intractable M-D syndrome.

Atypical clinical presentation mimicking stroke in an adult patient caused by a rare diffuse leptomeningeal glioneuronal tumour.

A diffuse leptomeningeal glioneuronal tumours (DLGNT) are very rare tumours of the central nervous system, typically characterized by enhancement of subarachnoid space with cystic lesions, diffuse leptomeningeal infiltration, and no primary mass. We report an atypical clinical presentation of DLGNT. A 48-year-old male was admitted to hospital with symptoms of ischaemic stroke. Magnetic resonance imaging of the head revealed contrast enhancement of the meninges and other parts of the brain. A stereotactic frame biopsy was performed on the patient, which revealed the DLGNT. Diffuse leptomeningeal glioneuronal tumours are mostly seen in individuals less than 18 years old and are characterized by slow growth and low-grade histological appearance. Diffuse leptomeningeal glioneuronal tumours can be aggressive in adults.

Rare iatrogenic iliac artery injury during lumbar disc surgery - a case report.

Damage to large abdominal vessels during lumbar discectomy surgery is a rare but life-threatening complication. A CASE REPORT: The authors present the case of a 57-year-old patient who received surgery for critical degenerative lumbal spinal stenosis on the L4-L5 level. The diagnosis was based on strong right sciatica and neurogenic claudication. A bilateral laminotomy from the right and a microdiscectomy were performed. During surgery, no bleeding from the intervertebral space was observed and blood pressure was low but stable from the beginning. After surgery, the patient was in good general and neurological condition, without preoperative right-sided sciatica. Within a few hours after the operation, the circulatory and respiratory systems were stable with normal saturation and the patient did not report shortness of breath. Paleness of the skin and mucous membranes was observed. Follow-up morphology tests performed at 6 and 10.5 hours after surgery showed a decrease in the level of erythrocytes. The patient had palpable tenderness in the left hypochondriac region. Suspicion of bleeding into the abdominal cavity from arteries or iliac veins was stated. Immediately, an angio-computed tomography (CT) of the abdominal cavity was performed, which confirmed the presence of a hematoma in the peritoneal space and a pseudoaneurysm of the left iliac artery. The patient was urgently transported to the Vascular Surgery Clinic, where a Y-type covered stent was implanted percutaneously into both iliac arteries. After the procedure, there were symptoms of ischaemia in the left lower extremity and intermittent claudication. A Doppler study showed signs of narrowing at the stent level on the left side. The patient was reoperated after a CT check-up and a second stent was implanted into the left iliac artery, which allowed vasodilation and true flow in the artery. CONCLUSIONS: The authors suggest that both the neurosurgeon and anaesthesiologist should have been aware of the possibility of such a rare but life-threatening complication as iliac vessel damage during lumbar discectomy surgery. A quick diagnosis and implementation of a proper procedure reduces the high mortality rate caused by this complication. In cases of a sudden unjustified drop in blood pressure during lumbar discectomy, an immediate laparotomy should be performed to find and repair the site of laceration of a vessel. In patients who are stable hemodynamically, performing an angio-CT function of the abdominal cavity is suggested and the damaged artery should be treated with a covered stent.

Recognition of biological movements and cognitive functioning in patients with central nervous system tumours.

Humans have a remarkable ability to perceive, identify, recognize and interpret biological movements. Point-light diplays (PLD) is the only method used to represent trajectories of human movements in visual material. Biological movement recognition might be disturbed in some disorders. AIM: The aim of the study was to examine if people with central nervous system (CNS) tumors bear deficits in recognizing biological movement. In addition, authors of this study decided to examine whether these deficits are associated with cognitive functions (especially executive functions). MATERIALS AND METHODS: The study was performed at Neurosurgical Department of the Institute of Psychiatry and Neurology in Warsaw, Poland. The patients who were admitted to Neurosurgical Department in the purpose of brain tumour removal were recruited to the study group. The control group consisted of healthy people. Neuropsychological tools (ACE-III, Rey figure, CTT, Ruff Figural Fluidity Test, WAIS-R Digit Span) and a program for biological motions recognition. RESULTS: In terms of age, education level and gender no statistical differences were observed between the control group and the research group. Our study revealed statistically significant differences in the recognition of biological movement in the control and study group. The correlation analysis between EF and biological movement recognition were found in the following domain of neurophysiological tests: RFFT unique combinations, M-ACE, ACE-III visual-spatial functions, CTT interference indicator. CONCLUSIONS: People affected by brain tumours have impaired ability to recognize biological movements.

Deep brain stimulation for the treatment of severe intractable anorexia nervosa.

Anorexia nervosa (AN) is a challenging multifactorial disorder with the highest mortality rate among all psychiatric disorders. Due to the low rate of long-term treatment success, new treatment options are needed. Here, we review Deep Brain Stimulation (DBS) as a treatment of Severe Intractable AN. The mechanisms of AN have shown significant involvement of the central nervous system especially the same brain regions which are involved with obsessive-compulsive disorder (OCD) and major depressive disorder (MDD). AN and OCD display many similarities in psychiatric practice such as compulsive behaviours and anxiety levels, which are related to networks in the brain, that can be altered using DBS. Our literature search revealed 8 studies totalling 28 individuals with AN and comorbid OCD or MDD. The most common stereotactic targets included the sub-callosal cingulate cortex (Brodmann area 25)/medial forebrain bundle (MFB) for AN and comorbid MDD and nucleus accumbens (NAc)/ventral striatum for AN and comorbid OCD. In most cases bilateral DBS of various structures of the reward system achieved good results in BMI, and core AN symptoms and psychiatric comorbidities showed sustained improvement. DBS is a promising treatment modality for AN and comorbid OCD or MDD. These results highlight promise and hope for patients with AN. However, further studies with larger patient populations are needed to shed light on the long-term outcomes of DBS and its effects in AN treatment.

Depression, sexual satisfaction, and other psychological issues in women with polycystic ovary syndrome.

Polycystic ovary syndrome (PCOS) increases the risk of depression, poor quality of life, and low sexual satisfaction of women. The aim of the study was to evaluate the prevalence of these disorders and to assess the need for psychological consultation at the time of PCOS diagnosis. A case-control single-center study of 250 women who were diagnosed with PCOS voluntarily filled in an anonymous, interactive questionnaire. The inquiry form included 27 questions covering the woman's characteristics, satisfaction with their outer appearance and sexual life, as well as the Beck Depression Inventory (BDI). Based on the BDI, 52% (130/250) of studied women presented depressive symptoms. This group had significantly higher body mass index (29.5 ± 8.1 vs. 24.6 ± 5.8; p<.001) and a lower level of self-attractiveness than other studied women (3.3 ± 2.2 vs. 5.4 ± 2.3; p<.001). Forty two percent (106/250) of women viewed themselves as unattractive. A correlation between reduced sexual satisfaction and a sense of low attractiveness was noted (r = 0.465, p<.001). Sixty four percent (160/250) of women believed that psychological consultation should be offered to all PCOS women. Treatment of PCOS women should be multidisciplinary and include psychological counseling, especially in obese PCOS women with inadequate family support and dissatisfied with their sexual life.

Labour in women with gestational diabetes mellitus.

OBJECTIVES: Gestational diabetes mellitus (GDM) constitutes one of the most common pregnancy complications and affects 3-5% of all pregnancies, with its incidence still growing. Due to possible maternal and fetal complications, the peripartum management of GDM patients continues to be a debatable issue. The aim of the study was to analyse the course and final way of delivery in women with gestational diabetes mellitus. The effectiveness of induction of labour (IOL) was also assessed and factors predisposing to cesarean section were identified. MATERIAL AND METHODS: The study group consisted of 204 women with GDM who delivered in the Academic Centre for Woman's and Neonate's Health in Warsaw over the years 2013 and 2014. The indications and ratios of elective and intrapartum cesarean sections were analysed. Patients qualified for induction of labour were compared depending on their final way of delivery. RESULTS: Over a half of all deliveries in the study group (53%) were cesarean sections. Elective surgeries accounted for 70% of all cesarean sections, predominantly due to a history of previous operational deliveries. Only 12% of the study group developed spontaneous uterine contractions and delivered vaginally. A comparison of the vaginal delivery group (n = 96) with cesarean section group (n = 108) pointed to high pregestational BMI value and advanced maternal age as factors increasing the patient's risk for surgical delivery (p = 0.0000 and p = 0.048 accordingly). The comparison of women undergo-ing IOL vs. omen with spontaneous uterine contractions showed no increase in the ratio of intrapartum cesarean sections in the IOL group - in both subgroups vaginal delivery was achieved in 75% of cases. CONCLUSIONS: Patients with GDM are more likely to undergo cesarean section, but the implementation of induction of labour at term does not further aggravate this risk. Major risk factors for operational delivery in GDM population included: advanced maternal age, high pregestational BMI value and undergoing insulin therapy.

Multifocal drug-resistant epilepsy in a patient with a newly discovered mutation in tuberous sclerosis complex 1 gene treated by deep brain stimulation in the anterior thalamic nucleus.

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutations in the tumor suppressor genes TSC1 or TSC2. TSC is characterized by the formation of multiple tumors in various organs. The most common neurological manifestation of the disorder is epilepsy present in 79-90% of cases. At least one-third of TSC patients develop drug-resistant epilepsy (DRE) which remains a great challenge for clinicians. Neuromodulation is an option in cases of multifocal epilepsy, epilepsy originating in eloquent areas, or the inability to identify the ictal onset zone. Deep brain stimulation of the anterior thalamic nucleus (ANT-DBS) may be used in the treatment of multifocal DRE. Here, we present a case of a patient with multifocal DRE caused by TSC, who was treated with ANT-DBS. A follow-up period of eight months showed that the patient's multifocal DRE was successfully treated by ANT-DBS.

Skin-related complications following deep brain stimulation surgery: A single-center retrospective analysis of 525 patients who underwent DBS surgery.

BACKGROUND: Although Deep Brain Stimulation (DBS) is a safe and proven treatment modality for patients suffering from debilitating movement and neuropsychiatric disorders, it is not free from complications. Management of skin erosion and infection following DBS surgery constitutes a challenge in everyday clinical practice. OBJECTIVES: Skin-related complications were evaluated in patients who underwent DBS surgery due to Parkinson's disease (PD), dystonia, essential tremor (ET), and other indications including Tourette syndrome (TS), Obsessive-Compulsive Disorder (OCD), and epilepsy. METHODS: A retrospective analysis of clinical data was performed on patients who underwent DBS surgery between November 2008 and September 2021 at the Department of Neurosurgery, Institute of Psychiatry and Neurology, Warsaw. RESULTS: 525 patients who underwent 927 DBS leads implantations were included in the analysis. There were 398 patients with PD, 80 with dystonia, 26 with ET, 7 with drug-resistant epilepsy, 5 with Multiple Sclerosis, 4 with Holme's or cerebellar tremor, 3 with TS, and 2 with OCD. 42 patients (8,0%) had 78 skin infection episodes. The overall level of skin erosion was 3,8% (20/525 patients). The risk of developing infection episode was connected with younger age at diagnosis (p = 0.017) and at surgery (p = 0.023), whereas the development of skin erosion was connected with the dystonia diagnosis (p = 0.012). Patients with dystonia showed the highest rate of infections and erosions (11/70 and 7/70 patients retrospectively). DISCUSSION: Postoperative skin complications are a serious side effect of DBS surgery. CONCLUSION: Our study suggests that dystonic patients are at higher risk of developing skin-related complications after DBS surgery.

Deep brain stimulation in a patient with progressive myoclonic epilepsy and ataxia due to potassium channel mutation (MEAK). A case report and review of the literature.

Progressive myoclonic epilepsy (PME) is characterized by prominent myoclonus, generalized tonic-clonic seizures, and less often focal, tonic, or absence seizures. The KCNC1 mutation is responsible for specific clinical phenotype of PME which has been defined as myoclonic epilepsy and ataxia due to potassium channel mutation (MEAK). We present a case of a 44 years-old male patient with genetically proven MEAK who underwent subthalamic nucleus/substantia nigra (STN/SNr) deep brain stimulation (DBS) for his pharmacological-refractory myoclonus and drug-resistant epilepsy (DRE). Since the age of 4-5 years, the patient had been suffering from intention tremor, and later the myoclonic jerks, ataxia involving the upper limbs and walking difficulties worsened. The first bilateral tonic-clonic seizure (BTCS) occurred at the age of 22. The patient agreed to staged bilateral implantation of DBS electrodes placed in the STN/SNr region. The follow-up lasts more than 24 months. The myoclonic jerks assessed by Unified Myoclonus Rating Scale (UMRS) were reduced by nearly 70 % and BTCS was completely abolished. The patient's ataxia and dysarthria did not improve. Early diagnosis with genetic testing may significantly help in counseling patients with PME and enables to undertake the surgical approach targeting the STN/SNr.

Clinical efficacy and safety of anterior thalamic deep brain stimulation for intractable drug resistant epilepsy.

BACKGROUND: Deep brain stimulation of the anterior nucleus of the thalamus (ANT DBS) is a neuromodulation therapy for patients with refractory focal seizures evolving into bilateral tonic-clonic seizures when pharmacotherapy as well other neuromodulation techniques including vagus nerve stimulation or responsive neurostimulation have failed. OBJECTIVE: We performed a prospective single-center study investigating the clinical efficacy and exact ANT DBS lead location in patients with DRE. METHODS: The primary outcome measure was the proportion of patients with more than 50 % reduction in diary-recorded seizures when compared to three preoperative months (baseline seizure frequency). The close postoperative follow-up was performed every 3 months. The seizure frequency, stimulation settings and adverse events were closely monitored during follow-up visits. We also analyzed the seizure outcome with location of ANT DBS active contacts. RESULTS: Between May 2020 and October 2022, 10 adult patients with a mean age of 38.5 years (range, 30-48 years) underwent bilateral ANT DBS surgery (mean duration of DRE 28.6 years, range 16-41 years). The median seizure count in three months period preceding surgery (baseline seizure count) was 43.2 (range, 4-150). Nine patients achieved more than 50 % seizure reduction at the last follow-up (mean range 3-33 13.6 months, months). ANT DBS caused seizure reduction 3 months after procedure as well as at last follow-up by 60.4 % and 73.3 %, respectively. Due to relatively small number of studying individuals we cannot precisely locate the area within ANT associated with good clinical outcome. Patients with temporal lobe epilepsy had a remarkable reduction of seizure frequency. No patient suffered transient or permanent neurological deficits. CONCLUSIONS: Clinical efficacy of ANT DBS may support more widespread utilization of this neuromodulation technique especially for seizures originating from temporal lobes.

Deep Brain Stimulation for Morbid Obesity: An Underutilized Neuromodulatory Treatment for Severely Obese Patients?

BACKGROUND: Morbid obesity (MO) has been steadily increasing in the last few years. Pharmacotherapy and bariatric surgeries remain the main treatment modalities for MO, although in the long-term they may lose their effectiveness. Other treatment approaches are urgently needed and deep brain stimulation (DBS) is a promising therapy. Disturbed energy homeostasis caused by intake of highly palatable and caloric foods may induce hedonic eating. The brain nuclei responsible for energy homeostasis and hedonia are the hypothalamic nuclei and nucleus accumbens. These brain structures constitute the stereotactic targets approached with DBS to treat MO. MATERIAL AND METHODS: We have performed a literature search of all available clinical applications of DBS for MO in humans. We were able to identify three case series reports and additional six case reports involving 16 patients. The selected stereotactic targets included lateral hypothalamus in eight patients, ventromedial hypothalamus in two patients, and nucleus accumbens in six patients. RESULTS: In general, the safety profile of DBS in refractory MO patients was good. Clinical improvement regarding the mean body mass index could be observed in obese patients. CONCLUSIONS: MO is a demanding condition. Since in some cases standardized treatment is ineffective, new therapies should be implemented. DBS is a promising therapy that might be used in patients suffering from MO, however, more studies incorporating more individuals and with a longer follow-up are needed to obtain more reliable results concerning its effectiveness and safety profile.

DNA Hydroxymethylation in High-Grade Gliomas.

BACKGROUND: Since the new World Health Organization (WHO) classification of nervous system tumors (2016, revised, 4th edition) has been released, gliomas are classified depending on molecular and genetic markers in connection with histopathology, instead of histopathology itself as it was in the previous classification. Over the last years, epigenetic analysis has taken on increased importance in the diagnosis and treatment of different cancers. Multiple studies confirmed that deoxyribonucleic acid (DNA) methylation and hydroxymethylation play an important role in the regulation of gene expression during carcinogenesis. METHODS: In this review, we aim to present the current state of knowledge on DNA hydroxymethylation in human high-grade gliomas (WHO grades III and IV). RESULTS: The correlation between DNA hydroxymethylation and survival in glioblastoma multiforme (GBM) patients was evaluated by different studies. The majority of them showed that the expression of 5-hydroxymethylcytosine (5-hmC) and ten-eleven translocation (TET) enzymes were significantly reduced, sometimes almost undetectable in high-grade gliomas in comparison with the normal brain. A decreased level of 5-hmC was associated with poor survival in patients, but high expression of the TET3 enzyme was related to a better prognosis for GBM patients. This points to the relevance of DNA hydroxymethylation in molecular diagnostics of human gliomas, including survival estimation or differentiating patients in terms of response to the treatment. CONCLUSION: Future studies may shed some more light on this epigenetic mechanism involved in the pathogenesis of human high-grade gliomas and help develop new targeted therapies.

The Profiles of Tet-Mediated DNA Hydroxymethylation in Human Gliomas.

Gliomas are the most common primary malignant intracranial brain tumors. Their proliferative and invasive behavior is controlled by various epigenetic mechanisms. 5-hydroxymethylcytosine (5-hmC) is one of the epigenetic DNA modifications that employs ten-eleven translocation (TET) enzymes to its oxidation. Previous studies demonstrated altered expression of 5-hmC across gliomagenesis. However, its contribution to the initiation and progression of human gliomas still remains unknown. To characterize the expression profiles of 5-hmC and TET in human glioma samples we used the EpiJET 5-hmC and 5-mC Analysis Kit, quantitative real-time PCR, and Western blot analysis. A continuous decline of 5-hmC levels was observed in solid tissue across glioma grades. However, in glioblastoma (GBM), we documented uncommon heterogeneity in 5-hmC expression. Further analysis showed that the levels of TET proteins, but not their transcripts, may influence the 5-hmC abundance in GBM. Early tumor-related biomarkers may also be provided by the study of aberrant DNA hydroxymethylation in the blood of glioma patients. Therefore, we explored the patterns of TET transcripts in plasma samples and we found that their profiles were variously regulated, with significant value for TET2. The results of our study confirmed that DNA hydroxymethylation is an important mechanism involved in the pathogenesis of gliomas, with particular reference to glioblastoma. Heterogeneity of 5-hmC and TET proteins expression across GBM may provide novel insight into define subtype-specific patterns of hydroxymethylome, and thus help to interpret the heterogeneous outcomes of patients with the same disease.

Atypical clinical presentation of glioblastoma mimicking autoimmune meningitis in an adult.

Glioblastoma (GBM) is the most malignant type of glial tumor associated with a very unfavorable prognosis. Typical radiological features of GBM include the presence of a tumor with irregular contrast-enhancing margins and central necrosis surrounded by a wide area of vasogenic edema. Here, we presented an atypical clinical presentation of GBM mimicking autoimmune meningitis. A 69-years-old previously healthy male was admitted to the emergency room due to signs of increasing cognitive impairment, weight loss, changes in behavior, difficulty in walking, and prolonged episodes of nausea over the past month. An magnetic resonance imaging (MRI) brain scan revealed hyperintense changes of the periventricular area surrounding brain ventricles in T2 and FLAIR, and post-contrast leptomeningeal enhancement and thickening of meninges involving cerebellar sulci. An additional MRI scan of the cervical spine showed an in-core contrastenhancing lesion on the C7-Th1 level as well as leptomeningeal thickening and post-contrast-enhancement around the spinal cord. Various laboratory tests and two stereotactic biopsies were performed with no essential to diagnosis clinical findings. A couple of months after first hospital admission, the patient died. Post-mortem examination of the brain revealed numerous foci of abnormal tissue inside the subarachnoid space, lateral ventricles, and cerebral aqueduct. Histological examination showed diffuse malignant astroglial neoplasm, and diagnosis of glioblastoma NOS WHO G IV was established. Even though the appearance of usual GBM is widely recognizable, one must bear in mind the possibility of unusual presentation. The presented case highlights the diagnostic difficulties of diffuse glioblastoma with atypical clinical presentation.

Deep brain stimulation of the medial forebrain bundle for treatment-resistant depression - a narrative literature review.

Purpose: Deep brain stimulation (DBS) is a relatively new and still experimental treatment modality for treatment-resistant depression (TRD). There is preliminary evidence that stimulation of brain reward circuit structures or their connecting white matter bundles may exert an antidepressant effect. The main nucleus of the reward circuit is the nucleus accumbens (NAc), which plays a critical role in reward-seeking behavior, motivation, and addiction. Also, white matter bundles connecting different structures of the reward circuit have been studied clinically as targets for DBS, including the medial forebrain bundle (MFB) - a central component of the mesolimbic dopaminergic reward circuit. This review aims to present the clinical outcomes of MFB DBS for TRD. Views: The scientific literature was reviewed using the following keywords: 'DBS', 'major depressive disorders', 'TRD', and 'MFB'. The identified studies were assessed on the basis of patient characteristics, clinical outcomes, and adverse events related to DBS. The search revealed five open-label clinical case studies and four case reports reporting the cumulative number of 35 patients treated by MFB DBS for TRD. Conclusions: The current clinical data of MFB DBS are limited by small sample size and the small number of clinical open-label trials. There is an urgent need for more clinical trials targeting the MFB for TRD. The results obtained in these studies showed a very rapid antidepressant effect observed within one week after the start of stimulation. MFB DBS for TRD should be considered as a last resort treatment due to its invasive character. However, this treatment may be a promising alternative for TRD patients.

Clinical Efficacy and Safety Profile of Anterior Thalamic Stimulation for Intractable Epilepsy.

INTRODUCTION: Deep brain stimulation of the anterior nucleus of the thalamus (ANT DBS) is a neuromodulation therapy for patients with refractory partial seizures. The ANT is the structure of a limbic system with abundant neuronal connections to temporal and frontal brain regions that participate in seizure propagation circuitry. STATE OF THE ART: We have performed a literature search regarding the clinical efficacy of ANT DBS. We discuss the surgical technique of the implantation of DBS electrodes with special attention paid to the targeting methods of the ANT. Moreover, we present in detail the clinical efficacy of ANT DBS, with a special emphasis on the stimulation parameters, a stimulation mode, and polarity. We also report all adverse events and present the current limitations of ANT DBS. CLINICAL IMPLICATIONS: In general, the safety profile of DBS in intractable epilepsy patients is good, with a low rate of surgery, hardware-related, and stimulation-induced adverse events. No significant cognitive declines or worsening of depressive symptoms was noted. At long-term follow-up, the quality-of-life scores have improved. The limitations of ANT DBS studies include a limited number of patients treated and mostly open-label designs with only one double-blind, randomized multicenter trial. Most studies do not report the etiology of intractable epilepsy or they include nonhomogeneous groups of patients affected by intractable epilepsy. There are no guidelines for setting initial stimulation parameters. All the variables mentioned may have a profound impact on the final outcome. CONCLUSIONS: ANT DBS appears to be a safe and efficacious treatment, particularly in patients with refractory partial seizures (three-quarters of patients gained at least 50% seizure reduction after 5 years). ANT DBS reduces most effectively the seizures originating in the temporal and frontal lobes. The published results of ANT DBS highlight promise and hope for patients with intractable epilepsy.

Deep brain stimulation for the treatment of refractory and super-refractory status epilepticus.

BACKGROUND: Status epilepticus (SE) and super-refractory status epilepticus (SRSE) are life-threatening medical emergencies. The first-line treatment for SE or SRSE includes i. v. anesthetic agents with respiratory support, AEDs, and steroids. Surgical interventions are indicated if medical treatments fail to abrupt the seizures. Apart from resective epilepsy surgery employed for the treatment of SE and SRSE, deep brain stimulation (DBS) of thalamic nuclei is aimed at directly influencing the brain function. OBJECTIVE: The main objective of this review is to present the efficacy and safety of DBS in SE and SRSE in humans. METHODS: We performed the literature search regarding DBS therapy in the treatment of SE and SRSE. All manuscripts written in English have been analyzed and included in this review. RESULTS: We were able to find 7 case reports describing DBS outcomes for the treatment of SE or SRSE in 8 patients. Six patients operated for SE or SRSE gained a significant seizure reduction or total abolition of seizures. One patient due to an infection and the other one due to a stimulation-related adverse event required the total DBS hardware removal. CONCLUSIONS: Case reports suggest that DBS can be considered as one of the treatment choices in SE and SRSE cases. A small number of treated cases and various thalamic nuclei targeted by DBS electrodes preclude drawing definitive conclusions. It can be speculated that a shorter duration of refractory SE or SRSE before a DBS procedure may efficiently reduce seizure frequency.