Polish cross-cultural adaptation of a disease-specific quality-of-life instrument: The Penn Acoustic Neuroma Quality-of-Life Scale.

BACKGROUND: The medical community has shown a growing interest in developing methods for measuring and comparing objective patient outcomes coupled with subjective patient assessments. Questionnaires enable healthcare professionals to obtain the patient's perspective about their experienced vestibular schwannomas (VS) symptoms quickly. To date, in Poland, a cross-cultural adapted version of a disease-specific questionnaire for the measurement of quality of life (QoL) in patients with VS has not been produced. OBJECTIVES: This study aimed to adapt the questionnaire evaluating disease-specific QoL in patients with VS (Penn Acoustic Neuroma Quality-of-Life Scale; PANQOL) to Polish and evaluate its psychometric properties. MATERIAL AND METHODS: One-hundred twenty-four patients aged between 24 and 85 years (mean (M) = 60.17 ±standard deviation (SD) = 13.27) diagnosed with VS and treated with Gamma Knife were included in the study. We used a questionnaire translated from English into Polish by a bilingual professional, verified through a back-translation. The final version consisted of 26 items. The internal consistency of the Polish version of the PANQOL scale domains was measured using the Cronbach's alpha (α). To verify the validity of PANQOL subscales, a correlation analysis was conducted between the domains of PANQOL and other questionnaires, including the Assessment of Quality of Life (AQoL-8D), the Glasgow Benefit Inventory (GBI), the 5 Well-Being Index (WHO-5), the Skarzynski Tinnitus Scale (STS) for the presence of dizziness, and the Gardner-Robertson classes. RESULTS: The majority of PANQOL domains showed excellent or good internal consistency (for a PANQOL total of 0.934; for subscales in the range of 0.916-0.424). Our analysis showed strong correlations between the total PANQOL score and AQoL-8D utility score, as well as between the subscales. We observed weak to moderately significant relationships between GBI and PANQOL domains (r = 0.18-0.43), the WHO-5 (r = 0.18-0.56) and the STS scale (r = -0.40- -0.19). CONCLUSIONS: The results demonstrated that the POL-PANQOL is a reliable and valid questionnaire for measuring QoL.

Subtotal resection of vestibular schwannoma with subsequent Gamma Knife irradiation - tumor growth control, facial and cochlear nerve outcome - preliminary results.

<b>Introduction:</b> The choice of treatment of vestibular schwannoma (VS) depends on several factors, including the tumor size, the patient's age and overall health, and the presence and severity of symptoms.<b>Aim:</b> The aim of the study was to assess the effectiveness of intentional subtotal resection (STR) of tumor followed by Gamma Knife surgery (GKS) in patients with larger VS (Koos 3 and 4).<b>Materials and methods:</b> The retrospective analysis was performed on 18 patients. Data of VS volumes measured in MRI, the facial nerve function assessed in the House-Brackmann scoring system (HB), and the results of audiological examination expressed on the Gardner-Robertson scale (GR) were collected preoperatively, postoperatively, and post-GKS.<b>Results:</b> Preoperatively, the main symptom was hearing loss observed in 13 out of 18 patients. The facial nerve function was assessed as HB 1 in 16, whereas HB 2 in 2 patients. The mean volume of the tumor in the initial MRI amounted to 16.81 cm³ . Postoperatively, the facial nerve was assessed as HB 1 or 2 in 16, whereas HB 3 in 2 patients. Serviceable hearing was presented by only 4 persons. The Mean diameter of the tumor after subtotal surgery amounted to 3.16 cm³ , 1.83 cm³ after GKS, and 1.58 cm³ at the last follow-up. The facial nerve function and hearing level remained the same as before GKS in all patients.<b>Conclusions:</b> STR followed by GKS can be a safe and effective method of treatment of large VS concerning the functional outcome of the facial nerve and the tumor volume growth control.

Planned Subtotal Resection following Stereotactic Radiosurgery of Koos 3 and 4 Vestibular Schwannomas.

Background/Objectives: Surgical resection of medium to large vestibular schwannomas (VSs, Koos grade 3 and 4) is a widely used approach, although stereotactic radiosurgery (SRS) is increasingly proposed as initial treatment. The quality of life-centered approach is challenged in cases where tumor growth control cannot be achieved with SRS, thus necessitating salvage surgery. We present a series of eight consecutive patients who required surgery due to continued tumor growth after SRS. Methods: Of the 146 patients with VS grades 3 and 4 initially treated with SRS, only eight patients (mean age, 54 ± 7.2 years; range, 42-63 years) required subsequent surgery. Their mean tumor volume was 9.9 ± 3.2 cm3. The mean time from SRS to first tumor progression and planned subtotal resection was 23 ± 5.9 months and 45 ± 17.5 months, respectively. SRS was not performed after the surgery in favor of a "wait and rescan" approach. Tumor residue was monitored on follow-up magnetic resonance imaging. In all patients, tumor growth control after planned subtotal resection was maintained at 63 ± 19.8 months. Results: None of the 146 patients had serious complications after SRS. In the eight patients who required surgery, tumor growth between 22% and 212% (mean, 4 cm3) was reported within 26 to 84 months after SRS. Before salvage surgery, they scored 1 point on the House-Brackmann scale. Subtotal excision was performed, and VIIth nerve function was preserved in all patients. At 63 ± 19.8 months, 3 patients had a House-Brackmann score of 1, four patients had a score of 2, and one patient had a score of 3. Conclusions: Surgical excision of medium to large VS after SRS can be relatively safe, provided that a quality of life-centered approach of subtotal resection is used.

Polish cross-cultural adaptation of the Glasgow Benefit Inventory as an instrument for the post-intervention measurement of change after Gamma Knife treatment.

<b>Introduction:</b> The Glasgow Benefit Inventory (GBI) is one of questionnaires for the measurement of treatment success, which is widely used in patients after vestibular schwannoma treatment.<b>Aim:</b> This study aimed to adapt the GBI originally written in English to Polish conditions and to evaluate its psychometric properties.<b>Material and methods:</b> The Polish version of the GBI used in this study was prepared by a bilingual translator, and its accuracy was ensured by back-translation. To identify the structure of the Polish version of the GBI, exploratory factor analysis was performed. The reliability was determined using Cronbach's α. To verify the validity, correlation analysis was conducted between the domains of GBI and other questionnaires.<b>Results:</b> The results of confirmatory factor analysis indicated that the original 3-factor model is not a good fit for the data. The exploratory factor analysis was performed and identified 4 factors. The total variance of the 4-factor solution was 66.25%. The Cronbach's α calculated for the total GBI reached 0.91. Our analysis confirmed significant weak or moderate correlations between tools for the measurement of general QoL (AQoL-8D: r = 0.411; p = 0.000) and health-related QoL (PANQOL: r = 0.367; p = 0.000; a tool dedicated for patients with VS), well-being (WHO-5: r = 0.432; p = 0.000), hearing and tinnitus (STS: r = -0.217; p = 0.016).<b>Conclusions:</b> This study reports the first adaptation of the GBI for patients with VS to Polish conditions. The results demonstrated that the Polish version of the GBI is a reliable and valid questionnaire that can be used to measure treatment success.

Unilateral gamma knife thalamotomy for tremor safety and efficacy in multimodal assessment: a prospective case-control study with two-year follow-up.

INTRODUCTION: Unilateral gamma knife thalamotomy (GKT) is a treatment option for pharmacoresistant tremor of various aetiologies. There have been to date no randomised controlled trials performed to assess its safety and efficacy. Our aim was to summarise a two-year multimodal observation of patients with tremor caused by Parkinson's Disease (PD) or essential tremor (ET). MATERIAL AND METHODS: 23 patients with PD (n = 12) or ET (n = 11) were included. They underwent assessments before, V0 (n = 23), and 12 months, V12 (n = 23), and 24 months, V24 (n = 15), after unilateral GKT. Patients were assessed with psychological tests and acoustic voice analysis. Tremor assessment was performed with a digitising table using the Fahn-Tolosa-Marin rating scale (FTMRS). The Unified Parkinson's Disease rating scale part III (UPDRS-III) was also used in the PD group. Gait and balance was assessed using clinical tests, stabilometric platform, and treadmill. RESULTS: No side effects were observed in a two-year follow-up. There was no notable deterioration observed in the patients' psychological evaluation, speech, or assessment of gait and balance. The scores were significantly lower (p = 0.01) in parts A and B of FTMRS one year after GKT. In post hoc analysis, the scores did not differ significantly between V0 and V24. In FTMRS part C (activities of daily living), no significant change was observed. There was no significant difference in total UPDRS part III score or in score of UPDRS part III domains 3 and 4 ('tremor at rest' and 'action and postural tremor of hands') between measurements. CONCLUSIONS: UGKT may be a safe treatment modality if performed in an experienced centre. Tremor reduction may diminish over time, and UGKT did not lead to cognitive, gait or speech deterioration in a long-term observation.

Outcomes and Prognostic Factors in the Treatment of Intracanalicular Vestibular Schwannomas Using Gamma Knife Stereotactic Radiation.

BACKGROUND: No gold standard has been developed for the therapy of intracanalicular vestibular schwannomas (IVS). Options for treatment include a conservative approach, microsurgery, or radiosurgery. Although the efficacy of these treatment has been well-documented, little is known about the determinants of outcome in IVSs following radiosurgery. Therefore, we examined the results in relation to age, gender, tumor volume, distance to fundus, microcyst existence, and radiosensitivity in this group. In addition, we investigated possible predictors of facial nerve function and hearing preservation. METHODS: Ninety-four patients with unilateral IVS were included in the evaluation (52 women and 42 males). The patients were separated into younger and older age groups based on their median age (55 years). The median IVS volume was 138 mm3, microcysts were identified in 16 tumors, and 63 tumors were adjacent to the fundus. The data were analyzed using Statistica software package ver. 13.3. RESULTS: At final follow-up, a statistically significant decrease in tumor volume and no statistically significant decline in hearing were noted, but no differences between age groups were found. The sex had no effect on overall tumor growth control, facial nerve preservation, or hearing preservation. Localization of IVS close to the fundus and the presence of tumor microcysts had no effect on the control of tumor growth, preservation of hearing, and sparing of facial nerve following radiosurgery. Cochlear dose had no influence on hearing preservation. Higher tumor volume was associated with its pseudoprogression during early follow-up and a greater risk of hearing loss. CONCLUSIONS: Age, sex, tumor volume, proximity to the fundus, and the existence of a microcyst were not predictive of radiosensitivity nor preservation of facial nerve function and hearing, based on the findings. There was no effect of cochlear dose on hearing. Initial greater tumor volume was associated with an increased probability of tumor pseudoprogression.

The S-100B level, intracranial pressure, body temperature, and transcranial blood flow velocities predict the outcome of the treatment of severe brain injury.

This study evaluates the applicability of S100B levels, mean maximum velocity (Vmean) over time, pulsatility index (PI), intracranial pressure (ICP), and body temperature (T) for the prediction of the treatment of patients with traumatic brain injury (TBI). Sixty patients defined by the Glasgow Coma Scale score ≤ 8 were stratified using the Glasgow Coma Scale into 2 groups: favorable (FG: Glasgow Outcome Scale ≥ 4) and unfavorable (UG: Glasgow Outcome Scale < 4). The S100B concentration was at the time of hospital admission. Vmean was measured using transcranial Doppler. PI was derived from a transcranial Doppler examination. T was measured in the temporal artery. The differences in mean between FG and UG were tested using a bootstrap test of 10,000 repetitions with replacement. Changes in S100B, Vmean, PI, ICP, and T levels stratified by the group were calculated using the one-way aligned rank transform for nonparametric factorial analysis of variance. The reference ranges for the levels of S100B, Vmean, and PI were 0.05 to 0.23 µg/L, 30.8 to 73.17 cm/s, and 0.62 to 1.13, respectively. Both groups were defined by an increase in Vmean, a decrease in S100B, PI, and ICP levels; and a virtually constant T. The unfavorable outcome is defined by significantly higher levels of all parameters, except T. A favorable outcome is defined by S100B < 3 mg/L, PI < 2.86, ICP > 25 mm Hg, and Vmean > 40 cm/s. The relationships provided may serve as indicators of the results of the TBI treatment.

Tumor control and hearing preservation after radiosurgery of intracanalicular vestibular schwannomas - systematic review.

Intracanalicular vestibular schwannomas (IVS) account for 8% of all vestibular schwannomas and their detection is still increasing due to high availability of magnetic resonance (MRI). Radiosurgery is one of several commonly acceptable methods of IVS treatment, but some risk may still exist with that treatment. The aim of this study is to analyze the clinical outcomes in tumor control and hearing preservation after radiosurgery of IVS. The retrospective analysis included 14 scientific papers available in the PubMed database. Assessment of tumor volume was performed based on gadolinium-enhanced T1-weighted scans. Hearing preservation was assessed using the Gardner-Robertson classification (GR class). Statistical analysis was performed using IBM SPSS Statistics 27. It was revealed that tumor growth control in IVS treated with radiosurgery was higher than in the wait-and-see strategy. The hearing preservation was similar in patients after wait and see and the surgical group. Radiosurgery was associated with low risk of facial nerve dysfunction.

Prognostic properties of the association between the S-100B protein levels and the mean cerebral blood flow velocity in patients diagnosed with severe traumatic brain injury.

Craniocerebral injury (CBI) is tissue damage caused by a sudden mechanical force. CBI can result in neurological, neuropsychological and psychiatric dysfunctions. Currently, the severity of CBI is assessed using the Glasgow Coma Scale, brain perfusion pressure measurements, transcranial Doppler tests and biochemical markers. This study aimed to determine the applicability of the S-100B protein levels and the time-averaged mean maximum cerebral blood flow velocity (Vmean) as a means of predicting the treatment outcomes of CBI in the first 4 days of hospitalization. The results validated the standard reference ranges previously proposed for the concentration of S-100B (0.05-0.23 µg/l) and the mean of cerebral blood flow velocity (30.9 to 74.1 cm/sec). The following stratification scheme was used to predict the success of treatment: Patients with a Glasgow Outcome Scale (GOS) score ≥4 or GOS <4 were stratified into 'favorable' and 'unfavorable' groups, respectively. The favorable group showed relatively constant levels of the S-100B protein close to the normal range and exhibited an increase in Vmean, but this was still within the normal range. The unfavorable group exhibited a high level of S-100B protein and increased Vmean outside of the normal ranges. The changes in the levels of S-100B in the unfavorable and favorable groups were -0.03 and -0.006 mg/l/h, respectively. Furthermore, the rate of decrease in the Vmean value in the unfavorable and favorable groups were -0.26 and -0.18 cm/sec/h, respectively. This study showed that constant levels of S-100B protein, even slightly above the normal range, associated with an increase in Vmean was indicative of a positive therapeutic outcome. However, additional research is required to obtain the appropriate statistical strength required for clinical practice.

Usability of the Level of the S100B Protein, the Gosling Pulsatility Index, and the Jugular Venous Oxygen Saturation for the Prediction of Mortality and Morbidity in Patients with Severe Traumatic Brain Injury.

The high frequency of traumatic brain injury imposes severe economic stress on health and insurance services. The objective of this study was to analyze the association between the serum S100B protein, the Gosling pulsatility index (PI), and the level of oxygen saturation at the tip of the internal jugular vein (SjVO2%) in patients diagnosed with severe TBI. The severity of TBI was assessed by a GCS score ≤ 8 stratified by Glasgow outcome scale (GOS) measured on the day of discharge from the hospital. Two groups were included: GOS < 4 (unfavorable group (UG)) and GOS ≥ 4 (favorable group (UG)). S100B levels were higher in the UG than in the FG. PI levels in the UG were also substantially higher than in the FG. There were similar levels of SjVO2 in the two groups. This study confirmed that serum S100B levels were higher in patients with unfavorable outcomes than in those with favorable outcomes. Moreover, a clear demarcation in PI between unfavorable and FGs was observed. This report shows that mortality and morbidity rates in patients with traumatic brain injury can be assessed within the first 4 days of hospitalization using the S100B protein, PI values, and SjVO2.

Current strategies for the treatment of malignant gliomas - experience of the Department of Neurosurgery, Brodno Masovian Hospital in Warsaw.

INTRODUCTION: Malignant gliomas (HGG) are the most common primary malignant brain tumors arising from glial cells. Between HGG, glioblastoma is the most common and the most malignant histological subtype with only a 27% 2-year survival rate. Current standard medical treatment of malignant gliomas is still not satisfactory, and may need some development and modification. We presented and discussed the achievements of the Department of Neurosurgery at Brodno Masovian Hospital in the treatment of malignant gliomas. MATERIAL AND METHODS: We step by step presented and discussed the policy in the treatment of malignant gliomas. We showed all steps starting from preparation of surgery (eg. neuroimaging) and finishing on the presentation the development of perioperative management - from intraoperative electrical stimulation mapping and monitoring which is nowadays already standard method to convection-enhanced delivery (CED) and gamma knife (GK) which are new and promising methods in the treatment of glioblastoma. RESULTS: All surgical methods described in this manuscript were introduced to achieve maximal and safe resection of malignant glioma. CED and GK are the last resort methods for patients with recurrent HGG. DISCUSSION: Department of Neurosurgery at Brodno Masovian Hospital deal with all types of brain tumors, including all types of high grade gliomas. As the first Department in Europe with close cooperation with the Department of Neurosurgery in San Francisco, we have started local infusions of drugs directly to the tumor in the real time of magnetic field, and we think that technology may change all approaches to the treatment of high grade gliomas.

Gamma knife surgery in the treatment of intracanalicular vestibular schwannomas.

OBJECTIVE: According to the literature, gamma knife surgery (GKS) is a promising method for intracanalicular vestibular schwannoma (IVS) management, providing excellent tumor growth control rates (91%-100%) and good hearing preservation rates (41%-76%), but this evidence originates primarily from a small series of patients. The aim of this study was to present the outcomes of GKS in the largest group of patients with IVS studied to date, with particular emphasis on the long-term outcomes of treatment. METHODS: The study included 136 consecutive patients with unilateral IVS, who underwent GKS in 2011-2015. Mean age of the patients was 54 ± 12.6 years. All patients were operated on with a 192-source cobalt-60 gamma knife unit. All patients had complete follow-up documentation and the mean duration of the follow-up was 52 ± 13.8 months (6-83 months). Neurological status (facial and trigeminal nerve), hearing and instability/dizziness presence were determined prior to GKS, immediately after the procedure, and during the follow-up visits. RESULTS: Tumor growth control was obtained in 124/136 (~91.2%) patients. Hearing improvement was observed in 32/136 (23.5%) patients, and there was a distinct cluster of 9 patients (6.6%) regaining serviceable hearing after GKS, whereas in 36 patients (26.5%) was stable. Four patients developed facial nerve dysfunction, including 3 periodic hemifacial spasm and 1 partial paresis, which resolved spontaneously within 12 months of GKS. None of the operated patients showed new, debilitating neurological deficits, including trigeminal sensory disturbances or hydrocephalus. CONCLUSIONS: GKS is a highly effective treatment for IVS, associated with low morbidity and good tumor growth control.

Successful bilateral pallidal stimulation in a patient with isolated lower limb dystonia coexistent with Langerhans cell histiocytosis and coeliac disease.

The authors report a case of bilateral globus pallidus internus (GPi) stimulation for treatment of medically intractable isolated lower limb dystonia. The 14-year-old girl developed dystonic movements in her left lower limb. At the age of 17, the patient was handicapped by dystonic movements in her lower limbs, and became wheelchair-bound. Pharmacological therapy and botulinum toxin injection resulted in transient and modest benefit. Moreover, the patient was diagnosed with histologically proven coeliac disease and Langerhans cell histiocytosis. Genetic testing revealed the presence of DYT-1 mutation. The 17-year-old girl underwent bilateral implantation of deep brain stimulation leads. Bilateral GPi stimulation resulted in remarkable improvement of phasic dystonic movements, and dystonic posture of lower limbs. Over 2 years postoperative follow-up, the patient is able to walk independently. Bilateral GPi stimulation appears to be an effective treatment modality for isolated lower limb dystonia.

Chronic bilateral pallidal stimulation in patients with generalized primary dystonia - multi-contact cathodal stimulation is superior to bipolar stimulation mode. Preliminary results.

BACKGROUND AND PURPOSE: Primary generalized dystonia (PGD) is a medically refractory progressive disease of the brain causing near total handicap of affected patients. The aim of the study was to assess the efficacy and safety of bilateral pallidal stimulation in patients with PGD. MATERIAL AND METHODS: The study population is composed of 5 patients with PGD. The formal objective assessment included the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). All stereotactic procedures were performed in general anaesthesia using a Leksell G stereotactic head frame without electrophysiological guidance. Immediately after insertion of deep brain stimulation (DBS) leads, the internal pulse generators (Itrel II or Soletra) were implanted subcutaneously in the chest wall or abdominal region. RESULTS: There were no complications related to the stereotactic procedures. The hardware-related complications (two broken DBS leads) were replaced successfully. There were no infections or erosions of implanted hardware. It has been observed that in the long-term follow-up period primary set bipolar stimulation mode lost its benefit achieved previously. Various stimulation combinations were investigated. Monopolar cathodal or especially multi-contact cathodal stimulation was the most effective one. The efficacy of bilateral pallidal stimulation was proved by the objective validated BFMDRS at long-term follow-up. CONCLUSIONS: Response to DBS may improve with the number of activated cathodal contacts within the globus pallidus internus.

Bilateral subthalamic nucleus stimulation in the treatment of advanced Parkinson's disease. Five years' personal experience.

BACKGROUND AND PURPOSE: The objective of the study was to assess bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) for patients with advanced Parkinson disease (PD). MATERIAL AND METHODS: The study population included 5 patients with bilateral STN DBS who completed a 5-year postoperative follow-up period. In all patients electrodes (Model 3387 or 3389) were stereotactically bilaterally inserted into the STN using a Leksell stereotactic G frame. The clinical rating tests included Unified Parkinson's Disease Rating Scale (UPDRS) and two motor-timed tests derived from CAPIT (rapid movements between two points and stand-walk-sit test). All patients were assessed in off and on condition before implantation and 1, 3 and 5 years in medication on and off condition and stimulation on condition and stimulation off condition. To compare preoperative to postoperative UPDRS scores, only mean values and standard deviations are presented because of the small study population. RESULTS: The stimulation effect was noted in the off state, resulting in a 59% improvement in motor scores of UPDRS at 5-year follow-up, when compared to preoperative scores. In the on state the stimulation improved motor scores by 17%. At 5-year follow-up, reduction of daily levodopa dose was 50%. CONCLUSIONS: Bilateral STN DBS is an effective and safe treatment for patients with advanced PD. Bilateral STN DBS contributes to improvement of parkinsonian symptoms in the off state and levodopa-induced dyskinesia. This can be correlated with a 50% reduction of daily levodopa dose 5 years postoperatively.

A patient with parkinsonism related to Gaucher's disease type I successfully treated by unilateral pallidotomy - a 3-year follow-up.

Gaucher's disease is the most common hereditary lysosomal storage disorder and presents with a wide clinical spectrum including parkinsonism. We present a patient first diagnosed with idiopathic Parkinson's disease at the age of 38 due to left-sided bradykinesia and rigidity. But thereafter the diagnosis of Gaucher's disease type I according to clinical pictures and clinical genetic testing was made. Despite the optimal enzyme replacement therapy, the parkinsonian symptoms worsened. He was referred for consideration for surgical treatment of his severe parkinsonism. At the age of 54 he underwent right-sided posteroventrolateral pallidotomy. The surgery was uneventful and he immediately improved. On the last examination performed 3 years later, he had mild parkinsonism and was independent in all activities of daily living. This case demonstrates a benefit of surgical treatment for genetically proven Gaucher's disease type I.

[Deep brain stimulation in the surgical management of major depressive disorder and obsessive-compulsive disorder]. / Gleboka stymulacja mózgu w leczeniu depresji i zaburzen obsesyjno-kompulsyjnych.

Despite significant advances in psychopharmacology, many patients with psychiatric disorders remain severely impaired by their conditions in their private, professional and social lives. Deep brain stimulation (DBS) is a proven method of surgical management of Parkinson's disease, dystonia and essential tremor. Electrodes implanted in deep brain structures have a direct neuromodulatory effect on neuronal structures responsible not only for movement disorders but also for psychiatric disorders. The stereotactic targets for psychiatric disorders include limbic parts of the nuclei of the limbic neuronal loop. At present, DBS is applied to treat medically refractory obsessive-compulsive disorder, depression and Tourette syndrome in small groups of patients. This overview includes the rationale for the use of DBS in psychiatric disorders, a summary of preliminary research done to date, and a discussion of achieved results.

Chronic bilateral pallidal stimulation in a patient with DYT-1 positive primary generalized dystonia. A long-term follow-up study.

Primary generalized dystonia is a medically refractory progressive disease of the brain causing near total functional handicap of affected patients. The authors present the effectiveness of bilateral globus pallidus internus (GPi) stimulation in one patient with primary DYT-1 positive dystonia. Pharmacotherapy completely failed to control generalized dystonic movements. The patient was referred for surgical treatment and underwent bilateral implantation of DBS leads in the GPi. The formal objective assessment included the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). Clinical and functional BFMDRS assessments were performed before and after surgery till 5 years postoperatively. All stimulation-induced side effects were reversible. Chronic bilateral pallidal stimulation is an effective and safe treatment in patients with primary generalized DYT-1 positive dystonia. The effectiveness of pallidal stimulation was proved by the objective validated BFMDRS assessment at 5 years follow-up examination.