Subthalamic Nucleus Deep Brain Stimulation in a Patient with Severe Axial Symptoms and Suboptimal Levodopa Responsive Parkinson's Disease.

AIM: Deep brain stimulation (DBS) is a well-established treatment option for improving function and quality of life (QoL) in carefully selected patients with Parkinson?s disease (PD). Patient selection is a crucial step that should be performed by an experienced multidisciplinary team according to the proposed inclusion and exclusion criteria to increase the QoL of patients. CASE REPORT: A 47-year-old bedridden woman with a 20-year history of PD presented with levodopa-unresponsive tremor and severe axial symptoms. Despite various antiparkinsonian medications, a suboptimal improvement was observed with the levodopa challenge test. After detailed evaluations, she underwent bilateral subthalamic nucleus DBS. During the 2-year follow-up, her axial symptoms improved significantly leading to a better QoL. CONCLUSION: Although levodopa-resistant axial symptoms are considered a relative contraindication to DBS surgery, this case report demonstrates that with an interdisciplinary approach and an accurate assessment of symptoms, even bedridden and latestage selected PD cases may benefit from DBS.

Deep Brain Stimulation of the Globus Pallidus Internus for Secondary Dystonia: Clinical Cases and Systematic Review of the Literature Regarding the Effectiveness of Globus Pallidus Internus versus Subthalamic Nucleus.

OBJECTIVE: Deep brain stimulation (DBS) is a frequently applied therapy in primary dystonia. For secondary dystonia, the effects can be less favorable. We share our long-term findings in 9 patients with severe secondary dystonia and discuss these findings in the light of the literature. METHODS: Patients who had undergone globus pallidus internus (GPi)-DBS for secondary dystonia were included. Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores, clinical improvement rates, follow-up periods, stimulation parameters and the need for internal pulse generator replacements were analyzed. The PubMed and Google Scholar databases were searched for articles describing GPi-DBS and subthalamic nucleus (STN)-DBS only for secondary dystonia cases. Keywords were "dystonia," "deep brain stimulation," "GPi," "dystonia," "deep brain stimulation," and "STN." RESULTS: A total of 9 secondary dystonia patients (5 male, 4 female) had undergone GPi-DBS with microelectrode recording in our units. The mean follow-up period was 29 months. The average BFMDRS score was 58.2 before the surgery, whereas the mean value was 36.5 at the last follow-up of the patients (mean improvement, 39%; minimum, 9%; maximum, 63%). In the literature review, we identified 264 GPi-DBS cases (mean follow-up, 19 months) in 72 different articles about secondary dystonia. The mean BFMDRS improvement rate was 52%. In 146 secondary dystonia cases, reported in 19 articles, STN-DBS was performed. The average follow-up period was 20 months and the improvement in BFMDRS score was 66%. CONCLUSIONS: Although GPi-DBS has favorable long-term efficacy and safety in the treatment of patients with secondary dystonia, STN seems a promising target for stimulation in patients with secondary dystonia. Further studies including a large number of patients, longer follow-up periods, and more homogenous patients are necessary to establish the optimal target for DBS in the management of secondary dystonias.

Effects of Deep Brain Stimulation of the Subthalamic Nucleus on the Postoperative Levodopa Response: One Year Follow Up.

AIM: To investigate the effect of preoperative levodopa responsiveness to clinical outcomes in the first postoperative year, and to evaluate the changes in the postoperative levodopa responsiveness in patients undergoing subthalamic nucleus (STN) deep brain stimulation (DBS). MATERIAL AND METHODS: Forty-nine Parkinson?s Disease (PD) patients undergoing bilateral DBS of the STN were included in this study. Their clinical motor symptoms were assessed preoperatively by UPDRS Part III score in both OFF and ON medication states. Postoperatively, the assessments were obtained in three consecutive conditions. Preoperatively and postoperatively, the percentage difference between these two scores was evaluated as levodopa response. RESULTS: Mean age was 54.6 ± 9 years (27?70). Levodopa response significantly decreased postoperatively by 56% a year. Compared with preoperative med on and postoperative stim on / med on scores, the clinical results of the first year were obtained and an improvement of 25% on the UPDRS 3 score was observed. Compared with preoperative levodopa response and clinical outcomes, better clinical results were obtained in patients with higher preoperative levodopa response (p < 0.05). CONCLUSION: In this study, we confirm that the response of L-dopa decreases after DBS of the STN. The reasons for this finding are not clear. However, DBS of the STN allows for the reduction of PD medications and improvement of daily life activities, motor function, motor fluctuations, and dyskinesia.

Microelectrode Recording for Deep Brain Stimulation of the Subthalamic Nucleus in Patients with Advanced Parkinson's Disease: Advantage or Loss of Time?

AIM: To investigate the effect of using microelectrode recording (MER) on the length of time required to carry out a deep brain stimulation (DBS) procedure of the subthalamic nucleus in patients with Parkinson's disease (PD). MATERIAL AND METHODS: The time required to include MER in the DBS operation was calculated for the first and second sides in 24 patients with PD. The number of microelectrodes used on each trajectory for the first and second sides, and the percentage of permanent electrodes implanted on each trajectory for the first and second sides, were quantified. RESULTS: The average times taken to use MER were 23.4 ± 6.2 minutes, 17.4 ± 6.5 minutes, and 41.2 ± 6.3 minutes for the first side, second side and total procedure, respectively. In 75% of patients, the permanent electrode was implanted at the planned target site for the first side, and in 61% of patients for the second side. CONCLUSION: MER extends the time required to carry out the DBS procedure. However, during surgery, it provides real-time information on the electrodes' neurophysiological locations and helps the surgical team choose an alternative target if the planned target does not produce satisfying results.

Characterizing the Intraoperative Microelectrode Recording-Induced Microlesion Effect on Motor Symptoms in Patients with Parkinson's Disease Undergoing Deep Brain Stimulation of the Subthalamic Nucleus.

AIM: To investigate microelectrode recording (MER)-induced microlesion effect (MLE) on the motor symptoms of 30 patients with Parkinson’s disease (PD) who underwent deep brain stimulation of the subthalamic nucleus. MATERIAL AND METHODS: MER-induced MLE was evaluated based on the difference between tremor, rigidity, and bradykinesia scores in the preoperative off-state and intraoperative state following MER and before test stimulation. RESULTS: MLE scores improved by 21.7% [left (L) side] and by 13.6% [right (R) side] from baseline (p < 0.05). Tremor scores improved by 31.5% (L) and by 14.2% (R) (p < 0.05), rigidity scores improved by 17.3% (L) and by 14.2% (R) (p < 0.05) and bradykinesia scores improved by 20.6% (L) and by 11.5% (R) (p < 0.05) from baseline. There was no significant difference between MLE and the number of microelectrodes used (p > 0.05). CONCLUSION: MER-induced MLE improved motor symptoms and was not correlated with the number of microelectrodes used during the procedure.

Familial Restless Legs Syndrome: A Family with all Female Patients.

BACKGROUND: Restless legs syndrome (RLS) is a chronic condition characterized by odd sensations in the body, most commonly in the legs and an irresistible urge to move them. More than half of the patients with RLS have a family history. Most of the RLS cases are women and most of the families show characteristics of an autosomal dominant pedigree. Here, we shall present a family consisting only of women; to our knowledge, such a family has not been reported yet. METHODS: The family presented here met the diagnosis criteria specified by International Restless Legs Syndrome Study Group (IRLSS). Clinical characteristic are described along with demographic properties. RESULTS: The patients were between 12 and 59 years of age with a mean age of 35.3 ± 14.4 years. All 7 cases were women. The pedigree of the patients exhibited an autosomal dominant inheritance pattern. CONCLUSIONS: The present family tree indicates that familial RLS can exhibit a heredity pattern which shows autosomal dominant inheritance. The present family is still under follow-up. Future research is required to support the present data.

Effect of Age and Disease Duration on the Levodopa Response in Patients with Advanced Parkinson's Disease for Deep Brain Stimulation of the Subthalamic Nucleus.

BACKGROUND: Deep brain stimulation (DBS) has become a preferred option for the treatment of motor symptoms in patients with advanced Parkinson's disease (PD). A good levodopa response (LR) is considered the most important criterion in determining the suitability of a patient for DBS. However, the effect of age and disease duration (DD) on the LR is still a subject of discussion. OBJECTIVE: Here, we investigated the effect of age and DD on the preoperative LR in PD patients to be selected for DBS. METHODS: From August 2011 to May 2015, 54 consecutive patients (29 men and 25 women) with advanced PD were evaluated for DBS of the STN and included in this retrospective study. RESULTS: Thirty-seven patients were found suitable for DBS of the STN and 29 of them underwent bilateral surgery. We found no significant correlation between DD and the LR. However, there was a significant negative correlation between the patients' age and the LR. CONCLUSION: The results indicate that the patients' age, rather than DD, has a negative effect on the LR. The study, therefore, indicates that PD patients with an advanced age and with a poor LR are not good candidates for DBS of the STN.

Why is Alzheimer disease confused with other dementias?

Alzheimer disease (AD) is the most common cause of dementia. The cardinal manifestation of AD is progressive loss of memory. However, there are some nonamnestic presentations of AD, also called atypical AD. Symptoms of AD can sometimes start suddenly. In the presence of atypical symptoms or sudden onset, it may be difficult to distinguish AD from other dementias. We would like to discuss the confusing features of atypical AD that mimic other dementias. In this review, the literature associated with confusing features of AD, suggesting other dementia syndromes, is reviewed. In addition, a case of semantic dementia (SD) with the complaint of forgetfulness previously diagnosed as AD is presented together with clinical and radiological clues of the differential diagnosis of dementia syndromes. As in our representative SD case, a careful clinical history, a detailed mental evaluation, and neuroimaging will overcome this difficulty in diagnosis.

Clinical REM sleep behavior disorder and motor subtypes in Parkinson's disease: a questionnaire-based study.

OBJECTIVES: Studies documenting the association between rapid eye movement sleep behavior disorder (RBD) and motor subtypes in Parkinson's disease (PD) are rare. Our hypothesis is that RBD may be more severe in non-tremor dominant (NTD) patients with RBD than those tremor dominant (TD) with RBD. In this study, we investigated the association between motor subtypes and clinical RBD in PD. PATIENTS AND METHODS: We evaluated 104 consecutive patients older than 18 years presenting with PD to the Neurology Clinic of the University Hospital for one year in this study. The clinical diagnosis of RBD was based on the minimal diagnostic criteria of International Classification of Sleep Disorders, revised. The Stavanger Sleepiness Questionnaire was used to rate the severity of clinical RBD. The patients were divided into two subgroups as TD and NTD. The patient and control groups were compared with each other for severity and frequency of clinical RBD, and the Unified Parkinson's Disease Rating Scale (UPDRS) and Hoehn-Yahr stage scores. The correlation between severity of clinical RBD and clinical severity of PD was analyzed in the patient groups. RESULTS: Of the patients, 45.2% (n=47) had the NTD subtype of PD and 54.8% (n=57) had the TD subtype of PD. There was no significant difference among the groups in terms of frequency and severity of clinical RBD. For the NTD patients, there was a weak positive correlation between severity of clinical RBD and clinical severity of PD. However, there was no correlation in the TD subgroup. CONCLUSION: In our study, frequency of clinical RBD was unrelated to motor subtypes of PD. However, in the present study, we found a weak correlation between clinical severity (UPDRS and the Hoehn-Yahr) of PD and severity of clinical RBD in the NTD subtype but not in the TD subtype.

Volumetric analysis of the subthalamic and red nuclei based on magnetic resonance imaging in patients with Parkinson's disease.

Parkinson's disease (PD) is associated with degeneration of the dopaminergic neurons in the substantia nigra. The subthalamic nucleus (STN) plays a pivotal role in the pathogenesis. However, there is not much known about the morphological changes in the STN. The red nucleus (RN) has many connections with the motor coordinating pathways although it is not primarily involved in the pathogenesis. In this study we aimed to compare the volumes of the STN and RN measured by magnetic resonance imaging in PD patients and controls to investigate how these structures are affected at the morphological level. Twenty patients with PD and twenty age/sex matched controls were enrolled in this study. Severity score was determined by Hoehn & Yahr staging: 6 at stage II and 14 at stage III in med-off state. Imaging was performed by a 1.5 Tesla (T) MR scanner. Measurements of total brain and normalized STN and RN volumes were performed by manual planimetry using Image J software. No statistically significant differences were observed between two groups based on age or gender and disease stage and nuclei volumes. The total estimated brain volumes were not different between PD patients and controls. However, normalized volumes of the STN and RN were 14% and 16% larger, respectively, in PD patients compared to the controls (p < 0.05). Our findings suggest that the volumes of the STN and RN are increased in patients with PD. These changes possibly reflect the altered metabolic activity of these regions demonstrated by neurophysiological studies.

Three-year experience in the Emergency Department: the approach to patients with spinal trauma and their prognosis.

BACKGROUND: Spinal cord injuries result in critical pecuniary and/or non-pecuniary losses due to the developing neurological problems. The objective of this study was to evaluate spinal injuries in terms of clinical severity and prognosis. Spinal injuries lead to serious clinical results due to the high rates of morbidity and mortality; however, there is a lack of reliable information on spinal injuries in our country. METHODS: Following the approval of the Faculty Ethics Committee, this retrospective study was conducted on 91 patients aged ?18 (59 male, 32 female) with spinal trauma who were admitted to the Emergency Department of Ondokuz Mayis University over three years. The patients were assessed in terms of demographics, clinical severity, developing complications, and mortality. RESULTS: Forty-three patients had complete injuries, while 48 had incomplete injuries. Forty-six patients suffered spinal injuries due to fall from height, 35 patients due to traffic accidents, and 10 patients due to other reasons. Several complications were observed in 52 patients, while no complication occurred in 39 patients. We determined that 19 of 92 patients involved in this study died, while 72 were discharged from the hospital. CONCLUSION: Spinal cord injuries generally result in unfavorable clinical results. Therefore, an appropriate approach (early diagnosis and true treatment) in emergency services has great significance.

Does probe's eye subthalamic nucleus length on T2W MRI correspond with microelectrode recording in patients with deep brain stimulation for advanced Parkinson's disease?

AIM: Subthalamic nucleus (STN) deep brain stimulation (DBS) has become a well-accepted treatment for patients with advanced Parkinson's disease (PD). During surgical planning for DBS, the length of the STN is taken into account and verified during microelectrode recording (MER) intraoperatively. Here, we addressed the question to which extent the length of the STN measured with the T2 weighted MRI in the probe's eye view corresponded with the intraoperatively determined length of the STN with MER. MATERIAL AND METHODS: We included 10 consecutive Parkinson's disease patients who underwent STN DBS surgery. The length of the STN in the probe's eye view mode was calculated along the trajectory of the central MER electrode crossing the STN. RESULTS: Our analysis showed no statistical difference between the length of the STN measured with the T2 weighted probe's eye view mode and the MER (right STN length 5.8 ± 0.9 mm MRI vs. 6.3 ± 0.5 mm MER, p > 0.05; left STN length 5.6 ± 0.4 mm MRI vs 5.8 ± 1 mm MER, p > 0.05). CONCLUSION: This means that the entry and the exit of the STN can be adequately estimated using the probe's eye view preoperatively.

Conversion of local anesthesia-guided deep brain stimulation of the subthalamic nucleus to general anesthesia.

BACKGROUND: Deep brain stimulation of the subthalamic nucleus (STN) is a widely applied procedure in the treatment of patients with advanced Parkinson disease and is generally performed under local anesthesia. Here we report our experience with the conversion to general anesthesia in two patients with advanced Parkinson disease because of fear reactions intraoperatively. CASE DESCRIPTION: Patients received general anesthesia with propofol and were implanted with electrodes at the level of STN guided by multiple-microelectrode electrophysiological recordings after obtaining informed consent. During the recordings the propofol levels were reduced. Postoperative clinical assessments showed marked improvements of motor disability with significant reductions of dopaminergic medication. CONCLUSION: Our case observations document the possibility of fear reactions intraoperatively and show the possibility of conversion to general anesthesia with a successful outcome.

Isolated unilateral vagus nerve palsy secondary to trauma.

A 41-year-old man presented to emergency service with loss of consciousness lasting 20 minutes after a piece of wood struck the right side of his face. Shortly after admission, he developed difficulty swallowing. On admission, he was alert and had normal vital findings. There was no motor, sensorial, or cerebellar deficit. Deviation of the uvula to the left side and pharyngeal reflex loss on the right side was obvious. The right vocal cord was paralyzed. Other cranial nerves were intact on examination. The patient's cranial computed tomography (CT), CT-angiogram, cranial and neck magnetic resonance (MR) imaging, MR-angiogram, and cervical and lung X-ray were normal. We evaluated this case with isolated unilateral vagus nerve palsy (VNP) secondary to trauma at the emergency department. Our case illustrated that trauma can cause isolated VNP with the absence of abnormal findings on imaging modalities.

Syncope associated with subthalamic nucleus deep brain stimulation in a patient with Parkinson's disease.

In advanced Parkinson's disease (PD), deep brain stimulation (DBS) may be an alternative option for the treatment of motor symptoms. Side effects associated with subthalamic nucleus (STN) DBS in patients with PD are emerging as the most frequent sensory and motor symptoms. DBS-related syncope is reported as extremely rare. We wanted to discuss the mechanisms of syncope associated with STN DBS in a patient with Parkinson's disease. Case report. Sixty-three-year-old female patient is followed up with diagnosis of idiopathic Parkinson's disease for 6 years in our clinic. The patient has undergone STN DBS due to painful dystonia and drug resistant tremor. During the operation, when the left STN was stimulated at 5 milliampere (mAmp), the patient developed presyncopal symptoms. However, when the stimulation was stopped symptoms improved. During the early period after the operation, when the right STN was stimulated at 1.3 millivolts (mV), she developed the pre-yncopal symptoms and then syncope. Our case shows that STN DBS may lead to directly autonomic symptoms resulting in syncope during stimulation-on (stim-on).

Evaluation of patients with delirium in the emergency department.

OBJECTIVE: Delirium is a neuropsychiatric syndrome characterized by acute onset and a fluctuating course of globally altered mental status. It has been reported that the frequency of delirium among patients hospitalized with any disorder ranges from 2 to 30%. However, in the literature, few studies have evaluated the frequency of delirium in hospitalized patients, including those older than 65 years. According to our knowledge, this is the first study on adult patients in all age groups in an emergency department. We aimed to classify delirium according to its etiological causes and to compare the age groups for frequency and these causes. MATERIAL AND METHODS: Forty-three patients were included in the study; they had been diagnosed with delirium according to the Diagnostic and Statistical Manual of Mental Disorders (DSM)-IV in an emergency department from December 2002 to December 2003. The patients were divided into two groups (group I: age <65 years, group II: age ≥65 years). Following a detailed history, each patient underwent a physical and neurological examination. Additionally, the Mini-Mental State Examination was administered. Radiological and hematological examinations were conducted on all patients who had indications for such testing. RESULTS: Of the patients with delirium, 18 were female and 25 were male. Mean age was 61.18 years (range 18 to 90 years). Of the 43 patients, 21 were in group I and 22 were in group II. The most common causes of delirium were metabolic disorders. There was no significant difference between group I and II for the frequency or etiological causes of delirium. CONCLUSION: In emergency departments, all patients with delirium should be evaluated for etiological factors, especially metabolic disorders, as treatment of the underlying cause is essential in delirium.

The myopathic effects of electrical injury.

BACKGROUND: In this study, we investigated the effect of voltage levels on muscle damage in patients with electrical injuries. METHODS: This retrospective study included 36 patients with electrical injury (high voltage, 21; low voltage, 15). Initial serum creatine kinase (CK), aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were obtained from each patient on admission. RESULTS: Although CK levels were observed to be higher in the high-voltage injured patients than in those exposed to a low voltage, the difference was not statistically significant. The serum AST and ALT levels were elevated in both groups, but there was no significant difference between the groups. CONCLUSION: Skeletal muscle damage can be caused by both high-voltage and low-voltage electrical injury, and in these patients, the degree of muscle damage may be consistent with the elevated initial serum levels of muscle enzymes, especially of CK. However, further researches are necessary to determine if there is strong evidence of a direct correlation between voltage level and the degree of muscle damage.

Wild mushroom poisonings in the Middle Black Sea region in Turkey: analyses of 6 years.

Wild mushroom poisoning (MP) is an important medical emergency that may have serious clinical outcome. The aim of this study was to evaluate the demographic and clinical features of patients with wild MP. This study was designed retrospectively by examining files of the patients with wild MP who were admitted to Ondokuz Mayis University Emergency Department, between January 2002 and December 2007. Patients > or =16 years of age were included in the study. A total of 317 patients poisoned by wild mushrooms (mean age, 42.0 +/- 16.3 years; 67.5% female) were studied. All poisonings were accidental, i.e. consumption of wild mushrooms collected from open fields and woodlands or purchased from local bazaars. The common symptoms and complaints on admission were nausea (86.8%) and vomiting (79.8%). The poisoning latent phase in most cases was <6 hours (86.8%). Most of the poisonings occurred in autumn (59.6%). Three patients died in the hospital due to acute liver failure and complications. The duration of hospitalization was a median 3 days (range: 1-12 days). The public should be informed about the probable hazards of wild mushroom ingestion.

Elevated serum hyaluronic acid and interleukin-6 levels in patients with mushroom poisoning admitted to the emergency department.

OBJECTIVE: To assess the possible use of hyaluronic acid (HA) and interleukin-6 (IL-6) together as a biochemical marker of liver damage in mushroom poisoning (MP). METHODS: We prospectively studied patients with MP who were admitted to the emergency service, between April 2005 and April 2007, Samsun, Turkey. Twenty-seven patients with MP were included in the study. Serum HA and IL-6 levels of the patients were determined using enzyme-linked immunosorbent assay daily for a total of 3 days. Ten healthy adults were included in the study to serve as controls. The patients were divided into survivors, and non-survivors. RESULTS: There was no significant difference between the patients and controls with respect to serum HA levels on admission (p>0.05). However, IL-6 levels on admission were significantly higher in the patients than the control group (p<0.01). Serum HA and IL-6 levels on admission, and the following days were significantly higher in non-surviving patients (n=5) than in surviving patients (n=22) (p<0.05). There was a significant correlation between HA and IL-6 (r=0.42, p<0.05) on admission. The HA concentration was also significantly correlated with aspartate aminotransferase, alanine aminotransferase, and creatinine levels during the observation period. Serum HA and IL-6 levels increased in non-surviving patients throughout the period of observation. CONCLUSION: Increased serum HA and IL-6 levels are associated with hepatic damage in acute MP. Hyaluronic acid may be a useful marker in the assessment of MP-induced acute liver failure in clinical practice.