Exploring continuity of care for women with prenatal diagnosis of congenital anomaly: A mixed method study.

AIMS: To map the pathway and service provision for pregnant women whose newborns require admission into the surgical neonatal intensive care unit at or soon after birth, and to examine the nature of continuity of care (COC) provided and the facilitators and barriers to woman- and family-centred care from the perspective of women/parents and health professionals. BACKGROUND: Limited research exists on current service and care pathways for families whose babies are diagnosed with congenital abnormality requiring surgery. DESIGN: A mixed method sequential design adhering to EQUATOR guidelines for Good Reporting of a Mixed Methods Study. METHODS: Data collection methods included: (1) a workshop with health professionals (n = 15), (2) retrospective maternal record review (n = 20), prospective maternal record review (17), (3) interviews with pregnant women given a prenatal diagnosis of congenital anomaly (n = 17) and (4) interviews with key health professionals (n = 7). RESULTS/FINDINGS: Participants perceived care delivered by state-based services as problematic prior to admission into the high-risk midwifery COC model. Once admitted to the high-risk maternity team women described care 'like a breath of fresh air' with a 'contrast in support', where they felt supported in their decisions. CONCLUSION: This study highlights provision of COC, in particular relational continuity between health providers and women as essential to achieve optimal outcomes. RELEVANCE TO CLINICAL PRACTICE: Provision of individualised COC offers an opportunity for perinatal services to reduce the negative consequences of pregnancy-related stress associated with diagnosis of foetal anomaly. PATIENT OR PUBLIC CONTRIBUTION: No patient or public was involved in the design, analysis, preparation or writing of this review.

Pregnancy complicated by refractory severe hypercholanaemia from sodium taurocholate co-transporting polypeptide deficiency.

Sodium taurocholate co-transporting polypeptide deficiency is a rare metabolic autosomal recessive condition resulting in critically elevated plasma bile acid levels. Hypercholanaemia in similar conditions such as intrahepatic cholestasis of pregnancy has been associated with an increased risk of adverse obstetric outcomes including stillbirth. We present the first case of Sodium taurocholate co-transporting polypeptide deficiency in a current pregnancy in a patient with one previous stillbirth in the context of severe hypercholanaemia, where conventional treatments for cholestasis including ursodeoxycholic acid, rifampicin and cholestyramine were ineffective. Therapeutic plasma exchange and novel treatment with elobixibat were trialed with mixed results. The pregnancy resulted in an iatrogenic preterm delivery of a live infant at 32 weeks gestation.

Remote telemedicine evaluation of deep brain stimulation candidacy: Retrospective cohort analysis.

OBJECTIVE: To determine whether initial presurgical evaluation of deep brain stimulation (DBS) candidacy with video telemedicine (VTEL) can reliably predict surgical candidacy (patients who will eventually undergo DBS surgery) and decrease resource utilization when compared to an in-person evaluation. METHODS: In this retrospective, cohort analysis, all out-of-state referrals to the San Francisco Veterans Affairs from 2008 to 2013 for DBS therapy were reviewed and their surgical outcomes were assessed until 2017. Patients were designated as good, borderline, or poor surgical candidates after initial evaluation, and their rates of undergoing DBS were recorded. An assessment of patient travel costs was performed. RESULTS: There were 60 out-of-state DBS referrals identified out of the 148 initial presurgical DBS evaluations completed for surgical treatment of dystonia, essential tremor, or Parkinson disease; 24 patients underwent in-person consultation and 36 patients underwent evaluation via VTEL. There was no difference between the rates of undergoing surgical treatment with DBS based on surgical candidacy for patients in the in-person and VTEL cohorts. Patients who underwent initial presurgical screening via VTEL saved time and money. CONCLUSIONS: VTEL can be used to facilitate presurgical screening for DBS and saves costs.

Phase I trial of caudate deep brain stimulation for treatment-resistant tinnitus.

OBJECTIVE: The objective of this open-label, nonrandomized trial was to evaluate the efficacy and safety of bilateral caudate nucleus deep brain stimulation (DBS) for treatment-resistant tinnitus. METHODS: Six participants underwent DBS electrode implantation. One participant was removed from the study for suicidality unrelated to brain stimulation. Participants underwent a stimulation optimization period that ranged from 5 to 13 months, during which the most promising stimulation parameters for tinnitus reduction for each individual were determined. These individual optimal stimulation parameters were then used during 24 weeks of continuous caudate stimulation to reach the endpoint. The primary outcome for efficacy was the Tinnitus Functional Index (TFI), and executive function (EF) safety was a composite z-score from multiple neuropsychological tests (EF score). The secondary outcome for efficacy was the Tinnitus Handicap Inventory (THI); for neuropsychiatric safety it was the Frontal Systems Behavior Scale (FrSBe), and for hearing safety it was pure tone audiometry at 0.5, 1, 2, 3, 4, and 6 kHz and word recognition score (WRS). Other monitored outcomes included surgery- and device-related adverse events (AEs). Five participants provided full analyzable data sets. Primary and secondary outcomes were based on differences in measurements between baseline and endpoint. RESULTS: The treatment effect size of caudate DBS for tinnitus was assessed by TFI [mean (SE), 23.3 (12.4)] and THI [30.8 (10.4)] scores, both of which were statistically significant (Wilcoxon signed-rank test, 1-tailed; alpha = 0.05). Based on clinically significant treatment response categorical analysis, there were 3 responders determined by TFI (≥ 13-point decrease) and 4 by THI (≥ 20-point decrease) scores. Safety outcomes according to EF score, FrSBe, audiometric thresholds, and WRS showed no significant change with continuous caudate stimulation. Surgery-related and device-related AEs were expected, transient, and reversible. There was only one serious AE, a suicide attempt unrelated to caudate neuromodulation in a participant in whom stimulation was in the off mode for 2 months prior to the event. CONCLUSIONS: Bilateral caudate nucleus neuromodulation by DBS for severe, refractory tinnitus in this phase I trial showed very encouraging results. Primary and secondary outcomes revealed a highly variable treatment effect size and 60%-80% treatment response rate for clinically significant benefit, and no safety concerns. The design of a phase II trial may benefit from targeting refinement for final DBS lead placement to decrease the duration of the stimulation optimization period and to increase treatment effect size uniformity.Clinical trial registration no.: NCT01988688 (clinicaltrials.gov).

Human caudate nucleus subdivisions in tinnitus modulation.

OBJECTIVE: The object of this study was to define caudate nucleus locations responsive to intraoperative direct electrical stimulation for tinnitus loudness modulation and relate those locations to functional connectivity maps between caudate nucleus subdivisions and auditory cortex. METHODS: Six awake study participants who underwent bilateral deep brain stimulation (DBS) electrode placement in the caudate nucleus as part of a phase I clinical trial were analyzed for tinnitus modulation in response to acute stimulation at 20 locations. Resting-state 3-T functional MRI (fMRI) was used to compare connectivity strength between centroids of tinnitus loudness-reducing or loudness-nonreducing caudate locations and the auditory cortex in the 6 DBS phase I trial participants and 14 other neuroimaging participants with a Tinnitus Functional Index > 50. RESULTS: Acute tinnitus loudness reduction was observed at 5 caudate locations, 4 positioned at the body and 1 at the head of the caudate nucleus in normalized Montreal Neurological Institute space. The remaining 15 electrical stimulation interrogations of the caudate head failed to reduce tinnitus loudness. Compared to the caudate head, the body subdivision had stronger functional connectivity to the auditory cortex on fMRI (p < 0.05). CONCLUSIONS: Acute tinnitus loudness reduction was more readily achieved by electrical stimulation of the caudate nucleus body. Compared to the caudate head, the caudate body has stronger functional connectivity to the auditory cortex. These first-in-human findings provide insight into the functional anatomy of caudate nucleus subdivisions and may inform future target selection in a basal ganglia-centric neuromodulation approach to treat medically refractory tinnitus.Clinical trial registration no.: NCT01988688 (clinicaltrials.gov).

Long-Term Outcomes of Bilateral Pallidal Deep Brain Stimulation for X-Linked Dystonia and Parkinsonism.

BACKGROUND: X-linked dystonia parkinsonism (XDP) causes adult-onset progressive dystonia and parkinsonism, which may not respond to pharmacotherapy. OBJECTIVE: Previous case reports have reported beneficial effects from bilateral pallidal (GPi) deep brain stimulation (DBS). Here, we report the long-term clinical outcomes of 3 patients treated at our center. METHODS: All patients presented with medication refractory dystonia and parkinsonism. They were followed prospectively. Clinical evaluations included the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and the Unified Parkinson's Disease Rating Scale (UPDRS). Adverse events were recorded. RESULTS: The average length of follow-up was 45.7 months. No serious adverse events occurred. All patients experienced an immediate and sustained improvement in dystonia. Mean percentage improvement in motor subscores of BFMDRS was 63.5% at the last follow-up visit. Parkinsonism was less responsive to neuromodulation, with a mean improvement in UPDRS-III of 39.5%. Standard pallidal stimulation parameters were used. Freezing of gait developed after DBS therapy in 2 patients, stimulation-induced in one and due to disease progression in the other. CONCLUSION: Bilateral pallidal DBS resulted in significant and sustained improvement in dystonia and moderate improvement in parkinsonism. Pallidal DBS represents an important treatment option for XPD for the management of motor symptoms.

Subthalamic nucleus deep brain stimulation in isolated dystonia: A 3-year follow-up study.

OBJECTIVE: To report long-term safety and efficacy outcomes of a large cohort of patients with medically refractory isolated dystonia treated with subthalamic nucleus (STN) deep brain stimulation (DBS). METHODS: Twenty patients (12 male, 8 female; mean age 49 ± 16.3 years) with medically refractory isolated dystonia were studied (14 were followed for 36 months). The primary endpoints were change in Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor score and Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) total score at 36 months compared to preoperative baseline. Multiple secondary outcomes were also assessed (ClinicalTrials.gov NCT00773604). RESULTS: Eighteen of 20 patients showed improvement 12 months after STN DBS with sustained benefit persisting for 3 years (n = 14). At 36 months, BFMDRS motor scores improved 70.4% from a mean 17.9 ± 8.5 to 5.3 ± 5.6 (p = 0.0002) and total TWSTRS scores improved 66.6% from a mean 41.0 ± 18.9 to 13.7 ± 17.9 (p = 0.0002). Improvement at 36 months was equivalent to that seen at 6 months. Disability and quality of life measures were also improved. Three hardware-related and 24 stimulation-related nonserious adverse events occurred between years 1 and 3 (including 4 patients with dyskinesia). CONCLUSIONS: This study offers support for long-term tolerability and sustained effectiveness of STN DBS in the treatment of severe forms of isolated dystonia. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that STN DBS decreases long-term dystonia severity in patients with medically refractory isolated dystonia.

Patient and Caregiver Perspectives of Preoperative Teaching for Deep Brain Stimulation Surgery.

Deep brain stimulation (DBS) has developed into an important therapy for Parkinson disease, essential tremor, and dystonia with more nurses in varied settings often preparing patients and families for this type of surgery. This exploratory study sought to obtain patient and caregiver perspectives of the current DBS teaching for Parkinson disease, essential tremor, and dystonia; to improve the teaching; and to standardize the education. Using survey methodology, 41 patients with movement disorder and 32 caregivers completed surveys about the preoperative instructions they received. Data analysis calculated frequencies for response rate, demographic information, multiple-choice questions, and Likert scale responses. Fill-in questions were summarized. Results overall showed that, because of the teaching, two thirds of patients and nearly two thirds of caregivers felt fully prepared for the DBS surgery. Patients' and caregivers' suggested recommendations for nurses and surgeons included requests for specific information such as attention to delivery of the education, more individualized care during the education, attention to pain during and after procedure, and postdischarge follow-up. The study identified unmet patient and caregiver needs, resulted in changes in practice, and serves as a guide toward standardization of educational approach and/or content.

Thalamotomy-Like Effects From Partial Removal of a Ventral Intermediate Nucleus Deep Brain Stimulator Lead in a Patient With Essential Tremor: Case Report.

BACKGROUND AND IMPORTANCE: The ventral intermediate nucleus of the thalamus is a primary target of deep brain stimulation (DBS) in patients with essential tremor. Despite reliable control of contralateral tremor, there is sometimes a need for lead revision in cases of infection, hardware malfunction, or failure to relieve symptoms. Here, we present the case of a patient undergoing revision after ventral intermediate nucleus (Vim) DBS failed to control his tremor. During the electrode removal, the distal portion of the lead was found to be tightly adherent to tissue within the deep brain. Partial removal of the electrode in turn caused weakness, paresthesias, and tremor control similar to the effects produced by thalamotomy or thalamic injury. CLINICAL PRESENTATION: A 48-year-old man with essential tremor had bilateral Vim DBS leads implanted 10 years earlier but had poor control of his tremor and ultimately opted for surgical revision with lead placement in the zona incerta. During attempted removal of his right lead, the patient became somnolent with contralateral weakness and paresthesias. The procedure was aborted, and postoperative neuroimaging was immediately obtained, showing no signs of stroke or hemorrhage. The patient had almost complete control of his left arm tremor postoperatively, and his weakness soon resolved. CONCLUSION: To the best of our knowledge, this is the first reported case of cerebral injury after DBS revision and offers insights into the mechanism of high-frequency electric stimulation compared with lesions. That is, although high-frequency stimulation failed to control this patient's tremor, thalamotomy-like injury was completely effective.

How clinical nurse and midwifery consultants optimise patient care in a tertiary referral hospital.

AIMS AND OBJECTIVES: To develop new insights into the phenomenon of clinical nurse/midwifery consultant clinical effectiveness in a tertiary referral hospital. BACKGROUND: International studies identify the pivotal role clinical nurse/midwifery consultants play in patient outcomes. There remains, however, a significant deficit in our knowledge of how these, or other advanced practice nurses and midwives, apply their extensive experience, ontological understandings and tacit knowledge to the enhancement of patient outcomes in complex healthcare environments. DESIGN: This study was underpinned by the principles of Heideggerian hermeneutic phenomenology. METHODS: Recruitment involved expressions of interest distributed hospital-wide to clinical nurse/midwifery consultants. Inclusion criteria specified a minimum of three years' experience. Fifteen clinical nurse/midwifery consultants, representing a broad range of specialties, were interviewed. Preliminary descriptive analysis of transcribed data was followed by in-depth hermeneutic analysis. RESULTS: The findings comprise four themes: walking beside the patient, anticipating the unexpected, pushing through barriers and leading within a complex system. These themes highlight how clinical nurse/midwifery consultants significantly influence pre-existing trajectories of patient care delivery through context-appropriate strategies. CONCLUSION: This study introduces new insights into the meanings advanced practice nurses and midwives develop from their engagements with patients and how these meanings inform clinical decisions. The paper accomplishes this by drawing on Heideggerian philosophical concepts such as ontological understandings, authenticity and care. It also casts light on the participants' shared understandings of how to synergise expectations within the team. RELEVANCE TO CLINICAL PRACTICE: The significance of this paper lies in the uncovering of ontological understandings and tacit knowledge of how consultant nurses and midwives optimise patient care delivery in challenging situations. The findings indicate, however, that the value of these internationally established roles is yet to be fully realised. The paper concludes by recommending strategies to assist newly appointed clinical nurse/midwifery consultants transition into these demanding autonomous roles.

Effect of frequency on subthalamic nucleus deep brain stimulation in primary dystonia.

BACKGROUND: Subthalamic nucleus deep brain stimulation (DBS) is an alternative target choice for treating primary dystonia, but little is known about the most effective programming parameters. OBJECTIVE: Here we prospectively evaluate the effect of low versus high frequency subthalamic nucleus DBS in patients with predominantly cervical or upper extremity primary dystonia. METHODS: Seven patients were stimulated at low frequency stimulation (60 Hz) for the first three months and then switched to high frequency stimulation (130 Hz) until month six. Severity of dystonia was determined by a blinded rater (unaware of the patient's pre or post-operative status) who scored the Burke Fahn Marsden dystonia rating scale movement score (BFMDRS-M) and the Toronto Western Spasmodic Torticollis Rating Scale severity score (TWSTRS-S) preoperatively, three, six, and twelve months post-surgery. RESULTS: Patients had a lower mean improvement of 16.6% in BFMDRS-M and 9.5% in TWSTRS-S at three months using low frequency stimulation compared to a 52.3% (p = 0.018) and 45.2% (p = 0.028), respectively, noted at six months using high frequency stimulation. At 12 months (using 130 Hz), the BFMDRS-M and TWSTRS-S improved by 51.8% (p = 0.022) and 56% (p = 0.034). Patients developed transient dyskinesia (during low and high frequency stimulation) which improved with programming adjustments. CONCLUSION: This study offers further support of the effectiveness of subthalamic nucleus DBS in the treatment of primary dystonia and finds that high frequency stimulation was more effective than low frequency stimulation.

Pharmacist's role in a Parkinson's disease and movement disorders clinic.

PURPOSE: The expanding role of a clinical pharmacist at a Veterans Affairs (VA) out-patient clinic for patients with Parkinson's disease (PD) and movement disorders is described. SUMMARY: San Francisco VA Medical Center added a clinical pharmacist to the multi-disciplinary team serving patients at an outpatient clinic operated by its Parkinson's Disease Research, Education and Clinical Center (PADRECC). During the first six months after joining the clinic team, the pharmacist met with 131 patients and made a total of 69 drug therapy recommendations that were implemented by neurologists, clinical nurse specialists, and other PADRECC providers. The results of a retrospective chart review suggested that in about 21% of the cases evaluated, the pharmacist's recommendations contributed to an improved medical outcome or the resolution of a medical problem. Anonymous surveys indicated that clinic providers (n = 33) and patients (n = 20) were satisfied with the pharmacist's services. Using a five-point Likert scale (scores ranged from 1 for "strongly disagree" to 5 for "strongly agree") that they had more time to devote to other clinic responsibilities with the pharmacist present in the clinic (mean score, 4.79); patients indicated that they had an improved understanding of their medications after speaking with the pharmacist (mean score, 4.88). CONCLUSION: A clinical pharmacist's regular involvement in an outpatient PD and movement disorders clinic has been well received by patients and clinic providers. The study results suggest that the pharmacist has made important contributions in areas such as therapeutic problem solving and medication education while freeing up providers for other responsibilities.

Long-term follow-up of pallidal deep brain stimulation in two cases of Huntington's disease.

BACKGROUND: Deep brain stimulator (DBS) implantation has been shown to be effective in the treatment of various movement disorders including Parkinson's disease, essential tremor and dystonia. However, there is limited information regarding the potential use of DBS in Huntington's disease (HD). In this study, the authors present their findings on the long-term motor and neurocognitive results of two HD patients (patient 1: 57 years, 42 cytosine-adenine guanine (CAG) repeats; patient 2: 50 years, 41 CAG repeats) who underwent staged bilateral globus pallidus interna DBS surgery. METHODS: The patients were evaluated at baseline and at five timepoints throughout a 2-year postoperative during which motoric ratings ((Unified Huntington's Disease Rating Scale), Activities of Daily Living scores (HD-ADL) and neurocognitive testing) were obtained. RESULTS: Both patients had a sustained decline in chorea 2 years after initial DBS surgery. Despite this improvement in chorea, one patient has had continuing deterioration in gait, bradykinesia and dystonia scores, which has caused his ability to perform activities of daily living to return to his baseline level of functioning prior to DBS surgery. Both patients have experienced further gradual decline in neurocognitive functioning, which appears to be independent of DBS and most likely related to disease progression. CONCLUSION: DBS implantation may be a potential treatment option for a subset of HD patients who have significant functional deficits due to chorea. However, appropriate selection of the best candidates for DBS appears to be challenging, given the difficulty in predicting disease course in HD due to its variable nature.

Governing peripherally inserted central venous catheters by combining continuous performance improvement and computerized physician order entry.

OBJECTIVE: Seattle Children's in Seattle, Washington sought to establish governance over peripherally inserted central catheters. Preventing overuse and creating an efficient placement process were of paramount importance. METHODOLOGY: We describe a process by which the marriage of continuous performance-improvement projects and computerized physician order entry has led to a reproducible reduction in peripherally inserted central catheter volumes and an increase in overall provider satisfaction with the ordering process. This was accomplished by increasing daily awareness of central venous catheters, establishing peripherally inserted central catheter-specific insertion criteria, establishing a governing vascular-access service, and creation of a peripherally inserted central catheter-specific computerized order set. RESULTS: After implementation of these measures, peripherally inserted central catheter insertion volumes decreased by 33.4%; these results have been sustained over a period of 19 months. From August 2006 to October 2006, 48% of peripherally inserted central catheters were placed on the same calendar day of order entry, 37% within 24 hours of order entry, and 15% within 48 to 72 hours. Overall, provider satisfaction with the ordering process improved according to a Likert scale. Scores increased from 2.68 of 5 to 3.55 of 5 over a 9-month period. This result was statistically significant at the 95th percentile level according to the t-test method. CONCLUSIONS: We conclude that properly constructed computerized order sets can be effective in altering physician ordering practices through standardization.

Fluoroscopic, EMG-guided injection of botulinum toxin into the longus colli for the treatment of anterocollis.

BACKGROUND: Anterocollis is a form of cervical dystonia characterized by forward neck flexion. While botulinum toxin is the treatment of choice for cervical dystonia, patients with anterocollis, who receive injections into the sternocleidomastoid and anterior scalene muscles, represent a disproportionate number of treatment failures. Deep cervical muscles such as the longus colli likely play an important role in neck flexion but are not routinely injected. OBJECTIVE: To describe a technique for longus colli injection in cases of anterocollis and to report the clinical outcomes of 10 such injections of botulinum toxin. METHODS: Three patients were referred for evaluation and treatment of anterocollis. All had previous treatment failures with sternocleidomastoid/anterior scalene injections or no activity noted on needle EMG investigation of these muscles. All patients received injections of botulinum toxin into the longus colli under fluoroscopic and EMG guidance. RESULTS: All patients experienced symptomatic improvement (eight of 10 injections). Two patients reported mild dysphagia without serious complications after dose increases in botulinum toxin. CONCLUSIONS: Incomplete muscle selection may be one cause of treatment failures in anterocollis. Deep cervical flexors such as the longus colli represent an under-recognized potential target for symptomatic treatment of anterocollis.

Thalamic deep brain stimulation for essential tremor: relation of lead location to outcome.

OBJECTIVE: Thalamic deep brain stimulation (DBS) is commonly used to treat essential tremor, but the optimal lead location within the thalamus has not been systematically evaluated. We examined the relation of lead location to clinical outcome in a series of essential tremor patients treated by thalamic DBS. METHODS: Fifty-seven leads in 37 patients were studied. Lead locations were measured by postoperative magnetic resonance imaging. Contralateral arm tremor was assessed in the DBS-on and DBS-off states using the Fahn-Tolosa-Marin tremor rating scale, with a mean follow-up of 26 months. Lead locations were statistically correlated, using analysis of variance, with percent improvement in tremor resulting from DBS activation. RESULTS: Improvement in tremor score was significantly correlated with lead location in both the anteroposterior and lateral dimensions. In the plane of the commissures, the optimal electrode location was determined statistically to be 6.3 mm anterior to the posterior commissure and 12.3 mm lateral to the midline, or 10.0 mm lateral to the third ventricle. CONCLUSION: Optimal electrode location for thalamic DBS in essential tremor corresponds to the anterior margin of the ventralis intermedius nucleus. Leads located greater than 2 mm (in the plane of the commissures) from the optimal coordinates are more likely to be associated with poor tremor control than leads within 2 mm of the optimal location. The incidence of true physiological tolerance to the antitremor effect of thalamic DBS (defined as poor tremor control in spite of lead location within 2 mm of the optimal site) was found to be 9%.

Pallidal neuronal discharge in Huntington's disease: support for selective loss of striatal cells originating the indirect pathway.

Chorea is the predominant motor manifestation in the early symptomatic phase of adult onset Huntington's disease (HD). Pathologically, this stage is marked by differential loss of striatal neurons contributing to the indirect pathway. This pattern of neuronal loss predicts decreased neuronal firing rates in GPi and increased firing rates in GPe, the opposite of the changes in firing rate known to occur in Parkinson's disease (PD). We present single-unit discharge characteristics (33 neurons) observed in an awake patient with HD (41 CAG repeats) undergoing microelectrode guided surgery for pallidal deep brain stimulation. Pallidal single-unit activity at "rest" and during voluntary movement was discriminated off line by principal component analysis and evaluated with respect to discharge rate, bursting, and oscillatory activity in the 0-200 Hz range. 24 GPi and 9 GPe units were studied, and compared with 132 GPi and 50 GPe units from 14 patients with PD. The mean (+/-SEM) spontaneous discharge rate for HD was 58+/-4 for GPi and 73+/-5 for GPe. This contrasted with discharge rates in PD of 95+/-2 for GPi and 57+/-3 for GPe. HD GPi units showed more bursting than PD GPi units but much less oscillatory activity in the 2-35 Hz frequency range at rest. These findings are consistent with selective early loss of striatal cells originating the indirect pathway.

The use of canines in the detection of human cancers.

OBJECTIVES: To determine whether canines could be trained to identify patients with cancer by sniffing the urine obtained from a patient with breast or prostate cancer from among samples obtained from healthy volunteers. DESIGN: Dogs of different breeds were trained by their owners to detect the urine sample from a patient with cancer from among 6 other age- and sex-matched healthy volunteers. After the training was completed, using new samples, 2 test runs were used for each patient with breast cancer and three runs for the patients with prostate cancer against the same matched samples. The configuration of the samples was different for each run. A total of 18 and 33 runs were carried out, respectively. RESULTS: For each cohort, specificity and sensitivity were measured. In the breast cancer tests, of 6 dogs, only 2 performed better than chance in specificity and none were more sensitive than chance. For the prostate sample testing, 4 dogs were used. Two performed significantly better than chance in specificity and none in sensitivity. CONCLUSIONS: Although this study did not produce the outcomes desired, the literature supports a potential to use canines for human cancer detection. Better management of urine samples and a more stringent training protocol during our study may have provided new evidence as to the feasibility of using canines for cancer detection. A comparison of the 3 dog cancer scenting studies is also presented.

Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome).

Idiopathic cranial-cervical dystonia (ICCD) is an adult-onset dystonia syndrome affecting orbicularis oculi, facial, oromandibular, and cervical musculature. ICCD is frequently difficult to treat medically. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is a highly effective treatment for idiopathic generalized dystonia, however less is known about the effect of GPi DBS on ICCD. In this article, we present the results from a pilot study assessing the effect of GPi DBS in a series of patients with ICCD. Six patients underwent bilateral stereotactic implantation of DBS leads into the sensorimotor GPi. Patients were evaluated with the Burke-Fahn-Marsden dystonia rating scale (BFMDRS) and Toronto western spamodic torticollis rating scale (TWSTRS) before surgery and 6 months postoperatively. At 6 months, patients showed a 72% mean improvement in the BFMDRS total movement score (P < 0.028, Wilcoxin signed rank test). The mean BFMDRS disability score showed a trend toward improvement (P < 0.06). The total TWSTRS score improved 54% (P < 0.043). Despite improvement in dystonia, mild worsening of motor function was reported in previously nondystonic body regions with stimulation in 4 patients. Although GPi DBS was effective in these patients, the influence of GPi DBS on nondystonic body regions deserves further investigation.

Multiple target deep brain stimulation for multiple sclerosis related and poststroke Holmes' tremor.

BACKGROUND: The results from thalamic deep brain stimulation (DBS) for atypical tremor syndromes including tremor from multiple sclerosis (MS) and stroke are often disappointing. Three recent case reports have suggested that simultaneous stimulation of multiple thalamic targets can result in sustained improvement in such cases. METHODS: We analyzed the effectiveness of multiple target DBS in one patient with MS-related tremor and another with poststroke Holmes' tremor. RESULTS: In the patient with MS tremor, we implanted bilateral ventralis intermedius (V.im.) and ventralis oralis anterior (V.o.a.) thalamic electrodes; this patient had significant tremor improvement with stimulation of either V.im. or V.o.a. targets; however, we did not observe additive effects with simultaneous stimulation. In our patient with a poststroke Holmes' tremor, we implanted DBS electrodes in unilateral V.im., V.o.a., and the globus pallidus internus (Gpi); this patient had moderate tremor reduction with Gpi stimulation alone; neither V.im. nor V.o.a. stimulation provided additional benefit. CONCLUSION: In one patient with MS tremor, simultaneous V.im. and V.o.a. stimulation was not superior to V.im. or V.o.a. stimulation alone. In one case of Holmes' tremor, Gpi stimulation was a useful alternative to thalamic stimulation.