Balance Difficulties Research Articles

Improvement in symptom severity, processing speed, gait, movement, and visual acuity in a patient with refractory functional neurological/ conversion disorder

Event Abstract Back to Event Improvement in symptom severity, processing speed, gait, movement, and visual acuity in a patient with refractory functional neurological/ conversion disorder Matthew M. Antonucci1, 2, 3* and Paul E. Link2, 3 1 Carrick Institute, Clinical Neuroscience, United States 2 Plasticity Brain Centers, Neurorehabilitation, United States 3 NeuroSynergy Associates, PA, Neurorehabilitation, United States Background: A 28-year-old, male, patient presents with a diagnosis of functional neurological/ conversion disorder. His condition was refractory to cognitive behavioral therapy (CBT), eye movement desensitization and reprocessing (EMDR), antidepressants, and anti-anxiolytics. His symptoms include twitching and spasms of his left hand and legs, balance issues, weakness, and difficulty walking which all have gotten worse within the last year. Grades Symptom Checklist (GSC) severity was 52/162. Trails A was 19.3 seconds, Trails B was 39.4 seconds, and his processing speed was 55 symbols matched correctly in 2 minutes. His Visual Acuity line difference (VA) was 2.0. Methods: A five-day, multimodal program of receptor based neurological rehabilitation was administered three times per day, one hour per session. Each session consisted of electrical somatosensory stimulation to the third branch of his trigeminal nerve, and his tibial nerve bilaterally, therapeutic exercises, vestibular rehabilitation exercises, and off-vertical axis rotations. Results: Following conclusion of five days of receptor based treatment there were significant improvements in GS (-86.5%), TA (-13%), TB (-14%), processing speed (+32.7%) and VA (-90%). Gait, movement parameters, weakness, balance difficulties, and gait also normalized. Conclusions: Short duration, multimodal, intensive programs of receptor based neurological rehabilitation may be a viable method to improve neurological integrity and performance in individuals with a diagnosis of functional neurological/ conversion disorder. The authors suggest further investigation into short duration, multi-modal, intensive approaches to restoring neurological function in individuals suffering from treatment-resistant functional neurological/ conversion disorder. Keywords: Conversion Disorder, Functional neurological disorder, tremors, Vestibular Rehabilitation, non-invasive nerve stimulation, Off-vertical axis rotation Conference: International Symposium on Clinical Neuroscience: Clinical Neuroscience for Optimization of Human Function, Orlando, United States, 7 Oct - 9 Oct, 2016. Presentation Type: Poster Presentation Topic: Abstracts ISCN 2016 Citation: Antonucci MM and Link PE (2016). Improvement in symptom severity, processing speed, gait, movement, and visual acuity in a patient with refractory functional neurological/ conversion disorder. Front. Neurol. Conference Abstract: International Symposium on Clinical Neuroscience: Clinical Neuroscience for Optimization of Human Function. doi: 10.3389/conf.fneur.2016.59.00065 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 02 Sep 2016; Published Online: 07 Sep 2016. * Correspondence: Dr. Matthew M Antonucci, Carrick Institute, Clinical Neuroscience, Orlando, United States, drnucci@gmail.com Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers Matthew M Antonucci Paul E Link Google Matthew M Antonucci Paul E Link Google Scholar Matthew M Antonucci Paul E Link PubMed Matthew M Antonucci Paul E Link Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.

Information Processing and Motor Control in Down Syndrome

Extensive research on the motor performance of individuals with intellectual disabilities (ID) and more specifically individuals with Down syndrome (DS) reflects performance deficits in physical and motor functioning. Earlier study, indicated that low levels of physical function and deficiencies in sensory processing, memory consolidation, and postural control impact their ability to acquire and perform motor skills under variable environmental contexts. In addition, indicated that movements are often uncoordinated, slower, variable and hesitant in initiation, while other researchers reported longer movements and reaction times as well as difficulties in balance and postural control. Further limitations in spontaneous or symbolic play and limited opportunities to practice motor skills were observed in school and/or community settings, primarily during their developmental years. It is apparent that these individuals display delayed motor development and atypical motor functioning, which may be attributed to structural differences in the neurological system, delayed brain development, and a compromised somatosensory system.

Jaundice in a baby with self-improving collodion ichthyosis and ALOX12B mutation- a challenging scenario

Autosomal recessive congenital ichthyoses are a heterogeneous group of rare cornification diseases. Genetic mutations are responsible for the condition, with some causing a relatively milder phenotype such as ‘self-improving collodion ichthyosis’. In most cases, affected babies are born with a thick parchment like membrane covering their body. These babies may have a problematic postnatal course, and are prone to complications. The authors present the report of a newborn collodion baby afflicted with ALOX12B mutation, who had a challenging post natal course. Difficulties in feeding, temperature control, hydration and electrolyte balance were encountered and required precise monitoring and formulation of an effective treatment strategy. Treatment of jaundice in the baby also presented a unique challenge, which was successfully managed.

Abnormal gait, reduced locomotor activity and impaired motor coordination in Dgcr2-deficient mice

It has been suggested that the DGCR2 gene plays a role in the pathogenesis of 22q11.2 deletion syndrome. To analyze its function, we used our Dgcr2-knock-out/EGFP-knock-in mice (Dgcr2-KO mice). At 20-26 weeks of age, approximately 20% of Dgcr2-KO mice showed gait abnormalities with trembling and difficulty in balancing. Footprint test revealed awkward movements in Dgcr2-KO mice soon after they were placed on the floor. Once they started walking, their stride lengths were not different from wild-type mice. In short-term open field test, Dgcr2-KO mice travelled a significantly shorter distance and walked more slowly than wild-type mice during the initial 5min after being placed in a new environment. In long-term open field test, Dgcr2-KO mice exhibited reduced cage activity compared to wild-type mice on the first day, but not on later days. Dgcr2-KO mice showed reduced latency to fall in the rotarod test, and the latency was not improved in the 3-day test. Histology revealed sparseness of cerebellar Purkinje cells in Dgcr2-KO mice. Our results suggest that Dgcr2 plays a role in motor control related to Purkinje cell function and that the deficiency of DGCR2 contributes at least to some of the symptoms of patients of 22q11.2 deletion syndrome.

Chiropractic Response to a Spontaneous Vertebral Artery Dissection

ObjectiveThe purpose of this case report is to describe a case in which early detection and proper follow-up of spontaneous vertebral artery dissection led to satisfactory outcomes. Clinical FeaturesA 34-year old white woman reported to a chiropractic clinic with a constant burning pain at the right side of her neck and shoulder with a limited ability to turn her head from side to side, periods of blurred vision, and muffled hearing. Dizziness, visual and auditory disturbances, and balance difficulty abated within 1hour of onset and were not present at the time of evaluation. A pain drawing indicated burning pain in the suboccipital area, neck, and upper shoulder on the right and a pins and needles sensation on the dorsal surface of both forearms. Turning her head from side-to-side aggravated the pain, and the application of heat brought temporary relief. The Neck Disability Index score of 44 placed the patient’s pain in the most severe category. Intervention and OutcomeThe patient was not treated on the initial visit but was advised of the possibility of a vertebral artery or carotid artery dissection and was recommended to the emergency department for immediate evaluation. The patient declined but later was convinced by her chiropractor to present to the emergency department. A magnetic resonance angiogram of the neck and carotid arteries was performed showing that the left vertebral artery was hypoplastic and appeared to terminate at the left posterior inferior cerebellar artery. There was an abrupt moderately long segment of narrowing involving the right vertebral artery beginning near the junction of the V1 and V2 segments. The radiologist noted a concern regarding right vertebral artery dissection. Symptoms resolved and the patient was cleared of any medications but advised that if symptoms reoccurred she was to go for emergency care immediately. ConclusionRecognition and rapid response by the chiropractic physician provided the optimum outcome for this particular patient.

Short-term exposure to L-type calcium channel blocker, verapamil, alters the expression pattern of calcium-binding proteins in the brain of goldfish, Carassius auratus

The influx of calcium ions (Ca2+) is responsible for various physiological events including neurotransmitter release and synaptic modulation. The L-type voltage dependent calcium channels (L-type VDCCs) transport Ca2+ across the membrane. Calcium-binding proteins (CaBPs) bind free cytosolic Ca2+ and prevent excitotoxicity caused by sudden increase in cytoplasmic Ca2+. The present study was aimed to understand the regulation of expression of neuronal CaBPs, namely, calretinin (CR) and parvalbumin (PV) following blockade of L-type VDCCs in the CNS of Carassius auratus. Verapamil (VRP), a potent L-type VDCC blocker, selectively blocks Ca2+ entry at the plasma membrane level. VRP present in the aquatic environment at a very low residual concentration has shown ecotoxicological effects on aquatic animals. Following acute exposure for 96h, median lethal concentration (LC50) for VRP was found to be 1.22mg/L for goldfish. At various doses of VRP, the behavioral alterations were observed in the form of respiratory difficulty and loss of body balance confirming the cardiovascular toxicity caused by VRP at higher doses. In addition to affecting the cardiovascular system, VRP also showed effects on the nervous system in the form of altered expression of PV. When compared with controls, the pattern of CR expression did not show any variations, while PV expression showed significant alterations in few neuronal populations such as the pretectal nucleus, inferior lobes, and the rostral corpus cerebellum. Our result suggests possible regulatory effect of calcium channel blockers on the expression of PV.

Effects of a balance exercise programme in myotonic dystrophy type 1: A pilot study

This pilot study aimed to evaluate the effects of balance exercises in adults with classical myotonic dystrophy type 1 (DM1) directly after intervention and at follow-up after 12 weeks. Eleven outpatients (median age 49 years, range 36–60 years) with balance difficulties participating in a 10 week intervention were assessed before, after and at follow-up. The primary outcome measure was balance confidence, measured with the Activities-specific Balance Confidence (ABC) scale. Secondary outcome measures were the Step Test, Timed Up and Go test, Timed 10 m Walk at maximum pace, isometric muscle force in legs and number of falls. The most important findings directly after the intervention were that nine of the 11 patients scored higher on the ABC scale (p = 0.02), and that the number of steps in the Step Test increased in nine of 10 participants. The increased number of steps remained increased at follow-up, but the increased confidence was not maintained for all patients. Timed Up and Go was unchanged but the time to walk 10 m increased. The knee extensor force and number of falls did not show any statistically significant changes. Unexpectedly, all patients deteriorated in the ankle dorsiflexor force (mean –17%) after intervention. The study showed that self-assessed balance confidence is likely to increase following an individualized balance exercise programme in outpatients with DM1.

Counterproductive work behavior and job satisfaction: A surprisingly rocky relationship

AbstractCounterproductive work behavior is detrimental to the organization or to coworkers. It includes both breaking rules or laws, as well as social norms. Many authors show a negative relationship between this behavior and work satisfaction, surmising that the lack thereof – and the ensuing desire to ‘get back’ at the employer – are the seeds of workplace deviance. A study carried out in Polish organizations found this relationship to be less clear. The study concerned work satisfaction and counterproductive behavior, organizational justice, stress at work and propensity for aggressive behavior. A cluster analysis identified a group of individuals in whom relatively high levels of job satisfaction are nevertheless accompanied by proclivity for counterproductive behavior. The configuration of results suggests that this group resorts to counterproductive behavior because of an inability to balance difficulties at work with personal inclinations. The implications of these results for personal management is discussed in the conclusions.

Abstract P1-09-04: Longitudinal evaluation of taxane-induced neuropathy in early stage breast cancer

Abstract Introduction Chemotherapy-induced peripheral neuropathy (CIPN) is a dose-limiting toxicity of several chemotherapy drug classes, including taxanes. Peripheral neuropathies have been shown to lead to pain, falls, and difficulty in walking and performing activities of daily living in a variety of patient populations. Although the prevalence of CIPN has been noted in cancer patients, the development of self-reported symptoms, gait changes and balance changes during treatment have not been well explored to date. We hypothesized that the use of taxane-based chemotherapy will result in significant changes in spatiotemporal gait and balance parameters, as well as self-reported quality of life and function. Methods We characterized the alterations in gait and balance that occur in non-metastatic breast cancer patients during taxane chemotherapy. We evaluated (1) spatiotemporal gait parameters, including cadence and step length, and (2) balance parameters, including time-to-contact and 95% ellipse area, using each patient as her own control. Laboratory assessment of gait and balance was conducted at baseline and at completion of therapy in selected patients. We compared the natural history of changes in gait and balance parameters with changes in CIPN status as measured by validated patient reported outcomes, including EORTC QLQ-C30, CIPN-20, and Brief Pain Inventory Short Form (BPI-SF), and the Duke Activity Status Index (DASI). Time points included pre-chemotherapy, after each cycle of chemotherapy, and one month after the end of therapy to collect information on neuropathy, pain and functional capacity. The preliminary data were illustrated using individual plots; trend lines (changing over time) were based on least square means at each time point, which were estimated using the linear mixed models for repeated measures. Results To date, 15 patients with localized breast cancer have been enrolled; patient recruitment is ongoing. The median age is 42 years (range 25-67). Ten patients (67%) received weekly paclitaxel, 1 patient (7%) received paclitaxel every 2 weeks, and 4 patients (27%) received docetaxel every 3 weeks. Preliminary results with these 15 patients, based on least square means at each timepoint, showed trends in several parameters. As treatments progressed, patients tended to develop more difficulty in quiet balance and in their ability to actively shift weight in the sagittal and frontal planes. From the CIPN-20, they also tended to develop increased difficulty with sensory and motor systems. From the QLQ-C30, their global health status also tended to worsen. For most of these parameters, the largest changes were observed between the 2nd and 3rd treatments, though some changes were not observed until the 4th treatment. From the BPI-SF, no trends in pain symptoms or pain interference were observed within this preliminary cohort. Conclusions Gait and balance testing is feasible in the clinical setting. Preliminary observations suggest that balance, function and quality of life may all be affected by taxane therapy, even without pain symptoms. The findings of this study will enable us to better characterize the neurotoxic effect of taxanes and to ultimately test the effectiveness of preventative measures and interventions. Funding by NCI R03CA182165-01. Citation Format: Maryam B Lustberg, Scott Monfort, Janani Singaravelu, Raquel E Reinbolt, Xueliang Pan, Bhuvaneswari Ramaswamy, Rachel M Layman, Robert Wesolowski, Ewa Mrozek, Erin Macrae, Charles Shapiro, Robyn Patrick, Charles L Loprinzi, Ajit Chaudhari. Longitudinal evaluation of taxane-induced neuropathy in early stage breast cancer [abstract]. In: Proceedings of the Thirty-Seventh Annual CTRC-AACR San Antonio Breast Cancer Symposium: 2014 Dec 9-13; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2015;75(9 Suppl):Abstract nr P1-09-04.

Do children with cerebral palsy change their gait when walking over uneven ground?

Independently ambulant children with Cerebral Palsy (CP) often report balance difficulties when walking in challenging settings. The aim of this study was to compare gait in children with CP to typically developing (TD) children walking over level ground and uneven ground, as an evaluation of dynamic balance. Thirty-four children participated, 17 with CP (10 hemiplegia and 7 diplegia, mean age 10 years) and 17 TD (mean age 10 years 1 month). Three-dimensional kinematic and kinetic data of the lower limbs and trunk were captured during walking over level and uneven ground using Codamotion®. Statistical analysis was performed using a mixed-effects model two-factor Analysis of Variance (Group×Surface). Over both surfaces, children with CP showed increased trunk movement in the sagittal (Group effect, p<0.001) and transverse planes (p<0.001), and increased pelvic movement in the coronal plane (p=0.008), indicating impaired trunk control. Peak separation between the centre of mass and centre of pressure was reduced in CP, indicating impaired dynamic balance (p=0.027). TD children made a number of significant adaptations to uneven ground, including reduced hip extension (mean difference 3.4°, 95% CI [−5.3, −1.0] p=0.006), and reduced ankle movement in the sagittal (5.2°, 95% CI [0.01, 10] p=0.049) and coronal planes (2.4°, 95% CI [0.3, 4.5], p=0.029), but these adaptations were not measured in CP. A significant Group×Surface interaction was detected for knee sagittal range (p=0.009). The findings indicate that children with CP walk show impaired control of trunk movement and are less able to adapt their gait to uneven ground, particularly at the ankle.

High mercury seafood consumption associated with fatigue at specialty medical clinics on Long Island, NY.

We investigated the association between seafood consumption and symptoms related to potential mercury toxicity in patients presenting to specialty medical clinics at Stony Brook Medical Center on Long Island, New York. We surveyed 118 patients from April–August 2012 about their seafood consumption patterns, specifically how frequently they were eating each type of fish, to assess mercury exposure. We also asked about symptoms associated with mercury toxicity including depression, fatigue, balance difficulties, or tingling around the mouth. Of the 118 adults surveyed, 14 consumed high mercury seafood (tuna steak, marlin, swordfish, or shark) at least weekly. This group was more likely to suffer from fatigue than other patients (p = 0.02). Logistic regression confirmed this association of fatigue with frequent high mercury fish consumption in both unadjusted analysis (OR = 5.53; 95% CI: 1.40–21.90) and analysis adjusted for age, race, sex, income, and clinic type (OR = 7.89; 95% CI: 1.63–38.15). No associations were observed between fish intake and depression, balance difficulties, or tingling around the mouth. Findings suggest that fatigue may be associated with eating high mercury fish but sample size is small. Larger studies are needed to determine whether fish intake patterns or blood mercury tests warrant consideration as part of the clinical work-up in coastal regions.

Quantifying dynamic and postural balance difficulty during gait perturbations using stabilizing/destabilizing forces

Intensity of balance exercises used to reduce fall risk is often poorly quantified. The study aimed to test whether balance difficulty can be rated during gait perturbations against balance difficulty during gait without perturbation, using the stabilizing/destabilizing forces. These forces represent the difficulty to maintain balance as the theoretical forces necessary to cancel body velocity and to set the body into an unstable posture, respectively. Ten healthy subjects walked on a split-belt treadmill, that also generated perturbations. Kinetic and kinematic data were collected during gait at comfortable and fast speeds without perturbation, and in five trials at comfortable speed with perturbations. Perturbations consisted of increasing or decreasing the speed of one belt to three different levels in each direction in a random order during the stance phase of 12 random steps per trial. The difficulty of maintaining balance was measured during the perturbation and the three following recovery steps. Compared to comfortable speed, higher stabilizing and lower destabilizing forces indicated higher balance difficulty during the perturbation step for faster-belt perturbations, and recovery steps for slower-belt perturbations. This was also associated with the center of mass shifted forward, and moving faster, and with the center of pressure closer to the forward limit of the base of support. Difficulty increased proportionally with the intensity of perturbation and was significantly higher for the more intense perturbations than at fast speed. Thus, the stabilizing/destabilizing forces seem adequate to evaluate balance difficulty during gait perturbations and could be used to determine the optimal difficulty for balance rehabilitation.

Role of body-worn movement monitor technology for balance and gait rehabilitation.

This perspective article will discuss the potential role of body-worn movement monitors for balance and gait assessment and treatment in rehabilitation. Recent advances in inexpensive, wireless sensor technology and smart devices are resulting in an explosion of miniature, portable sensors that can quickly and accurately quantify body motion. Practical and useful movement monitoring systems are now becoming available. It is critical that therapists understand the potential advantages and limitations of such emerging technology. One important advantage of obtaining objective measures of balance and gait from body-worn sensors is impairment-level metrics characterizing how and why functional performance of balance and gait activities are impaired. Therapy can then be focused on the specific physiological reasons for difficulty in walking or balancing during specific tasks. A second advantage of using technology to measure balance and gait behavior is the increased sensitivity of the balance and gait measures to document mild disability and change with rehabilitation. A third advantage of measuring movement, such as postural sway and gait characteristics, with body-worn sensors is the opportunity for immediate biofeedback provided to patients that can focus attention and enhance performance. In the future, body-worn sensors may allow therapists to perform telerehabilitation to monitor compliance with home exercise programs and the quality of their natural mobility in the community. Therapists need technological systems that are quick to use and provide actionable information and useful reports for their patients and referring physicians. Therapists should look for systems that provide measures that have been validated with respect to gold standard accuracy and to clinically relevant outcomes such as fall risk and severity of disability.

Integration of in-situ CO2-oxy coal gasification with advanced power generating systems performing in a chemical looping approach of clean combustion

Underground coal gasification (UCG) is a clean coal technology to utilize deep coal resources effectively. In-situ CO2-oxy coal gasification may eliminate the operational difficulty of the steam gasification process and utilize CO2 (greenhouse gas) effectively. Furthermore, it is necessary to convert the clean gasified energy from the UCG into clean combustion energy for an end-use. In order to achieve efficient clean power production, the present work investigates the thermodynamic feasibility of integration of CO2 based UCG with power generating systems operating in a chemical looping combustion (CLC) of product gas. The use of CO enriched syngas from O2/CO2 based UCG reduces the difficulty of the heat balance between a fuel reactor and an air reactor in a nickel oxygen-carrier based CLC system. Thermodynamic analyses have been made for various routes of power generation systems such as subcritical, supercritical and ultra-supercritical boiler based steam turbines and gas turbines for the UCG integrated system. It is shown, based on mass and energy balance analysis, that the integration of CO2 based UCG with the CLC system reduces the energy penalty of carbon capture and storage (CCS) significantly. A net thermal efficiency of 29.42% is estimated for the CCS incorporated system, which operates in a subcritical condition based steam turbine power plant. Furthermore, it is found that the efficiency of the proposed steam turbine system increases to 35.40% for an ultra-supercritical operating condition. The effect of operating temperature of the air reactor and the fuel reactor of the CLC system on the net thermal efficiency of combined cycle power plant is investigated. It is found that a net thermal efficiency of 42.53% can be obtained for the CCS incorporated combined cycle power system operating at an air reactor temperature of 1200°C.

Writer's Cramp as the First Symptom of Spinocerebellar Ataxia 14.

A 57-year-old right-handed man with no family history of neurological disorder presented with an 18-month history of abnormal posturing of his hand upon writing. Abnormal posturing did not affect any other activities, but was significantly disabling given that he had to write frequently as part of his work. Neurological examination was normal apart from abnormal posturing of his right hand upon writing, with hyperextension of the right thumb, with (possibly secondary compensatory) hyperflexion at the distal interphalangeal joint. He received partial benefit from botulinum toxin injections and was able to successfully manage his symptoms for the subsequent 8 years. At the age of 65, he became aware of progressive balance difficulty. When walking, he started veering off to the left or to the right inconsistently. On examination, ocular movements were normal. There was a bilateral postural jerky tremor of his hands (right>left). Finger-nose and heel-shin tests were normal. He had rebound phenomenon in both upper limbs and minor difficulties when tandem walking (see Videos 1 and 2). The remaining neurological examination was normal. Brain MRI revealed mild atrophy of the cerebellum (Fig. 1). Genetic testing for the most common forms of SCAs was performed and he was found to carry a previously described mutation (c.413T>A, p.V138E) in PRKCG, leading to diagnosis of SCA14. 1 Discussion We have reported here on a patient with a 10-year history of isolated writer’s cramp before developing balance problems accompanied by cerebellar signs on examination and confirmed to carry a pathogenic SCA14 mutation. At first presentation, there were no clinical clues by history or examination to suggest cerebellar involvement. Clinically, our case had, in fact, a typical writer’s cramp with evident mirroring, which is thought to be suggestive of primary dystonia, even though we might argue, in retrospect, that age at onset (i.e., 55 years) was outside the usual range of patients with isolated writer’s cramp, who often develop their initial symptoms in their thirties. SCA14 accounts for 2% of all SCAs and is caused by

MS-21 * SUBEPENDYMOMAS DEMONSTRATE LINEAGE MARKERS SUGGESTING AN ORIGIN WITHIN MULTIPOTENTIAL PROGENITORS OF THE SUBVENTRICULAR ZONE

OBJECTIVE: Subependymomas are infrequent, low-grade gliomas associated with the ventricular system and the spinal cord. Little is known about the origin and natural history of these slow-growing lesions. METHODS: We identified all patients with pathologically proven subependymomas presenting to our institution between 1998 and 2013. We retrospectively reviewed clinical, radiographic, histological, and surgical outcomes data in all patients who underwent surgical resection. Immunohistochemical analyses for a number of cell lineage markers was performed. RESULTS: A total of 24 patients with pathologically proven subependymomas were identified. Of these, 7 asymptomatic lesions were discovered at autopsy and 17 symptomatic cases were treated surgically. There were 8 lateral ventricle tumors, 5 spinal tumors and 4 fourth ventricular tumors. Symptomatic intracranial lesions most commonly presented with headaches, changes in vision, and balance difficulty. Subependymomas had no distinguishing radiographic features that provided definitive pre-operative diagnosis. At last follow up, no patient treated surgically experienced recurrence, although one patient with histologically mixed subependymoma-ependymoma subsequently developed and was treated for a remote glioblastoma. Immunohistochemical analyses demonstrated mixed populations of cells that were variably positive for Olig2, NeuN, Sox2, and CD44. CONCLUSION: Subependymomas demonstrate a mixed population of cells expressing neuronal and glial lineage markers as well as putative stem cell markers, suggesting these tumors may arise from multipotential progenitors that reside in the subventricular zone. Definitive diagnosis requires surgical sampling. While the clinical course is usually benign following surgical resection, the rare association with glial malignancy and the potential for malignant progression merits further investigation.

Nutritional status, assessment, requirements and adequacy of traumatic brain injury patients.

Traumatic Brain Injury (TBI) has been considered as a serious public health problem. Each year, traumatic brain injuries are contributing to a substantial number of cases of permanent disability and deaths and it can be classified according to the severity into penetrating and closed head injury. Symptoms, beside to be unconscious can be defined as vomiting, nausea, headache, dizziness, lack of motor coordination, difficulty in balancing, blurred vision and lightheadedness, bad taste in the mouth, ringing in the ears, fatigue and lethargy as well as changes in sleep patterns. The brain is known to be the functional regulator for all the metabolic activities inside the body and TBI patients mostly have a complex metabolic alterations including aberrant cellular metabolism, abnormal metabolic processes, changes in hormones functions and inflammatory cascade. The TBI patient's status needed to be assessed medically and nutritionally since the medical status of the patients can affect the nutrition part. Data from the four assessment tools are needed to be correctly used and interpreted in order to make a proper nutritional diagnosis, clinical assessment, biochemistry as well as anthropometric measurements. Regardless the methods used for assessing TBI patients, having adequate intake and medical care can lead to a reduction in hospital costs, numbers of day hospitalized, numbers of hours of mechanical ventilation and in the overall infection rates.

Vestibular dysfunction in Apert Syndrome

Objectives: Apert syndrome affects the craniofacial structures resulting in craniosynostosis, craniofacial anomalies and syndactyly. Although many characteristic features are recognized, vestibular dysfunction is a little known feature that can often be present. This can cause a delay in the attainment of gross motor milestones and impair motor function. We aimed to examine this cohort to review the incidence of vestibular hypofunction and to assess the effect on motor function. Study design: We performed a retrospective analysis of 55 Apert syndrome patients who had undergone audiological review at Great Ormond Street Hospital for Children. Results: 50.9% of patients experienced balance difficulties; 18.1% of patients (all of whom had delayed motor milestones) underwent clinical examination and vestibular assessment. Results showed only 20% of patients had bilateral vestibular function present; 30% of patients had unilateral and 50% bilateral vestibular hypofunction. Two patients who initially presented with imbalance and vestibular hypofunction experienced significant improvement in symptoms on re-examination following vestibular physiotherapy. Conclusion: Vestibular dysfunction is a feature of Apert syndrome. Although the imbalance may be attributable to mechanical problems arising from syndactyly, it is important to recognize that a vestibular component can coexist. This is not well recognized, poorly understood and scarcely treated. We aim to increase awareness of vestibular dysfunction in Apert syndrome and advocate a high index of suspicion, particularly in the presence of motor milestone delay. Early detection, thorough vestibular assessment and prompt initiation of vestibular physiotherapy can aid vestibular compensation, rehabilitating patients and maximizing their functional attainment.

Impaired balance and neurodevelopmental disabilities among children with congenital cytomegalovirus infection.

Although cytomegalovirus (CMV) is the most common congenital infection, existing research has not provided us with a full picture of how this can affect children in the future. The aim of this case-control study was to evaluate disabilities in a well-defined group of children with congenital cytomegalovirus (CMV) infection, who had been fitted with cochlear implants because of severe hearing impairment. A multidisciplinary team assessed 26 children with congenital CMV infection for balance difficulties, neurodevelopmental disabilities and language and visual impairment. We also included a control group of 13 children with severe hearing impairment due to connexin 26 mutations. The majority of the children with congenital CMV infection (88%) displayed balance disturbances, including walking at a later age, but there were no cases in the control group. The CMV group also displayed frequent neurodevelopmental disabilities and feeding difficulties. Congenital CMV infection affects the general development of the brain and gives rise to a complex pattern of difficulties. Identifying comorbid conditions is very important, as children with associated difficulties and disabilities need more support than children with just hearing impairment. Congenital CMV infection needs to be considered in children with hearing impairment and/or balance disturbance and/or neurodevelopmental disabilities.

The evolution of primary progressive apraxia of speech.

Primary progressive apraxia of speech is a recently described neurodegenerative disorder in which patients present with an isolated apraxia of speech and show focal degeneration of superior premotor cortex. Little is known about how these individuals progress over time, making it difficult to provide prognostic estimates. Thirteen subjects with primary progressive apraxia of speech underwent two serial comprehensive clinical and neuroimaging evaluations 2.4 years apart [median age of onset = 67 years (range: 49-76), seven females]. All underwent detailed speech and language, neurological and neuropsychological assessments, and magnetic resonance imaging, diffusion tensor imaging and (18)F-fluorodeoxyglucose positron emission tomography at both baseline and follow-up. Rates of change of whole brain, ventricle, and midbrain volumes were calculated using the boundary-shift integral and atlas-based parcellation, and rates of regional grey matter atrophy were assessed using tensor-based morphometry. White matter tract degeneration was assessed on diffusion-tensor imaging at each time-point. Patterns of hypometabolism were assessed at the single subject-level. Neuroimaging findings were compared with a cohort of 20 age, gender, and scan-interval matched healthy controls. All subjects developed extrapyramidal signs. In eight subjects the apraxia of speech remained the predominant feature. In the other five there was a striking progression of symptoms that had evolved into a progressive supranuclear palsy-like syndrome; they showed a combination of severe parkinsonism, near mutism, dysphagia with choking, vertical supranuclear gaze palsy or slowing, balance difficulties with falls and urinary incontinence, and one was wheelchair bound. Rates of whole brain atrophy (1.5% per year; controls = 0.4% per year), ventricular expansion (8.0% per year; controls = 3.3% per year) and midbrain atrophy (1.5% per year; controls = 0.1% per year) were elevated (P ≤ 0.001) in all 13, compared to controls. Increased rates of brain atrophy over time were observed throughout the premotor cortex, as well as prefrontal cortex, motor cortex, basal ganglia and midbrain, while white matter tract degeneration spread into the splenium of the corpus callosum and motor cortex white matter. Hypometabolism progressed over time in almost all subjects. These findings demonstrate that some subjects with primary progressive apraxia of speech will rapidly evolve and develop a devastating progressive supranuclear palsy-like syndrome ∼ 5 years after onset, perhaps related to progressive involvement of neocortex, basal ganglia and midbrain. These findings help improve our understanding of primary progressive apraxia of speech and provide some important prognostic guidelines.