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Objective: The Mobile Universal Lexicon Evaluation System (MULES) is a test of rapid picture naming under investigation. Measures of rapid automatic naming (RAN) have been used for over 50 years to capture aspects of vision and cognition. MULES was designed as a series of 54 grouped color photographs (fruits, random objects, animals) that integrates saccades, color perception and contextual object identification. We examined MULES performance in youth, collegiate and professional athletes at pre-season baseline and at the sidelines following concussion., Methods: Our study teams administered the MULES to youth, collegiate and professional athletes during pre-season baseline testing. Sideline post-concussion time scores were compared to pre-season baseline scores among athletes with concussion to determine degrees and directions of change., Results: Among 681 athletes (age 17 ± 4 years, range 6-37, 38% female), average test times at baseline were 41.2 ± 11.2 s. The group included 280 youth, 357 collegiate and 44 professional athletes; the most common sports were ice hockey (23%), soccer (17%) and football (11%). Age was a predictor of MULES test times, with longer times noted for younger participants (P < .001, linear regression). Consistent with other timed performance measures, significant learning effects were noted for the MULES during baseline testing with trial 1 test times (mean 49.2 ± 13.1 s) exceeding those for trial 2 (mean 41.3 ± 11.2 s, P < .0001, paired t-test). Among 17 athletes with concussion during the sports seasons captured to date (age 18 ± 3 years), all showed increases (worsening) of MULES time scores from pre-season baseline (median increase 11.2 s, range 0.6-164.2, P = .0003, Wilcoxon signed-rank test). The Symptom Severity Score from the SCAT5 Symptom Evaluation likewise worsened from pre-season baseline following injury among participants with concussion (P = .002)., Conclusions: Concussed athletes demonstrate worsening performance on the MULES test compared to their baseline time scores. This test samples a wide network of brain pathways and complements other vision-based measures for sideline concussion assessment. The MULES test demonstrates capacity to identify athletes with sports-related concussion., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: The spectrum of symptoms exhibited by patients with essential tremor (ET) extends far beyond the classical tremor. This study aims to explore and establish the presence of subtle balance abnormalities in ET using dynamic posturography (DP)., Methods: DP was performed on 18 patients with ET and 26 controls. Diagnosis of ET was based on the Consensus Statement of the Movement Disorder Society on Tremor. Dynamic stability which included the overall balance index, anterior-posterior index and mediolateral index, and limits of stability were measured., Results: Patients with ET had significantly impaired balance indices. Impairment of dynamic stability revealed poor static balance control in all directions. Lower limits of stability scores indicated a smaller range of motion prior to which patients have to shift foot balance. No correlations were observed between age at evaluation, age at onset, duration of illness and the balance indices., Conclusions: Dynamic posturography reveals significant balance impairment in patients with ET which is unrelated to the age at onset, age at evaluation or duration of illness. This finding concurs with pre-existing reports and adds to the growing body evidence of cerebellar involvement in ET., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: In this prospective study, involvement of sensory nerve fibres in ALS patients was assessed using functional and structural measures in the form of quantitative sensory testing (QST) and skin and nerve biopsies., Methods: Thirty-two ALS patients and 32 healthy subjects were evaluated with a QST battery comprising thresholds of mechanical detection, mechanical pain, vibration detection, cold detection, warm detection, heat pain, and pinprick sensation. Skin biopsies were evaluated in 31 ALS patients by intraepidermal nerve fibre density (IENFD) and axonal swelling ratios, and growth-associated protein 43 (GAP-43) antibody staining. Sural nerve biopsies were evaluated using teased fibre analysis in eight patients., Results: Mean values for QST parameters and IENFD in ALS patients were within normal range. However, the patients had increased axonal swelling ratios and GAP-43 antibody staining was negative in all patients., Conclusions: Although QST and IENFD were affected in only a small subset of ALS patients, the axonal swellings observed in all patients indicate that the affection is more frequent, and suggests that IENFD count may not be sufficient. The negative GAP-43 staining suggested an insufficiency of regeneration in small sensory nerve fibres., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Clinicians frequently experience difficulty in eliciting the reflexes of elderly patients using standard methods due to paratonia/frontal rigidity. If reflexes are incorrectly thought to be absent, important diagnostic errors may be made. Neurologists use alternative methods when technical difficulties require them, but these are not widely used by non-neurologists., Methods: A neurologist and a medical student both used standard and non-standard techniques to assess reflexes of the lower limb in geriatric inpatients, aged over 65, to determine which method permitted the most confident assessment of the presence of knee and ankle reflexes., Results: 45 patients were assessed. The consultant found that in 20 patients (44%) all three knee reflex methods examined produced similar results. When the methods produced different results, the "superior patellar supine" method was the best single method overall (best or equal best in 19 patients (42%)). For the ankle reflex all four reflex methods examined produced similar results in only 7 patients (16%). When the methods produced different results the "Achilles strike elevated" method was best or equal best in 32 patients (71%) and the "plantar strike" method in 29 patients (64%). If the student had relied on standard methods alone, reflexes would have been incorrectly called absent in 28 limbs (37%) for knee jerks and 52 limbs (84%) for ankle jerks. Supplementing standard methods with alternative methods reduced these error rates to 19% and 21% respectively., Conclusions: Our findings indicate that a reasonable practical approach is to assess the knee reflex with the standard method and then, if a definite reflex has not been recorded, move on to use the "superior patellar supine" method; and for the ankle reflex begin with the "plantar strike method" and then, if necessary, move on to use the "Achilles strike elevated" method., (Copyright © 2016. Published by Elsevier B.V.)

The use of rodent stroke models allow for the understanding of stroke pathophysiology. There is currently no gold standard neurological assessment to measure deficits and recovery from stroke in rodent models. Agreement on a universal preclinical stroke scale allows for comparison of the outcomes among conducted studies. The present study aimed to compare three routinely used neurological assessments in rodent studies (i.e., Garcia, Modo, and Longa) to determine which is most effective for accurately and consistently quantifying neurological deficits in the context of focal middle cerebral artery occlusion (MCAo) in rats. Focal MCAo was induced in 22 male Wistar rats using a novel transfemoral approach. Rodents were assessed for neurological deficit pre-injury as well as 3 and 24h post-injury. Data was analyzed to determine Pearson correlation coefficients in addition to McNemar's χ(2) values between each pair of neurological assessments. All three stroke scales, Garcia, Modo, and Longa, showed statistically significant changes between the baseline and the 3-hour neurological assessments. A trend towards neurological recovery was observed in all three stroke scales between the 3 and 24-hour endpoints. The three scales were highly correlated with each other, with Garcia and Modo having the strongest correlation. Of the three pairwise analyses, the comparison between the Garcia and Longa tests demonstrated the highest McNemar's χ(2) value, indicating least marginal homogeneity between these two tests. The combination of high correlation between Garcia and Modo tests along with greatest marginal heterogeneity observed between the Garcia and Longa test lead us to recommend the use of Garcia and Longa neurological scales when researchers are hoping to capture the broadest range of neurological factors using only two stroke scales., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Introduction: 'Pushing the wall' has found acceptance in medical teachings. Other methods of scapular winging are less known. Comparative evaluation of the five available methods has not been undertaken. This study focuses on evaluation of the available methods in groups of neuromuscular disorders to select the most sensitive method and to characterize patterns of scapular winging. A survey of methods practiced by clinicians also forms a part of the study., Materials and Methods: Prospective study. Part A: questionnaire based survey of clinicians to explore the preferred method of examination for scapular winging. Part B: comparative analysis of five methods of scapular winging in four categories of neuromuscular disorders [facioscapulohumeral muscular dystrophy (FSHD), limb girdle muscular dystrophy, dystrophinopathies and neurogenic disorders]., Results and Conclusions: Forward lowering of arms was the most sensitive method [100%]. The use of this method in clinical teachings and routine bedside examination should be promoted. Pushing the wall was the most popular method, but was fourth in the sensitivity [60.41%]. Arm maneuvers can bring out winging, when it is not apparent at rest. FSHD patients had a unique combination of winging at rest, persistence of winging throughout the range of motion and elevation of scapulae., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Freezing of gait is an episodic gait disorder that may occur in patients with Parkinson's disease. The risk factors for this disorder are poorly understood. To determine the relevant risk factors for this condition, we screened 160 consecutive patients with Parkinson's disease for freezing of gait and assessed 36 potentially related variables. Freezers and non-freezers were compared using statistical univariate analysis, followed by bivariate and multivariate logistic regression, receiver operating characteristics curves and Kaplan-Meier estimates. Seventy-one patients (44.4%) reported freezing of gait. At onset, the mean disease duration was 8.1±6.3years. Freezers experienced falls more frequently than non-freezers (57.7% vs 23.6%, p<0.001). Disease duration was the independent variable most associated with freezing of gait (OR=1.10, 95% CI=1.01-1.19, p=0.020). Its specificity was 77%, but its sensitivity was low, and Hoehn and Yahr staging and the UPDRS (part III) score showed similar accuracy to that of disease duration in predicting freezers. Previous antiparkinsonian treatments and predominant motor signs (tremor/akinesia-rigidity subtypes) at the onset of Parkinson's disease were not related to freezing of gait. Patients who developed Parkinson's disease before the age of 60years experienced freezing of gait earlier than older patients (log-rank, p<0.005). Freezing of gait is a common and disabling motor complication of Parkinson's disease that is related to the progression of the disease. It is not primarily associated with dopamine replacement therapy and may occur early in young patients., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Background: There have been no recent population-based studies on all-cause adult neurological morbidity in sub-Saharan Africa. We have developed a screening survey to improve the feasibility in performing these studies., Methods: Our screening instrument contains both history questions and examination items. We pilot tested this instrument in the Hai District, Tanzania, and Butajira, Ethiopia using trained individuals from the local communities. To measure sensitivity, we applied the instrument blindly to 25 previously-identified subjects with Parkinson's disease, stroke or epilepsy. To measure specificity, we examined 42 randomly selected previously screened subjects. We also compared the validity of the entire instrument to the history-only section., Results: There were 669 adult subjects screened in both communities (150 screen-positives, and 519 screen-negatives). The sensitivity of the instrument was 100% (95% CI 84.2-100%) and the specificity was 82.4% (95% CI 66.1-92.0%). However, when restricting the instrument to the history-only section, the sensitivity remained unchanged, but the specificity became 91.2% (95% CI 76.3-97.7%; p=0.48)., Conclusions: We have created a valid tool to screen adults for neurologic morbidity in resource-poor communities. The use of the history-only section of the tool is adequate as a screen and will improve feasibility., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Aim: We systematically performed autonomic testing on patients with polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome (POEMS) to determine whether autonomic function is preserved in such patients., Methods: We studied 17 POEMS patients, 17 diabetic neuropathy (DN) patients and 17 age-matched normal subjects. Blood pressure responses to the head-up tilt test and heart rate variability were used to evaluate cardiovascular autonomic function. Sweat responses and cutaneous vasoconstriction to several stimuli were recorded via the finger tips to estimate cutaneous sympathetic function. In addition, motor nerve conduction studies were performed., Results: Although the results of the autonomic testing were normal in POEMS patients, motor disability was severe, and motor nerve conduction studies provided evidence of extensive axonal loss. The DN patients showed significantly impaired autonomic responses despite mild motor dysfunction., Conclusions: Autonomic function was normal in POEMS patients, indicating the preservation of autonomic fibers and selective involvement of large fibers., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Background: Disability/problems, one phenomenon underlying people's need for health care services, can be viewed both from the perspectives of people with stroke (felt problems), and the health professionals (assessed problems)., Objective: The aim was to describe felt problems three months after stroke and to explore the concurrence between felt problems and assessed problems., Method: The patients (n=203) received care in the stroke units at Karolinska University Hospital, Sweden. Felt problems, drawn from an open question, were categorized. Results from established assessment tools: Katz Extended Index of ADL (KI); Barthel Index (BI) and Stroke Impact Scale (SIS) represented assessed problems. Items/domains in the assessment tools that corresponded to the categories of felt problems were identified and comparisons performed., Result: The category Fatigue had the largest number of felt problems (n=58, 28%). Fourteen out of the 24 categories of felt problems had corresponding items/domains in the assessment tools. KE/BI failed to identify 16-57% and SIS 0-33% of the felt problems., Conclusion: There was a substantial lack of concurrence between felt and assessed problems. The results indicate that the use of standardized instruments should be complemented by a dialog if health services are to be based on problems experienced by the patients., (Copyright © 2011 Elsevier B.V. All rights reserved.)

We investigated the utility of home-based gait monitor assessment in patients with spinocerebellar ataxia (SCA). Nineteen patients with different forms of SCA were examined using a step activity monitor (SAM), clinical scales and common tests of functional motor performance including the scale for assessment and rating of ataxia (SARA), disease staging, a timed 25 foot walk test and a 9 hole peg-board test. The patient wore the SAM bracelet on his/her ankle for seven 24 hour periods at home. The objective monitor measurements were highly associated with disease duration and with the functional stage of disease (P<0.01). Monitor measurements were significantly correlated to SARA scores with the exception of the percent of steps expended in moderate and high speeds of activity. Fewer monitor measures had significant correlations with walk and peg board scores. The objective SAM outputs also possessed high internal consistency, high intraclass correlation coefficients and could be fitted to a single factor by factor analysis. We conclude that the SAM is a simple and cost effective method for obtaining data on gait parameters over extended periods in patients with SCA. The SAM has validity and reliability and can generate unbiased, continuous data that takes into account daily variations in physical performance., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Background: Herpes zoster affects essentially sensory fibres with segmental distribution. Abdominal wall paresis is a rare complication., Aims of the Study: We present the case of a 72 year-old man with herpes zoster infection in T11-T12 left dermatomes and segmental abdominal wall protrusion., Methods: Electromyography (EMG) and dermatomal somatosensory evoked potentials (DSEPs) were performed 27 days after symptoms onset., Results: EMG confirmed acute axonal lesion in left external oblique muscle and left paraspinal muscles at T11-T12 level and DSEPs assessed topographic distribution: there was no response in the left side at T12 dermatome. Three months following the onset of shingles, the abdominal wall protrusion had completely resolved., Conclusions: Neurophysiological examination, including EMG and DSEPs, confirms motor and sensory loss in this unusual post-herpetic complication., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Background: Sensory and motor dysfunction in multiple sclerosis (MS) is often assessed with rating scales which rely heavily on clinical judgment. Quantitative devices may be more precise than rating scales., Objective: To quantify lower extremity sensorimotor measures in individuals with MS, evaluate the extent to which they can detect functional systems impairments, and determine their relationship to global disability measures., Methods: We tested 145 MS subjects and 58 controls. Vibration thresholds were quantified using a Vibratron-II device. Strength was quantified by a hand-held dynamometer. We also recorded Expanded Disability Status Scale (EDSS) and Timed 25-Foot Walk (T25FW). t-tests and Wilcoxon-rank sum were used to compare group data. Spearman correlations were used to assess relationships between each measure. We also used a step-wise linear regression model to determine how much the quantitative measures explain the variance in the respective functional systems scores (FSS)., Results: EDSS scores ranged from 0-7.5, mean disease duration was 10.4 ± 9.6 years, and 66% were female. In relapsing-remitting MS, but not progressive MS, poorer vibration sensation correlated with a worse EDSS score, whereas progressive groups' ankle/hip strength changed significantly with EDSS progression. Interestingly, not only did sensorimotor measures significantly correlate with global disability measures (i.e., EDSS), but they had improved sensitivity, as they detected impairments in up to 32% of MS subjects with normal sensory and pyramidal FSS., Conclusions: Sensory and motor deficits in MS can be quantified using clinically accessible tools and distinguish differences among MS subtypes. We show that quantitative sensorimotor measures are more sensitive than FSS from the EDSS. These tools have the potential to be used as clinical outcome measures in practice and for future MS clinical trials of neurorehabilitative and neuroreparative interventions., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Syncope associated to orthostatic hypotension (OH), urinary incontinence and constipation is common symptoms in demented patients, mainly in dementia with Lewy bodies (DLB) and in Parkinson's disease dementia (PDD). Alzheimer's disease (AD) and fronto temporal lobar degeneration (FTLD) show less autonomic dysfunction. Urinary symptoms are a prominent component of normal pressure hydrocephalus (NPH). There are non invasive tests including standard cardiovascular tests, 123 I-metaiodobenzylguanide (MIBG) cardiac scintigraphy, urodynamic tests, gastrointestinal motility studies, sweating reflexes and pupillary responses that assess autonomic dysfunction in these patients. The study of autonomic symptoms and abnormal tests in patients with dementia is useful to prevent morbidity due falls, severe constipation and to avoid side effects of drugs that interfere with autonomic function., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Objective: To investigate the effect of mild cognitive impairment (MCI) on balance., Methods: 87 subjects with subjective memory impairment were enrolled, and subdivided into two groups, MCI and non-MCI, according to diagnostic criteria of amnestic subtype of MCI according to the 1999 MCI international panel (Current Concepts in Mild Cognitive Impairment). These two groups were matched for age and gender. Posturography was used to assess balance by measuring the mediolateral and anteroposterial sway speed and distance in the standing position, with both opened and closed eyes., Results: The mediolateral sway speed and distance were higher in the MCI group than the non-MCI group, with both opened and closed eyes (p<0.05). However, there was no significant difference between the MCI group and non-MCI in anteroposterior sway speed and distance. These results were confirmed in a multivariate model adjusting for gender, age, weight, height, foot size, and education. The mediolateral, and anteroposterior sway speed and distance values were higher on eye closing status than on eye opening status in both the MCI and control groups (p<0.00)., Conclusion: The falling risk is assumed to be higher in MCI subjects than in non-MCI subjects, especially due to decreased mediolateral balance, as shown in our adjusted analysis. These findings underscore the importance of specific balance exercise in which mediolateral balance is measured and visual compensation training programs for MCI subjects in order to prevent fall and related fracture, as well as the importance of programs for improvement of cognitive function., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Objectives: To describe the long term evolution and outcome of a homogeneous cohort of patients (n=14) with large demyelinating lesions (LDLs) as the first clinical event from a group of idiopathic inflammatory demyelinating diseases of central nervous system., Methods: Detailed review of LDLs from December 2002-January 2007 was made. Patients had at least two magnetic resonance imaging (MRI) and minimum follow-up of 2years. The disability was assessed using Estimated Expanded Disability Status Scale (EDSS) and Rappaport Disability Rating Scale (DRS) at onset and last follow-up., Results: Fourteen consecutive LDL patients (male=7), with mean age 32.7years and mean follow-up of 45.5months were included. Motor deficits (79%) and cognitive symptoms (43%) marked the onset, none had optic neuritis. All except two responded to steroids. Follow-up MRI showed complete resolution in 43%, 57% showing marked reduction in size. On follow-up, 2 relapsed. Mean EDSS and DRS at presentation were 5.93 and 9.07 and at last follow-up were 1.75 and 2.25 (p<0.001)., Conclusions: Our data suggests that, patients presenting with LDLs as their first clinical event behaves distinctly in their presentation, imaging characteristics, prognosis and long term outcome as compared to MS and ADEM. Albeit significant disability at the onset, these patients show an excellent response to treatment with good functional recovery in long term and rare relapses., (Copyright 2010 Elsevier B.V. All rights reserved.)

Triple A syndrome is caused by mutations in the gene encoding ALADIN, leading to achalasia, alacrima and addisonism. Neurologic manifestations of the disease include motor neuron disease-like presentations, motor-sensory or autonomic neuropathy, optic atrophy, cerebellar ataxia, Parkinsonism, and mild dementia. We report a 60-year-old Japanese man with triple A syndrome. He was born to non-consanguineous parents. He underwent a surgical operation for achalasia at age 40, and thereafter, he developed a slowly progressive gait disturbance. Neurological examinations at age 60 revealed limb muscle wasting and weakness with pyramidal tract signs, distal-dominant sensory disturbance, optic atrophy, and autonomic dysfunction. Alacrima was detected using Schirmer test. All of these features were consistent with typical triple A syndrome. He lacked adrenal insufficiency that is frequently observed in patients with the classic phenotype of triple A syndrome. His sural nerve biopsy showed a moderate loss of myelinated fibers and hypomyelination. He was homozygous for a missense mutation, p.R155H, in the disease-causing gene, AAAS. Seven patients with genetically-confirmed, adult or late-onset triple A syndrome, including ours, have been reported to date. All the patients showed upper and lower motor neuron signs (100%), while sensory disturbance (29%) and autonomic dysfunction (57%) were less frequent. Careful assessment for alacrima followed by molecular genetic analysis of AAAS should be considered in patients who show a combined phenotype of motor neuron disease and sensory/autonomic disturbance, even in elderly patients., (Copyright 2010 Elsevier B.V. All rights reserved.)

Background: Lyme neuroborreliosis has not been previously reported in Taiwan. The purpose of this study was to describe the important characteristics of Lyme neuroborreliosis caused by Borrelia burgdorferi-like species in Taiwanese patients., Methods: Patients with acute neurological symptoms without a clear explanation during September 2005 to December 2008 were enrolled. All patients received extensive auxiliary investigations, including clinical evaluation by neurological examination, electrophysiological and neuroimaging studies, serological screening tests of serum and cerebrospinal fluid (CSF) by indirect immunofluorescent assay, and Western immunoblot analysis conducted for seroreactivities in serum and CSF of Borrelia burgdorferi-specific IgM and IgG antibodies., Results: Eleven patients who presented with acute central nervous system (CNS) syndrome fulfilled the diagnostic criteria for neuroborreliosis. In the CSF studies, lymphocytic pleocytosis with elevation of total protein and normal glucose was noted in 9 patients. The clinical syndromes were confirmed by magnetic resonance imaging and electrophysiological studies. Ten of 11 patients were found to have brain stem or spinal cord lesions on neuroimaging studies, and 8 patients had concomitant peripheral nervous system involvement with electrophysiological evidence of multiple root lesions with an acute or subacute course., Conclusions: Lyme neuroborreliosis presenting as acute CNS syndrome with peripheral nervous system involvement simultaneously in the disease course, especially acute myelopolyradiculitis, in Taiwan is different from Lyme neuroborreliosis seen in Europe and North America. The treatment outcome is variable and based on the severity of initial neurological deficits, early diagnosis, and early management., (Copyright (c) 2010 Elsevier B.V. All rights reserved.)

Purpose: Time from stroke onset to treatment (OTT) is potentially an important factor affecting subsequent outcome in patients treated with t-PA. The aim of the study was to assess the correlation between OTT and early recanalization rate after IV-t-PA therapy., Methods: Consecutive stroke patients treated with t-PA within 3h of onset were prospectively studied. Patients with major brain artery occlusion on MRA before t-PA infusion were enrolled. The correlation between OTT and the early recanalization rate within 1h after t-PA infusion was determined., Results: 102 patients (M1 occlusion, 41 patients; M2, 19; ICA, 31; BA, 8; and PCA, 3) were enrolled. Follow-up MRA within 1h after t-PA infusion showed early recanalization in 42 (41.2%) patients (complete in 13 patients, partial in 29). The early recanalization rate was 53.8% with OTT 74), ICA occlusion, baseline NIHSS score (<10), and glucose (>150 mg/dl), adjusted OR for early recanalization of OTT 130-180 min was 2.97 (95% CI 1.27-6.96, P=0.012)., Conclusion: Early recanalization depended on time from stroke onset to IV-t-PA administration. Thus, t-PA should be given to acute stroke patients as soon as possible., (Copyright (c) 2010 Elsevier B.V. All rights reserved.)

Background: Few studies have explored motor imagery (MI) as a way of accessing the higher-level of control of complex body movements involved in gait or balance. The objective of this study was 1) to measure and compare the time needed to complete the Timed Up & Go test (TUG), the time needed to imagine performing the same test (iTUG) and to calculate the time difference between both of these conditions (delta time) in a sample of young and older adults, and 2) to examine whether there was an association between the Timed Up & Go test results (TUG, iTUG, delta time), age and cognitive decline., Methods: A total of 162 subjects (38 healthy young adults, mean age 25.7+/-2.3 years, 73.7% women and 124 older inpatients, mean age 85.3+/-6.5 years, 76.6% women) were included in this cross-sectional study. The mean+/-SD of TUG, iTUG and delta time, age and the Mini Mental State Examination (MMSE) score were used as main outcomes., Results: Age was associated with an increase in time of TUG (P<0.001) and of delta time (P=0.015), and with a decrease in time of iTUG (P<0.001), whereas cognitive decline was only associated with increase in delta time (P=0.030). There was an increase in time of TUG (P<0.001) and in delta time (P<0.001) for subjects who used a walking aid. The increase in delta time depended on the MMSE score when the subjects did not use a walking aid (P for trend=0.001)., Conclusions: iTUG is clinically feasible among frail older adults and may quickly inform any clinician about higher-level changes in control of gait and balance in older adults., (Copyright 2010 Elsevier B.V. All rights reserved.)

Unlabelled: We report a Spanish family affected from a late onset, hand-involved and autosomal dominant distal myopathy associated to Caveolin-3 mutation. Signs of muscle hyperexcitability and hyperckemia were observed in the youngest relatives but not motor symptoms., Patients and Methods: Neurological examination was performed in all members of the family. Muscle biopsy sample was taken from the proband and DNA genomics was amplified for the two exons of Cav-3 by the polymerase chain reaction (PCR) in all the affected members and in three asymptomatic relatives., Results: Signs of muscle hyperexcitability and hyperckemia were observed in the affected members from early ages. Cav-3 expression was greatly reduced in the sarcolemma of the proband's muscle. Genetic studies revealed a G --> A transition at nucleotide position 80 in exon 1 of the Cav-3 gene (c.80G>A), generating a Arg --> Gln change at codon 27 (p.R27Q) of the amino acid chain in heterozygous state, while no mutation was found in unaffected members., Conclusions: Signs of muscle hyperexcitability and hyperckemia at early ages may predict the development of a late onset autosomal dominant hand-involved myopathy associated to Cav-3 mutation in the family reported herein.

Eclampsia is a poorly understood disorder characterized by seizures or unexplained coma in setting of gestational hypertension. Its neurological manifestations are varied and are an important cause of the morbidity and mortality associated. We present a comprehensive prospective study of forty women recruited over four years describing neurological symptoms and signs, neuroimaging and laboratory studies as well as prognosis including 3-6 months follow-up. The seizures occurred in the postpartum period in majority of women (55%), while 45% had seizures before labor, and the rest (5%) during labor. Interestingly, one third of the women suffered their first seizures more than 48 h postpartum (late postpartum eclampsia). A sizable minority suffered more than one seizure and some had documented partial seizures. Headache preceded seizures by more than a day and was described as throbbing or pounding pain by most. The visual symptoms in decreasing frequency were blurring, blindness, scotoma and visual processing deficits. The most common finding during the neurological exam was memory deficits, followed by increased deep tendon reflexes (asymmetric in some), visual perception deficits, visual information processing deficits, altered mental status and cranial nerve deficits. Intracranial or intraspinal pressure when examined was elevated. Among neuroimaging studies, MRI was more sensitive compared to CT scan. The MRI abnormalities included both white as well as gray matter and the most common location of abnormalities was high frontal/parietal lobe. The laboratory studies revealed proteinuria in majority, but not in all. The liver function tests were abnormal in many, while few patients had HELLP syndrome. The neurological deficits resolved by the time of discharge in all. At follow-up, some patients developed new neurological problems such as recurrent headaches or seizures.

Functional outcome has become a key parameter for the determination of the efficacy of therapeutic interventions. Unfortunately, functional tests are not established for filament perforation induced subarachnoid hemorrhage (SAH). Therefore, we evaluated generally applied functional tasks for their potential to discriminate between various degrees of neuronal damage. Rats were subjected to SAH by an endovascular filament and were randomly assigned to controls treated with 0.9% NaCl, moderately neuroprotective therapy with 7.5% NaCl, and highly effective neuroprotection by 7.5% NaCl+6% dextran 70 (HSD). Functional deficit was quantified daily using beam balance task, prehensile traction task, rotarod, a 6-point motor function score and a general neurological 100-point score. Only the HSD group exhibited significantly more surviving neurons at postoperative day 7. Despite significant variations in histomorphometry, beam balance, prehensile traction and rotarod failed to distinguish between groups. On the other hand, the 100-point neuroscore showed improved neurological recovery on postoperative day 1 for HSD. The 100-point neuroscore failed to discriminate between treatment arms at later time points and therefore seems to reflect predominantly early neurological dysfunction. In conclusion, the results of pure motor tasks after experimental SAH in rats should be carefully interpreted. The integration of a test regimen to examine long term cognitive deficits after rat SAH might be valuable to gain additional information about the functional consequences of morphological damage.

An approach combining reperfusion mediated by thrombolytics with pharmacological neuroprotection aimed at inhibiting the physiopathological disorders responsible for ischemia-reperfusion damage, could provide an optimal treatment of ischemic stroke. We investigate, in a rat embolic stroke model, the combination of rtPA with citicoline as compared to either alone as monotherapy, and whether the neuroprotector should be provided before or after thrombolysis to achieve a greater reduction of ischemic brain damage. One hundred and nine rats have been studied: four were sham-operated and the rest embolized in the right internal carotid artery with an autologous clot and divided among 5 groups: 1) control; 2) iv rtPA 5 mg/kg 30 min post-embolization 3) citicoline 250 mg/kg ip x3 doses, 10 min, 24 h and 48 h post-embolization; 4) citicoline combined with rtPA following the same pattern; 5) rtPA combined with citicoline, with a first dose 10 min after thrombolysis. Mortality, neurological score, volume of ischemic lesion and neuronal death (TUNEL) after 72 h and plasma levels of IL-6 and TNF-alpha, were considered to assess ischemic brain damage. Compared with controls, the use of citicoline after thrombolysis produced the greatest reduction of mortality caused by the ischemic lesion (p<0.01), infarct volume (p=0.027), number of TUNEL positive cells in striatum (p=0.014) and plasma levels of TNF-alpha at 3 h (p=0.027) and 72 h (p=0.011). rtPA induced reperfusion provided a slight non-significant reduction of infarct volume and neuronal death, but it reduced mortality due to brain damage (p<0.01) although an increase in the risk of fatal bleeding was noted. CiT as monotherapy only produced a significant reduction of neuronal death in striatum (p=0.014). The combination of CiT before rtPA did not add any benefit to rtPA alone. The superiority of the combined treatment with rtPA followed by citicoline suggests that early reperfusion should be followed by effective neuroprotection to inhibit ischemia-reperfusion injury and better protect the tissue at risk.

Objective: To investigate frequency and associated factors of post polio syndrome (PPS) in an Italian cohort of people with prior poliomyelitis., Methods: We screened subjects admitted for poliomyelitis at the paediatric hospital of the University of Palermo during the time frame 1945-1960. Patients who developed PPS were identified through a structured questionnaire and a neurologic examination. PPS diagnosis was made according to specified diagnostic criteria. Frequency of PPS was calculated in the selected cohort of polio survivors. The association with the investigated risk factors (sex, age at onset of polio, extension and severity of polio, education, associated diseases, cigarette smoking, trauma, polio vaccination) was analysed by the calculation of the odds ratio., Results: Forty-eight participants met the adopted diagnostic criteria for PPS, giving a prevalence of 31.0%. The prevalence rate was significantly higher in women than in men (p=0.02). Logistic regression analyses revealed a significant inverse association with onset of poliomyelitis at over 12 months of age (OR 0.33; CI 0.14-0.79) a higher degree of education (OR 0.20; CI 0.07-0.79), and a significant association with the presence of other diseases (OR 9.86; CI 3.69-26.34)., Conclusions: In our survey one-third of patients with prior poliomyelitis had PPS. Higher age at onset of poliomyelitis is inversely associated with PPS. The association with other diseases may indicate that a chronic physical stress, particularly in already weak motor units, can contribute to the development of signs and symptoms of PPS. Our results also suggest the impact of socio-economic conditions on the risk of PPS.

Genetic linkage studies have provided evidence for a late-onset Alzheimer's disease (AD) susceptibility locus on chromosome 21q. We have tested, in a two-stage association study, whether allelic or haplotype variation of the beta-amyloid cleaving enzyme-2 (BACE2) locus on chromosome 21q affects the risk of late-onset AD. In stage-1, an unselected population-based sample of Finns aged 85 years or over (n=515) was analysed. Neuropathologic examination including beta-amyloid load quantification was possible in over 50% (n=264) of these subjects. AD patients (n=100) and controls (n=48) were defined by modified neuropathological NIA-RI criteria. Positive associations were taken as a hypothesis, and tested in stage-2 using 483 AD families from the USA. Four single nucleotide polymorphisms (SNPs) of BACE2 gene were tested in stage-1. A SNP close to exon-6 was associated with neuropathologically verified AD (p=0.02) and also with beta-amyloid load in non-selected autopsied subjects after conditioning with APOE genotype (p=0.001). In haplotype analysis a specific, relatively common haplotype (H5) was found to associate with AD (p=0.004) and a second haplotype (H7) showed a weaker association with protection against AD (p=0.04). In stage-2, the SNP association was not replicated, whereas the haplotype H5 association was replicated (p=0.004) and a trend to association was found with the putative protective haplotype H7 (two-sided p=0.08). BACE2 haplotype association with AD in two independent datasets provides further evidence for an AD susceptibility locus on chromosome 21q within or close to BACE2.

Objective: A recent nationwide survey of myelitis with atopic diathesis in Japan disclosed that the disease frequently shows a chronic persistent course. A neuropathological study of the spinal cord also revealed chronic active inflammation. Since the effects of various immunotherapies have not been studied extensively in this condition, we evaluated the efficacies of various immunotherapies in patients with myelitis with atopic diathesis., Patients and Methods: Forty-two treatments in 26 patients with myelitis with atopic diathesis were retrospectively analyzed. One of the following therapies was administered: (1) corticosteroids (CS) (pulse therapy followed by oral administration with gradual tapering); (2) intravenous immunoglobulin (IVIG) (400 mg/kg/day for 5 consecutive days); (3) plasma exchanges (PE); or (4) PE followed by IVIG or CS (PE+IVIG/CS). The therapeutic efficacies were evaluated by thorough neurological examination and laboratory tests including MRI, somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs)., Results: Objective neurological findings improved in 89% of the PE group and 90% of the PE+IVIG/CS group, compared with only 72% of the CS and 60% of the IVIG groups. Improvement determined by laboratory tests was seen in 57% of the PE and 57% of the PE+IVIG/CS groups, compared with only 15% of the CS and none of the IVIG groups. Thus, the improvement rate determined by laboratory tests was significantly greater for therapies including PE than for those without PE (p=0.0187)., Conclusions: These data suggest that immunotherapy is effective in myelitis with atopic diathesis despite a chronic persistent course, and that PE is the most beneficial immunotherapy.

Objective: The neurophysiological assessment of intramedullary spinal cord lesions has been unsatisfactory. Previous studies in patients with syringomyelia suggest that testing of cutaneous silent periods (CSPs) may be useful to assess centromedullary lesions., Methods: The authors studied nine patients with intramedullary spinal cord lesions of different etiologies. Eight patients with cervical lesions presented with hypalgesia, hypothermesthesia, or pain in at least one upper extremity; five of them had also upper limb weakness or sensory impairment. One patient with a thoracic lesion had normal upper limb function. The authors recorded CSPs in abductor pollicis brevis muscle following digit II and digit V stimulation. Somatosensory evoked potentials (SEPs) were obtained following median and tibial nerve stimulation. Motor evoked potentials (MEPs) were obtained in biceps brachii, abductor digiti minimi and tibialis anterior muscles following transcranial magnetic or electrical stimulation., Results: CSP abnormalities were found in all patients with cervical lesions, but not in the patient with a thoracic lesion. Cortical median nerve SEPs had normal latencies in all patients, while tibial nerve SEPs, upper limb MEPs, and lower limb MEPs were delayed in five patients each. In one patient, abnormal CSP were the only neurophysiological finding. CSP abnormalities were associated with hypalgesia and hypothermesthesia in 95% of the studies., Conclusion: Upper extremity CSP testing is a sensitive neurophysiological technique for the assessment of cervical intramedullary lesions. In particular, abnormal CSPs are highly associated with spinothalamic dysfunction.