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Sue Leurgans

Publications and source records attributed to Sue Leurgans.

At least 19 recordsLinked to original sources

Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS): Process, format, and clinimetric testing plan.

This article presents the revision process, major innovations, and clinimetric testing program for the Movement Disorder Society (MDS)-sponsored revision of the Unified Parkinson's Disease Rating Scale (UPDRS), known as the MDS-UPDRS. The UPDRS is the most widely used scale for the clinical study of Parkinson's disease (PD). The MDS previously organized a critique of the UPDRS, which cited many strengths, but recommended revision of the scale to accommodate new advances and to resolve problematic areas. An MDS-UPDRS committee prepared the revision using the recommendations of the published critique of the scale. Subcommittees developed new material that was reviewed by the entire committee. A 1-day face-to-face committee meeting was organized to resolve areas of debate and to arrive at a working draft ready for clinimetric testing. The MDS-UPDRS retains the UPDRS structure of four parts with a total summed score, but the parts have been modified to provide a section that integrates nonmotor elements of PD: I, Nonmotor Experiences of Daily Living; II, Motor Experiences of Daily Living; III, Motor Examination; and IV, Motor Complications. All items have five response options with uniform anchors of 0 = normal, 1 = slight, 2 = mild, 3 = moderate, and 4 = severe. Several questions in Part I and all of Part II are written as a patient/caregiver questionnaire, so that the total rater time should remain approximately 30 minutes. Detailed instructions for testing and data acquisition accompany the MDS-UPDRS in order to increase uniform usage. Multiple language editions are planned. A three-part clinimetric program will provide testing of reliability, validity, and responsiveness to interventions. Although the MDS-UPDRS will not be published until it has successfully passed clinimetric testing, explanation of the process, key changes, and clinimetric programs allow clinicians and researchers to understand and participate in the revision process.

Disability Evaluation↗

Hippocampal disconnection contributes to memory dysfunction in individuals at risk for Alzheimer's disease.

The concept of amnestic mild cognitive impairment (MCI) describes older people who show a decline predominantly in memory function, but who do not meet criteria for dementia. Because such individuals are at high risk for developing Alzheimer's disease, they are of great interest for understanding the prodromal stages of the disease process. The mechanism underlying memory dysfunction in people with MCI is not fully understood. The present study uses quantitative, high-resolution structural MRI techniques to investigate, in vivo, the anatomical substrate of memory dysfunction associated with MCI. Changes in brain structures were assessed with two imaging techniques: (i) whole-brain, voxel-based morphometry to determine regions of reduced white matter volume and (ii) sensitive volumetric segmentation of the entorhinal cortex and hippocampus, gray matter regions that are critically important for memory function. In participants with amnestic MCI, compared with age-matched controls, results showed a significant decrease in white matter volume in the region of the parahippocampal gyrus that includes the perforant path. There was also significant atrophy in both the entorhinal cortex and the hippocampus. Regression models demonstrated that both hippocampal volume and parahippocampal white matter volume were significant predictors of declarative memory performance. These results suggest that, in addition to hippocampal atrophy, disruption of parahippocampal white matter fibers contributes to memory decline in elderly individuals with MCI by partially disconnecting the hippocampus from incoming sensory information.

Aged↗

The malignant course of "benign hallucinations" in Parkinson disease.

OBJECTIVE: To monitor progression of "benign hallucinations" in Parkinson disease (PD). METHODS: We searched our data repository for subjects with PD with 3 sets of neuropsychological testing during 3 years and Unified Parkinson's Disease Rating Scale thought disorder scores taken at 4- to 12-month intervals during this period. We found 48 patients with benign hallucinations, defined as a thought disorder score of 2 (benign hallucinations, insight retained), receiving no treatment for hallucinations. We followed up thought disorder scores under best medical management for at least 3 years or until a thought disorder score of 3 (loss of insight) or 4 (delusions) occurred. In subjects whose thought disorder scores remained at 2, we assessed neuroleptic use and decreases in PD medications to abate hallucinations. RESULTS: Most subjects (81%) progressed to thought disorder scores of 3 or 4. In 7 (78%) of 9 subjects who retained a thought disorder score of 2, PD medications were reduced to treat hallucinations, and 3 subjects (33%) also received neuroleptics. If the composite end point (any of the criteria) was used, 96% of subjects progressed, with only 2 subjects continuing with stable, untreated benign hallucinations. CONCLUSIONS: Because hallucinations progress, the concept of benign hallucinations is prognostically misleading. Though hallucinations with retained insight may be "benign" for the moment, they portend serious consequences. The term benign hallucinations of PD should be considered generally unsound and dropped from operative vocabulary.

Aged↗

Gilles de la Tourette syndrome: patient's knowledge and concern of adverse effects.

The objective of this study was to assess awareness and concern of neuroleptic (NL)-induced side effects in Gilles de la Tourette syndrome (GTS) patients. Although NLs are effective tic suppressants, they can be associated with various side effects. Data on patient knowledge and concern about side effects can guide educational efforts. One hundred consecutive GTS patients or parents in a tertiary referral medical center responded to a standardized, in-person questionnaire. They were given a list of 15 side effects and asked which could be ascribed to NLs (9) or not (6). Side effect concern was rated on a 0 (none) to 10 (extreme) scale. The mean age for the 100 patients was 19.4+/-14 years; 55 had a history of NL use, and 45 were NL-naive. Less than half the cohort met criteria for being well informed. Only one third of the listed NL side effects were accurately identified by at least 75% of the respondents. Patients with past or current NL treatment were more accurate in identifying NL side effects but less concerned about them than NL-naive patients. The side effects of greatest concern were seizures, tardive dyskinesia, thinking and emotion disturbances, and cardiac irregularities. Overall, patient awareness of NL side effects is insufficient, and although past exposure to NLs enhances knowledge, it decreases concern.

Adolescent↗

Age-related influences on the clinical characteristics of new-onset hallucinations in Parkinson's disease patients.

The objective of this study was to determine the demographic influences on the sensory characteristics (pure visual vs. nonvisual or mixed visual/nonvisual) of new-onset hallucinations in Parkinson's disease (PD). We utilized 6-year longitudinal interview data from 60 PD patients who had never hallucinated at baseline and reinterviewed them at 6, 18, 48, and 72 months to assess the presence and type of hallucination that developed as the first form of hallucination. We analyzed data by Generalized Estimating Equations methods and by nonparametric tests. Over 6 years, 37 of 60 patients developed hallucinations, and the first hallucinations were pure visual in 18, pure nonvisual in 9, and mixed visual/nonvisual in 10. At the time of first onset of hallucinations, patients whose hallucinations were nonvisual or mixed were significantly older than those with purely visual hallucinations (mean age, 69.8+/-8.3 vs. 61.9+/-10.6; P=0.031). PD duration in the two groups, however, was statistically comparable (9.6+/-4.4 vs. 12.9+/-8.6 years). Though classically described as visual, hallucinations in PD frequently involve other sensory modalities. Age-related disinhibition may facilitate wider cortical activation in PD and potentiate aberrant signaling that invokes other types of hallucinations besides the classic visual forms.

Age Factors↗

Does seasonal variation affect hallucinations in PD? A longitudinal study.

The objective of this study was to examine the chronobiology of visual hallucinations in Parkinson's disease (PD) patients to test whether hallucinations are more severe during darker months (winter) than summer months. We extracted longitudinal data on the UPDRS Thought Disorder (TD) score, taken as part of regular patient management on sequential visits through a 1-year cycle in hallucinating PD patients. To reduce the confounding effects of medication adjustments, analysis was restricted to patients with a baseline evaluation that did not involve a medication change. The primary outcome was the seasonal change in hallucination severity, defined as a change in TD score or neuroleptic use. Using Wilcoxon rank-sum tests, we compared the exacerbation of hallucination by season, focusing on winter (December through February) versus summer (June through August). A total of 51 hallucinating patients met entry criteria, and their longitudinal data were analyzed. There was no difference in hallucination severity with changing season; the level of wintertime exacerbation was no greater than summertime exacerbation (P = 0.42). Although hallucinations frequently develop in dimmed environments and evening hours, the darkness of wintertime does not exacerbate hallucinations in PD subjects on stable medications. Because we found no evidence of seasonal variations, we cannot recommend phototherapy or other strategies affecting chronobiological systems as research priorities to treat PD hallucinations. (c) 2006 Movement Disorder Society.

Darkness↗

Effect of CX516, an AMPA-modulating compound, on cognition and behavior in fragile X syndrome: a controlled trial.

A Phase II, 4-week randomized, double-blind, placebo-controlled clinical trial was conducted to evaluate the safety and efficacy of the Ampakine compound CX516 as a potential treatment for the underlying disorder in fragile X syndrome (FXS). After baseline screening, subjects with FXS (n = 49) underwent a 1-week placebo lead-in and then were randomized to study drug or placebo for a 4-week period. Cognitive and behavioral outcome measures were administered prior to treatment, at the end of treatment, and 2 weeks posttreatment. There were minimal side effects, no significant changes in safety parameters, and no serious adverse events. There was a 12.5% frequency of allergic rash in the CX516 group and 1 subject developed a substantial rash. There was also no significant improvement in memory, the primary outcome measure, or in secondary measures of language, attention/executive function, behavior, and overall functioning in CX516-treated subjects compared to placebo. This study did demonstrate that many outcome measures were reproducible in this test-retest setting for the FXS population, yet some were too difficult or variable. Adult subjects with FXS were able to complete an intensive clinical trial, and some valid outcome measures were identified for future FXS trial design. Problems with potency of CX516 in other studies have suggested dosing may have been inadequate for therapeutic effect and thus it remains unclear whether modulation of AMPA-mediated neurotransmission is a viable therapeutic strategy for the treatment of FXS.

Adolescent↗

White matter changes in mild cognitive impairment and AD: A diffusion tensor imaging study.

Diffusion tensor imaging (DTI) can detect, in vivo, the directionality of molecular diffusion and estimate the microstructural integrity of white matter (WM) tracts. In this study, we examined WM changes in patients with Alzheimer's disease (AD) and in subjects with amnestic mild cognitive impairment (MCI) who are at greater risk for developing AD. A DTI index of WM integrity, fractional anisotropy (FA), was calculated in 14 patients with probable mild AD, 14 participants with MCI and 21 elderly healthy controls (NC). Voxel-by-voxel comparisons showed significant regional reductions of FA in participants with MCI and AD compared to controls in multiple posterior white matter regions. Moreover, there was substantial overlap of locations of regional decrease in FA in the MCI and AD groups. These data demonstrate that white matter changes occur in MCI, prior to the development of dementia.

Aged↗

Hallucinations and sleep disorders in PD: six-year prospective longitudinal study.

OBJECTIVE: To prospectively assess the relationship of hallucinations to sleep disorders in patients with Parkinson disease (PD) over 6 years. BACKGROUND: Several studies suggest an association between hallucinations and sleep disruption, but no longitudinal study has examined their progression and relationship, nor whether sleep alterations predict future hallucinations. METHODS: Eighty-nine PD patients were recruited to fill cells of normal sleep without hallucinations (n = 20); sleep fragmentation only (n = 20); vivid dreams/nightmares (n = 20); hallucinations with insight (n = 20); hallucinations without insight (n = 9). At baseline, 6 months, 18 months, 4 years, and 6 years, sleep disorders and hallucinations were assessed by standardized scales with the longitudinal data analyzed by generalized estimating equations and other nonparametric tests. RESULTS: At 6 years, we could account for all subjects (49 interviewed, 40 deceased or too ill for interview). Hallucination prevalence and severity increased over time (p < 0.0001). The odds of being a hallucinator increased by a factor of 1.39 at each successive time point. Sleep disorders, however, fluctuated widely among patients and time points, with no evidence of progression in severity (p = 0.73). The prevalence of sleep fragmentation did not differ between subjects with vs without hallucinations (OR = 1.43, p = 0.13). The presence of vivid dreams/nightmares, however, was highly correlated with the concurrent presence (OR = 2.32) and severity of hallucinations (OR = 3.02, both p < 0.0001). Vivid dreams/nightmares among non-hallucinators did not predict future development of hallucinations (OR = 0.94, p = 0.51). CONCLUSIONS: Hallucinations and global sleep disorders follow different patterns of progression in Parkinson disease and are separate behavioral abnormalities. Sleep alterations are not necessarily harbingers of hallucinations.

Aged↗

Transcultural comparison of psychogenic movement disorders.

Prompted by the lack of cross-cultural comparative data, and because a better understanding in the different clinical presentations of psychogenic movement disorders (PMDs) is relevant to neurological assessment and interventions, we compared the phenomenology, anatomical distribution, and functional impairment of PMDs in the United States and Spain. Consecutive patients diagnosed with PMD by a movement disorder specialist from one US site and from eight Spanish university centers were included in the study. The two groups were similar in their movement types, anatomical distribution, and functional impairment. PMDs were more prevalent in women than in men and were most common in upper and lower extremities. Gait and speech dysfunctions were distributed similarly in both countries. We found action tremor to be the most frequent PMD in both countries.

Adult↗

Rating scale for psychogenic movement disorders: scale development and clinimetric testing.

We developed and tested the clinimetric properties of a scale for psychogenic movement disorders (PMDs). PMDs are disabling but lack any generally accepted treatment strategies. To develop treatments, means of assessing disease severity must be provided. No scale to assess PMDs existed. The PMD scale developed here rates 10 phenomena (rest tremor, action tremor, dystonia, chorea, bradykinesia, myoclonus, tics, athetosis, ballism, cerebellar incoordination), 2 functions (gait, speech), and 14 body regions. To study interrater agreement, three movement disorder neurologists independently rated 88 videotapes of PMD patients. Data analysis was performed using a kappa coefficient of agreement, Kendall's coefficient of concordance, Spearman correlations, and intraclass correlation coefficients. Validity and scale responsiveness were tested as well. All phenomena and speech and gait dysfunction occurred in the patient sample. A wide range of affected body regions, severity, and incapacitation was captured. Ratings showed excellent interrater reliability for presence or absence of each phenomenon (kappa range, 0.63 to 0.86). Kendall's concordance coefficients for phenomenology, function, and total PMD scores were 0.92, 0.93, and 0.91. Spearman correlations between raters ranged from 0.86 to 0.90. The scale was responsive to changes that occurred as a result of a neuropsychiatric intervention. The PMD scale adequately captures the complex movements of PMDs and can be used to assess PMDs and test the efficacy of intervention strategies.

Adolescent↗

Effects of estrogen replacement therapy on cholinergic basal forebrain neurons and cortical cholinergic innervation in young and aged ovariectomized rhesus monkeys.

Estrogen modulates the function of cholinergic basal forebrain neurons in aged female rats. The present study tested the hypothesis that estrogen enhances the phenotype of cholinergic basal forebrain neurons and their cortical cholinergic innervation in young adult and aged ovariectomized rhesus monkeys. Sixteen monkeys (9 young and 7 aged) received two injections of estradiol cypionate or vehicle separated by 3 weeks. All monkeys were killed 1 day after the last injection. Quantitative immunofluorescence in the vertical limb of the diagonal band (VLDB) revealed enhanced optical density for choline acetyltransferase (ChAT) in both young and aged monkeys treated with estrogen. In contrast, optical density for low-affinity p75 neurotrophin receptor immunoreactivity in the VLDB did not change after estrogen treatment in either aged or young animals. Quantitative immunofluorescence for either ChAT or the low-affinity p75 neurotrophin receptor in the nucleus basalis Meynert failed to reveal differences between vehicle and estrogen treatment in either age group. Quantitative estimates of acetylcholinesterase (AChE) fiber density revealed that estrogen-treated aged monkeys but not their younger counterparts had decreased numbers of AChE-positive fibers in layer II of frontal, insular, and cingulate cortices. These data indicate that estrogen administered in a manner simulating natural hormonal cyclicity produces modest age-specific chemical phenotypic and regional changes in select neuronal subfields of the cholinergic basal forebrain and their cortical projection sites in nonhuman primates.

Aging↗

Genetic polymorphisms in Parkinson disease subjects with and without hallucinations: an analysis of the cholecystokinin system.

BACKGROUND: Hallucinations in patients with Parkinson disease (PD), occurring in about one third of those receiving long-term dopaminergic therapy, contribute to morbidity and mortality. In matched Chinese PD subjects with and without hallucinations, the presence of the -45 C/T locus in the cholecystokinin (CCK) gene, particularly when combined with the CCK receptor, CCKAR (cholecystokinin A receptor), C polymorphism, was associated with increased hallucination risk. Because CCK gene polymorphisms vary across ethnic groups, the presence of similar associations in white PD subjects merits investigation. OBJECTIVE: To determine whether polymorphisms of CCK and CCK receptor genes are associated with hallucinations in white PD subjects. DESIGN: Case-control study of PD subjects with and without chronic hallucinations matched for age and dopaminergic medication. Genomic DNA was analyzed for CCK, CCKAR, and CCKBR (cholecystokinin B receptor) polymorphisms by polymerase chain reaction. Genotype distributions and allele frequencies were compared between groups and in matched pairs. RESULTS: Comparing matched pairs, we found more frequent representation of the CCK T allele in hallucinating PD subjects, although this finding was not statistically significant (P =.06). Of 5 cases with both CCK T and CCKAR C alleles, 4 were hallucinators. Cases and controls did not differ in CCKAR or CCKBR polymorphisms. CONCLUSIONS: Our study supports a previous association of hallucinations in PD subjects with the CCK T allele and the combined CCK T and CCKAR C allele, suggesting that the CCK system may influence the development of hallucinations in PD subjects. The lower representation of the T allele in our white sample limited our statistical power. Further assessment of the T allele as a risk factor for hallucinations would include longitudinal study of nonhallucinators to detect the evolution of hallucinations relative to T allele frequency.

Aged↗

Short-term and practice effects of metronome pacing in Parkinson's disease patients with gait freezing while in the 'on' state: randomized single blind evaluation.

OBJECTIVE: In a randomized single blind parallel study, we tested the efficacy of an auditory metronome on walking speed and freezing in Parkinson's disease (PD) patients with freezing gait impairment during their 'on' function. BACKGROUND: No pharmacological treatment is effective in managing 'on' freezing in PD. Like visual cues that can help overcome freezing, rhythmic auditory pacing may provide cues that help normalize walking pace and overcome freezing. DESIGN/METHODS: Non-demented PD patients with freezing during their 'on' state walked under two conditions, in randomized order: unassisted walking and walking with the use of an audiocassette with a metronome recording. The walking trials were randomized and gait variables were rated from videotapes by a blinded evaluator. Outcome measures were total walking time (total trial time-total freezing time), which was considered the time over a course of specified length, freezing time, average freeze duration and number of freezes. All outcomes were averaged across trials for each person and then compared across conditions using Signed Rank tests. RESULTS: Twelve non-demented PD patients with a mean age of 65.8 +/- 11.2 years, and mean PD duration of 12.4 +/- 7.3 years were included. The use of the metronome slowed ambulation and increased the total walking time (P < 0.0005) only during the first visit, without affecting any freezing variable. In the nine patients who took the metronome recording home and used it daily for 1 week while walking, freezing remained unimproved. CONCLUSIONS: Though advocated in prior publications as a walking aid for PD patients, auditory metronome pacing slows walking and is not a beneficial intervention for freezing during their 'on' periods.

Acoustic Stimulation↗

Aspirin and ticlopidine for prevention of recurrent stroke in black patients: a randomized trial.

CONTEXT: Blacks are disproportionately affected by stroke, and they are about 2 times more likely than most other individuals in the United States to die of or experience stroke. OBJECTIVE: To determine the efficacy and safety of aspirin and ticlopidine to prevent recurrent stroke in black patients. DESIGN, SETTING, AND PATIENTS: Randomized, double-blind, investigator-initiated, multicenter trial of 1809 black men and women who recently had a noncardioembolic ischemic stroke and who were recruited between December 1992 and October 2001 from 62 academic and community hospitals in the United States and followed up for up to 2 years. INTERVENTION: A total of 902 patients received 500 mg/d of ticlopidine and 907 received 650 mg/d of aspirin. MAIN OUTCOME MEASURES: Recurrent stroke, myocardial infarction, or vascular death was the composite primary end point (according to intention-to-treat analysis). The secondary outcome was fatal or nonfatal stroke. RESULTS: The blinded phase of the study was halted after about 6.5 years when futility analyses revealed a less than 1% probability of ticlopidine being shown superior to aspirin in the prevention of the primary outcome end point. The primary outcome of recurrent stroke, myocardial infarction, or vascular death was reached by 133 (14.7%) of 902 patients assigned to ticlopidine and 112 (12.3%) of 907 patients assigned to aspirin (hazard ratio, 1.22; 95% confidence interval, 0.94-1.57). Kaplan-Meier curves for time to event for the primary outcome did not differ significantly (P =.12 by log-rank test). Kaplan-Meier curves for time to the secondary outcome of fatal or nonfatal stroke approached a statistically significant reduction favoring aspirin over ticlopidine (P =.08 by log-rank test). The frequency of laboratory-determined serious neutropenia was 3.4% for patients receiving ticlopdine vs 2.2% for patients receiving aspirin (P =.12) and 0.3% vs 0.2% for thrombocytopenia, respectively (P =.69). One ticlopidine-treated patient developed thrombocytopenia, which was thought to be a case of possible thrombotic thrombocytopenia purpura, and recovered after therapy with plasmapheresis. CONCLUSIONS: During a 2-year follow-up, we found no statistically significant difference between ticlopidine and aspirin in the prevention of recurrent stroke, myocardial infarction, or vascular death. However, there was a nonsignificant trend for reduction of fatal or nonfatal stroke among those in the aspirin group. Based on these data and the risk of serious adverse events with ticlopidine, we regard aspirin as a better treatment for aspirin-tolerant black patients with noncardioembolic ischemic stroke.

Black or African American↗

Progression of gait, speech and swallowing deficits in progressive supranuclear palsy.

OBJECTIVE: To identify outcome measures for clinical trials in progressive supranuclear palsy (PSP), the authors determined the time to key motor impairments in a well-defined patient cohort. METHODS: The records of consecutive patients with probable PSP, defined by National Institute of Neurological Disorders and Stroke-Society for Progressive Supranuclear Palsy workshop criteria on first visit to a tertiary care center, were selected for study if the patients were seen regularly at 3- to 6-month intervals, had at least 24 months of care at the Rush movement disorder center, and were originally seen in the movement disorder center before any of the following key motor impairments developed: unintelligible speech, no independent walking, inability to stand unassisted, wheelchair-bound, or recommendation for feeding tube placement. Using standardized criteria from the Unified Parkinson's Disease Rating Scale and Hoehn and Yahr stages taken at each clinic visit, the authors recorded the time that each patient reached the key motor impairments and death. Median months from first symptom onset and from first consultation at the movement disorder center to each motor impairment were derived from Kaplan-Meier curves. RESULTS: Fifty subjects, mean age at study entry 64.2 years, were followed to death (n = 21, mean duration of surveillance 53.6 months) or in ongoing fashion (n = 29, mean duration of surveillance 46.2 months). Eighty-eight percent of the sample met at least one milestone. The median time from disease onset to the first key motor impairment was 48 months, 24 months after first consultation. The three gait items occurred temporally close, and the authors considered them thereafter as a single milestone, occurring at a median disease duration of 57 months, 34 months after first consultation. Unintelligible speech occurred at a median disease duration of 71 months, median 44 months after first consultation. As a composite end point, speech/gait accounted for 98% of the sample's first key motor impairment. Need for nasogastric tube was rarely the first milestone (8%). CONCLUSIONS: Gait impairment and unintelligible speech are milestones that occur rapidly in PSP and can be monitored with standardized rating scales. The authors suggest that clinical trials use these indices to assess how interventions alter anticipated progression of clinical deterioration in PSP.

Adult↗

Preservation of brain nerve growth factor in mild cognitive impairment and Alzheimer disease.

BACKGROUND: The status of nerve growth factor (NGF) levels during the prodromal phase of Alzheimer disease (AD), characterized by mild cognitive impairment (MCI), remains unknown. OBJECTIVE: To investigate whether cortical and/or hippocampal NGF levels are altered in subjects with MCI or different levels of AD severity. DESIGN AND MAIN OUTCOME MEASURES: An NGF enzyme-linked immunosorbent assay determined protein levels in the hippocampus and 5 cortical areas in people clinically diagnosed as having no cognitive impairment, MCI, mild AD, or severe AD. SETTING AND PATIENTS: Subjects were from the Rush Religious Orders Study and the University of Pittsburgh Alzheimer's Disease Research Center (Pittsburgh, Pa). RESULTS: We found no changes in cortical or hippocampal NGF levels across groups; in MCI, levels did not correlate with an increase in choline acetyltransferase activity in these regions. CONCLUSION: Brain NGF levels appear sufficient to support the cholinergic plasticity changes seen in MCI and remain stable throughout the disease course.

Aged↗

Rating scales for dystonia: a multicenter assessment.

The evaluation of dystonia requires a reliable rating scale. The widely used Fahn-Marsden Scale (F-M) has not been sufficiently tested across multiple centers and investigators. The Dystonia Study Group developed the Unified Dystonia Rating Scale (UDRS) and a Global Dystonia Rating Scale (GDS) to serve as instruments to assess dystonia severity. In this study, 25 dystonia experts evaluated the UDRS, F-M, and GDS for internal consistency and reliability. One hundred dystonia patients were videotaped using a standardized videotape protocol. Each examiner rated 20 patients using the UDRS, F-M, and GDS in random order. The examiner then assessed each scale for ease of use. Statistical analysis used Cronbach's alpha, intraclass correlation coefficients (ICC), generalized weighted kappa statistic, and Kendall's coefficient of concordance. The UDRS, F-M, and GDS showed excellent internal consistency (Cronbach's alpha 0.89-0.93) and good to excellent correlation among the raters (ICC range from 0.71-0.78). Inter-rater agreement was fair to excellent (Kendall's 0.54-0.87; kappa 0.37-0.91) being lowest for eyes, jaw, face, and larynx. The modifying ratings (Duration in the UDRS and Provoking Factor in the F-M) showed less agreement than the motor severity ratings. Among scales, the total scores correlated (Pearson's r, 0.977-0.983). Overall, 74% of raters found the GDS the easiest to apply. The GDS with its simplicity and ease of application may be the most useful dystonia rating scale.

Dystonia↗