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Frontiers in neurology. 01/2013; 4:14.
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Alfonso Fasano,
Anabela Valadas,
Kailash P Bhatia,
L K Prashanth,
Anthony E Lang,
Renato P Munhoz,
Francesca Morgante,
Daniel Tarsy,
Andrew P Duker,
Paolo Girlanda,
Anna Rita Bentivoglio, Alberto J Espay
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ABSTRACT: The facial phenotype of psychogenic movement disorders has not been fully characterized. Seven tertiary-referral movement disorders centers using a standardized data collection on a computerized database performed a retrospective chart review of psychogenic movement disorders involving the face. Patients with organic forms of facial dystonia or any medical or neurological disorder known to affect facial muscles were excluded. Sixty-one patients fulfilled the inclusion criteria for psychogenic facial movement disorders (91.8% females; age: 37.0 ± 11.3 years). Phasic or tonic muscular spasms resembling dystonia were documented in all patients most commonly involving the lips (60.7%), followed by eyelids (50.8%), perinasal region (16.4%), and forehead (9.8%). The most common pattern consisted of tonic, sustained, lateral, and/or downward protrusion of one side of the lower lip with ipsilateral jaw deviation (84.3%). Ipsi- or contralateral blepharospasm and excessive platysma contraction occurred in isolation or combined with fixed lip dystonia (60.7%). Spasms were reported as painful in 24.6% of cases. Symptom onset was abrupt in most cases (80.3%), with at least 1 precipitating psychological stress or trauma identified in 57.4%. Associated body regions involved included upper limbs (29.5%), neck (16.4%), lower limbs (16.4%), and trunk (4.9%). There were fluctuations in severity and spontaneous exacerbations and remissions (60%). Prevalent comorbidities included depression (38.0%) and tension headache (26.4%). Fixed jaw and/or lip deviation is a characteristic pattern of psychogenic facial movement disorders, occurring in isolation or in combination with other psychogenic movement disorders or other psychogenic features. © 2012 Movement Disorder Society.
Movement Disorders 10/2012; 27(12):1544-51. · 4.51 Impact Factor
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ABSTRACT: BACKGROUND: A mean of 10 years elapse before patients with Parkinson's disease (PD) reach Hoehn & Yahr (H&Y) stage 4, and 14 years for stage 5. A small proportion of PD patients survive and are ambulatory for ≥20 years. We sought to identify features associated with long-duration PD (dPD). METHODS: This five-center, case-control study compared 136 PD patients with ≥20 years of duration and H&Y stage ≤4 (dPD) to 134 H&Y-, age- and gender-matched PD patients between 10 and 15 years of disease (cPD). RESULTS: By study design, there were no between-group differences in age, gender and H&Y. dPD subjects were younger at onset (p < 0.0001), had more psychosis (p: 0.038), were receiving higher levodopa equivalent daily doses (p: 0.02), were predominantly left-handed (p: 0.048), and had greater frequency of left-sided onset (p: 0.015) compared to cPD subjects. Both groups had similar rates of resting tremor, dementia and REM sleep behavior disorder. CONCLUSIONS: Early disease onset, left-handedness and left-sided onset are associated with long disease and ambulatory PD survival. The neurobiological basis of the prognostic value of lateralization deserves further investigation.
Parkinsonism & Related Disorders 08/2012; · 3.80 Impact Factor
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ABSTRACT: Movement disorders are one of the most rapidly growing fields of neurology from both the clinical and neurobiological perspectives. Despite many recent advances in genetic, pathogenetic, clinical and therapeutic fields, several shortcomings remain in the diagnostic and therapeutic realms, with a plethora of challenges threatening further advances. Aimed at recognizing and bridging these knowledge gaps, the 2nd International Conference on Knowledge Gaps in Parkinson's Disease and Other Movement Disorders was held last February in Santa Margherita Ligure, Italy, with the support of the Movement Disorder Society and the Italian Association for Parkinson's Disease and Extrapyramidal Disorders. The 3-day symposium, which was attended by approximately 300 clinicians and researchers from around the world, gathered around 27 leading young and senior clinical scientists, each of whom addressed the field's main knowledge gaps and brainstormed on how to bridge or minimize their impact. This meeting report summarizes the topics that gathered the most attention from the speakers and the audience.
Expert Review of Neurotherapeutics 07/2012; 12(7):781-4.
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ABSTRACT: The reliability and applicability of published diagnostic criteria for psychogenic movement disorders (PMDs) have never been examined.
Eight movement disorder and six general neurologists rated 14 patients diagnosed with PMD and 14 patients diagnosed with organic movement disorders. Raters provided a dichotomous judgment (i.e., psychogenic or organic) upon review of video-based movement phenomenology and a category of diagnostic certainty based on the Fahn-Williams and Shill-Gerber criteria after accessing standardized clinical information. We measured interobserver agreement on the diagnosis and clinical certainty judgment of PMD.
In both groups of raters, agreements were "fair" on the video-based dichotomous judgment, but improved to "substantial" after access to standardized clinical information. "Slight" to "poor" agreement was reached for the "probable" and "possible" categories of diagnostic certainty corresponding to both diagnostic criteria.
Diagnosis according to clinical available criteria for PMD yields poor diagnostic agreement.
Movement Disorders 04/2012; 27(4):548-52. · 4.51 Impact Factor
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Expert Review of Neurotherapeutics 02/2012; 12(2):111-3.
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ABSTRACT: Patients with Parkinson's disease (PD) receive therapies aimed at addressing a diverse range of motor symptoms. Motor complications in the form of symptom fluctuations and dyskinesias that commonly occur with chronic PD medication use may not be effectively captured by Unified Parkinson's Disease Rating Scale (UPDRS) assessments performed in the clinic. Therefore, home monitoring may be a viable adjunct tool to provide insight into PD motor symptom response to treatment. In this pilot study, we sought to evaluate the feasibility of capturing PD motor symptoms at home using a computer-based assessment system. Ten subjects diagnosed with idiopathic PD used the system at home and ten non-PD control subjects used the system in a laboratory. The Kinesia system consists of a wireless finger-worn motion sensor and a laptop computer with software for automated tremor and bradykinesia severity score assessments. Data from control subjects were used to develop compliance algorithms for rejecting motor tasks performed incorrectly. These algorithms were then applied to data collected from the PD subjects who used the Kinesia system at home to complete motor exams 3-6 times per day over 3-6 days. Motor tasks not rejected by the compliance algorithms were further processed for symptom severity. PD subjects successfully completed motor assessments at home, with approximately 97% of all motor task data files (1222/1260) accepted. These findings suggest that objective home monitoring of PD motor fluctuations is feasible.
Journal of neuroscience methods 01/2012; 203(1):152-6. · 2.30 Impact Factor
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Alberto J Espay,
Joe P Giuffrida,
Robert Chen,
Megan Payne,
Filomena Mazzella,
Emily Dunn,
Jennifer E Vaughan,
Andrew P Duker,
Alok Sahay,
Sang Jin Kim,
Fredy J Revilla,
Dustin A Heldman
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ABSTRACT: Although movement impairment in Parkinson's disease includes slowness (bradykinesia), decreased amplitude (hypokinesia), and dysrhythmia, clinicians are instructed to rate them in a combined 0-4 severity scale using the Unified Parkinson's Disease Rating Scale motor subscale. The objective was to evaluate whether bradykinesia, hypokinesia, and dysrhythmia are associated with differential motor impairment and response to dopaminergic medications in patients with Parkinson's disease. Eighty five Parkinson's disease patients performed finger-tapping (item 23), hand-grasping (item 24), and pronation-supination (item 25) tasks OFF and ON medication while wearing motion sensors on the most affected hand. Speed, amplitude, and rhythm were rated using the Modified Bradykinesia Rating Scale. Quantitative variables representing speed (root mean square angular velocity), amplitude (excursion angle), and rhythm (coefficient of variation) were extracted from kinematic data. Fatigue was measured as decrements in speed and amplitude during the last 5 seconds compared with the first 5 seconds of movement. Amplitude impairments were worse and more prevalent than speed or rhythm impairments across all tasks (P < .001); however, in the ON state, speed scores improved exclusively by clinical (P < 10(-6) ) and predominantly by quantitative (P < .05) measures. Motor scores from OFF to ON improved in subjects who were strictly bradykinetic (P < .01) and both bradykinetic and hypokinetic (P < 10(-6) ), but not in those strictly hypokinetic. Fatigue in speed and amplitude was not improved by medication. Hypokinesia is more prevalent than bradykinesia, but dopaminergic medications predominantly improve the latter. Parkinson's disease patients may show different degrees of impairment in these movement components, which deserve separate measurement in research studies. © 2011 Movement Disorder Society.
Movement Disorders 09/2011; 26(14):2504-8. · 4.51 Impact Factor
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ABSTRACT: Frontotemporal dementia is commonly associated with parkinsonism in several sporadic (i.e., progressive supranuclear palsy, corticobasal degeneration) and familial neurodegenerative disorders (i.e., frontotemporal dementia associated with parkinsonism and MAPT or progranulin mutations in chromosome 17). The clinical diagnosis of these disorders may be challenging in view of overlapping clinical features, particularly in speech, language, and behavior. The motor and cognitive phenotypes can be viewed within a spectrum of clinical, pathologic, and genetic disorders with no discrete clinicopathologic correlations but rather lying within a dementia-parkinsonism continuum. Neuroimaging and cerebrospinal fluid analysis can be helpful, but the poor specificity of clinical and imaging features has enormously challenged the development of biological markers that could differentiate these disorders premortem. This gap is critical to bridge in order to allow testing of novel biological therapies that may slow the progression of these proteinopathies.
Journal of Molecular Neuroscience 09/2011; 45(3):343-9. · 2.50 Impact Factor
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Neurology 08/2011; 77(5):508-9. · 8.31 Impact Factor
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ABSTRACT: To report the rare but distinct clinical and neuropathological phenotype of non-familial, rapidly progressive parkinsonism and dementia associated with frontotemporal lobar degeneration with motor neuron disease (FTLD-MND).
Subjects included two 70-year-old women presenting with rapidly progressive severe postural instability, axial-predominant parkinsonism, oculomotor dysfunction and frontal-predominant dementia with language impairment and pseudobulbar palsy. One had diffuse weakness without signs of lower motor neuron disease. Post-mortem evaluations included immunohistochemistry with antiphospho-TAR DNA-binding protein 43 (TDP-43) and genetic analysis of the TARDBP and PGRN genes.
Subjects died within 14 months from symptom onset. TDP-43-positive neuronal intracytoplasmic inclusions were prominent in the primary motor cortex, granule cell layer of the hippocampus, and several cranial and spinal cord nuclei. TDP-43 globular glial inclusions (GGI) were identified in one case. There were no mutations in PGRN or TARDBP genes.
FTLD-MND due to TDP-43-proteinopathy should be considered in patients with rapidly progressive parkinsonism and dementia phenotype, especially when aphasia and/or weakness are also present.
Journal of neurology, neurosurgery, and psychiatry 07/2011; 82(7):751-3. · 4.87 Impact Factor
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Movement Disorders 06/2011; 26(7):1371. · 4.51 Impact Factor
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ABSTRACT: Bradykinesia encompasses slowness, decreased movement amplitude, and dysrhythmia. Unified Parkinson's Disease Rating Scale-based bradykinesia-related items require that clinicians condense abnormalities in speed, amplitude, fatiguing, hesitations, and arrests into a single score. The objective of this study was to evaluate the reliability of a modified bradykinesia rating scale, which separately assesses speed, amplitude, and rhythm and its correlation with kinematic measures from motion sensors. Fifty patients with Parkinson's disease performed Unified Parkinson's Disease Rating Scale-directed finger tapping, hand grasping, and pronation-supination while wearing motion sensors. Videos were rated blindly and independently by 4 clinicians. The modified bradykinesia rating scale and Unified Parkinson's Disease Rating Scale demonstrated similar inter- and intrarater reliability. Raters placed greater weight on amplitude than on speed or rhythm when assigning a Unified Parkinson's Disease Rating Scale score. Modified bradykinesia rating scale scores for speed, amplitude, and rhythm correlated highly with quantitative kinematic variables. The modified bradykinesia rating scale separately captures bradykinesia components with interrater and intrarater reliability similar to that of the Unified Parkinson's Disease Rating Scale. Kinematic sensors can accurately quantify speed, amplitude, and rhythm to aid in the development and evaluation of novel therapies in Parkinson's disease.
Movement Disorders 04/2011; 26(10):1859-63. · 4.51 Impact Factor
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Neurology 04/2011; 76(17):1529-30. · 8.31 Impact Factor
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Movement Disorders 03/2011; 26(5):913-4. · 4.51 Impact Factor
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Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 01/2011; 122(1):5-6. · 3.12 Impact Factor
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Alberto J Espay
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ABSTRACT: Motor fluctuations and dyskinesias are common motor complications that manifest within the first few years from the initiation of therapy in patients with Parkinson disease. These complications negatively affect the quality of life and represent an important source of disability. A growing number of therapeutic options including treatments aimed at prolonging the efficacy of levodopa (eg, selective monoamine oxidase-B inhibitors and catechol-O-methyltransferase inhibitors), administration of longer-acting dopamine agonists (eg, rotigotine, sustained-release ropinirole), and continuous administration of intraduodenal levodopa exist or will soon become available. Patients who maintain a good response to levodopa but continue to experience disabling motor complications despite the best medical management may benefit from a regimen of subcutaneous apomorphine, ideally delivered by a subcutaneous pump, or deep-brain stimulation of the subthalamic nucleus or internal portion of the pallidum. Emerging therapies for motor complications are expected to further enhance continuous (physiologic) delivery of dopaminergic drugs and extend the reach of therapies beyond the dopaminergic system to influence not only the motor but also the vast range of nonmotor complications of this multisystemic disease.
Neurologic Clinics 11/2010; 28(4):913-25. · 2.34 Impact Factor
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ABSTRACT: A 37-year-old man with insulin-dependent diabetes who had undergone kidney transplantation 7 years earlier presented with delirium caused by diabetic ketoacidosis. His calculated serum osmolarity peaked at 304 mOsm per liter, and the serum glucose level reached 528 mg per deciliter (29.3 mmol per liter). The initial serum sodium level was 132 mmol per liter, which normalized to 139 mmol per liter over 18 hours. The patient's mental status normalized 2 days after the administration of insulin and intravenous hydration. Three weeks later, he noted that his right arm and right leg were “fidgety,” a symptom that rapidly increased in . . .
New England Journal of Medicine 10/2010; 363(17):e27. · 53.30 Impact Factor
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ABSTRACT: A 13-year old girl presented with slowly progressive rest tremor of the hands, bradykinesia, and rigidity. The symptoms improved with dopaminergic medications, but severe drug-induced dyskinesias developed early. She subsequently developed cognitive slowing and difficulty initiating saccadic eye movements. She went on to have deep brain stimulation surgery. Experts discuss the syndromal diagnosis and predict the underlying pathology. The pathological diagnosis is given and clinical learning points are considered.
Movement Disorders 09/2010; 25(12):1860-7. · 4.51 Impact Factor
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ABSTRACT: The long-term benefits of subthalamic nucleus deep brain stimulation (STN DBS) applied earlier in the disease course, before significant disability accumulates, remain to be determined. We developed a Markov state transition decision analytic model to compare effectiveness in quality-adjusted life years (QALYs) of STN DBS applied to patients with PD at an "early" ("off time" 10-20%) versus "delayed" stage ("off time" >40%). A lifelong time horizon and societal perspective were assumed. Probabilities and rates were obtained from literature review; utilities were derived using the time trade-off technique and a computer-assisted utility assessment software tool applied to a cohort of 22 STN-DBS and 21 non-STN-DBS PD patients. Uncertainty was assessed through one- and two-way sensitivity analyses and probabilistic sensitivity analysis using second-order Monte Carlo simulations. Early STN DBS was preferred with a quality-adjusted life expectancy of 22.3 QALYs, a gain of 2.5 QALYs over those with delayed surgery (19.8 QALYs). Early STN DBS was preferred in 69% of 5,000 Monte Carlo simulations. Early surgery was robustly favored through most sensitivity analyses. Delayed STN DBS afforded greater QALYs when using utility estimates exclusively from non-STN-DBS patients and, for the entire group, if the rate of motor progression were to exceed 25% per year. Although decision modeling requires assumptions and simplifications, our exploratory analysis suggests that STN DBS performed in early PD may convey greater quality-adjusted life expectancy when compared to a delayed procedure. These findings support further evaluation of early STN DBS in a controlled clinical trial.
Movement Disorders 07/2010; 25(10):1456-63. · 4.51 Impact Factor
