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Parkinson's disease can only be diagnosed clinically. New practice parameters issued by the American Academy of Neurology reinforce the role of careful observation and a good history, especially since some of the early signs and symptoms of this chronic movement disorder offer clues to its future course. [ABSTRACT FROM AUTHOR]

Background: Reported risk factors for the development of a psychogenic movement disorder (PMD) include young age, female gender, history of abuse, current or past psychiatric disorder, lower levels of education and socioeconomic status, and employment as a healthcare worker. Although employment in healthcare is included in several diagnostic criteria for PMD, as well as in many case series, this association has never been validated., Methods: Using the University of Maryland Movement Disorder Database (UMMDD), we identified PMD cases, as well as patients with isolated focal dystonia as controls. An experienced movement disorder specialist diagnosed all patients, and all cases met criteria for clinically established PMD. Demographic and occupational histories were obtained from medical records and were supplemented by telephone interviews. PMD cases and controls were compared using t tests/χ 2 tests., Results: Controls (n = 148) were older than PMD cases (n = 132), with an average age of 61.4 and 52.1 years, respectively ( P  <   0.001); there were no significant differences between groups with respect to gender, education level, and ethnicity. The proportion of healthcare workers was not significantly different between PMD cases and controls (25% of PMD cases vs. 20% of controls; P  =   0.28)., Conclusions: In contrast to traditional teaching, this investigation demonstrates that in our patient population, patients with a PMD were no more likely to be employed as healthcare workers than patients with isolated focal dystonia. This study calls into question the use of employment in healthcare as a reliable criterion to support the diagnosis of PMD.

Purpose: To investigate whether patient-spouse co-reporting (patient reporting with assistance from their spouse) results in the same ratings of health-related quality of life (HRQoL) as patient ratings without co-reporting, and to assess whether mutuality of the marital relationship is a determinant of co-reported ratings. Patients are the best source of HRQoL; however, co-reporting is common in clinical settings, but has not been compared to independent patient reporting of HRQoL., Methods: Fifty-nine Parkinson's disease (PD) patient-spouse pairs completed the Short Form Health Status Survey (SF-12), measuring mental and physical HRQoL. Initially, the patient and spouse completed the SF-12 independently (about the patient). Then, patient-spouse pairs completed the SF-12 together assessing the patient's HRQoL. Patients and spouses independently completed the Mutuality Scale rating the intimacy of their relationship., Results: Patients rated physical HRQoL higher (M = 46.6) than spouses alone (M = 43.4, p < 0.01) and co-reporting (M = 44.8, p < 0.05). Co-reporting rated physical HRQoL in between that of patients and spouses, (p < 0.05). Spouses who reported greater mutuality showed greater concordance with the patient regarding the patient's mental HRQoL (B = -0.43, p < 0.05)., Conclusion: Consistency of the mode of completion of HRQoL instruments is important since co-reporting may alter HRQoL ratings in PD and lead to inaccurate conclusions. Mutuality is a mediator of mental HRQoL.

Objective: To make evidence-based recommendations regarding management of tardive syndromes (TDS), including tardive dyskinesias (TDD), by addressing 5 questions: 1) Is withdrawal of dopamine receptor blocking agents (DRBAs) an effective TDS treatment? 2) Does switching from typical to atypical DRBAs reduce TDS symptoms? 3) What is the efficacy of pharmacologic agents in treating TDS? 4) Do patients with TDS benefit from chemodenervation with botulinum toxin? 5) Do patients with TDS benefit from surgical therapy?, Methods: PsycINFO, Ovid MEDLINE, EMBASE, Web of Science, and Cochrane were searched (1966-2011). Articles were classified according to a 4-tiered evidence-rating scheme; recommendations were tied to the evidence., Results and Recommendations: Clonazepam probably improves TDD and ginkgo biloba probably improves TDS (both Level B); both should be considered as treatment. Risperidone may improve TDS but cannot be recommended as treatment because neuroleptics may cause TDS despite masking symptoms. Amantadine and tetrabenazine might be considered as TDS treatment (Level C). Diltiazem should not be considered as TDD treatment (Level B); galantamine and eicosapentaenoic acid may not be considered as treatment (Level C). Data are insufficient to support or refute use of acetazolamide, bromocriptine, thiamine, baclofen, vitamin E, vitamin B6, selegiline, clozapine, olanzapine, melatonin, nifedipine, fluperlapine, sulpiride, flupenthixol, thiopropazate, haloperidol, levetiracetam, quetiapine, ziprasidone, sertindole, aripiprazole, buspirone, yi-gan san, biperiden discontinuation, botulinum toxin type A, electroconvulsive therapy, α-methyldopa, reserpine, and pallidal deep brain stimulation as TDS treatments (Level U). Data are insufficient to support or refute TDS treatment by withdrawing causative agents or switching from typical to atypical DRBA (Level U).

Background: Japan and the United States (US) have different cultures of caregiving including differences in family structure and social programs that may influence caregiver strain. Differences in caregiver strain between regions in Japan and in the US have not been investigated in patient-spouse dyads in PD., Objectives: To compare caregiver strain in spouses of PD patients between Yamagata, Japan and Maryland, US. Correlations between caregiver strain and patient/spousal variables are also examined., Methods: In Yamagata and Maryland, spouses of patients with PD completed questionnaires assessing caregiver strain. Patients and spouses completed scales assessing mental health, and medical co-morbidity. PD severity and disability were assessed with the Unified Parkinson's Disease Rating Scale and the Schwab and England Activities of Daily Living Scale. Results in the two regions were compared with Chi-square and Student's t-tests. Relationships between caregiver strain and patient/spousal variables were analyzed with univariate correlations and multivariate regression., Results: 178 Spouse-patient pairs were assessed. The level of caregiver strain in PD did not differ between Yamagata, Japan and Maryland, US despite differences in demographics and social support programs in the two regions. Yamagata spouses reported physical, time and financial constraints, while Maryland spouses reported more emotional distress. In both regions, spousal depression was a significant contributor to caregiver strain., Conclusion: Different approaches to reduce caregiver strain will likely be necessary in Yamagata and Maryland since the contributing factors to caregiver strain are influenced by differences in culture and social supports in each country., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Diagnosis of leptomeningeal leukemia (and more broadly, leptomeningeal metastasis [LM]) is based on:

Background: Dual task (DT) performance assesses the ability to perform two tasks simultaneously. Difficulty with DT performance may be a sensitive indicator of early Parkinson's disease (PD) impairment. The objective of this study was to assess what elements of a DT performance (cognition or gait) are most associated with impairment and disability in PD., Methods: Performance in single and DT conditions was examined in 154 PD patients. The single task assessments included the time required to walk 50 feet (gait speed) and the number of words generated in a verbal fluency task (word generation). The DT comprised simultaneous performance of the single tasks. Impairment and disability were measured with the Unified Parkinson's Disease Rating Scale, Hoehn &Yahr, Berg Balance Scale, and Older Americans Resource and Services Scale. Age, education, and gender were control variables. Standardized residuals from regressions of DT upon single task performance were computed separately for word and gait, indicating the extent that the individual performed proportionally better/worse than predicted in DT considering their single task performance., Results: Multiple regressions revealed that individuals who performed worse than expected in DT-word had greater impairment and disability. Dual task-gait was not significant in any model. Verbal fluency during DT performance is more closely associated with PD-related impairment and disability than gait speed during DT., Conclusion: This suggests that subjects prioritize gait performance at the expense of cognitive performance, and that DT word generation may be a sensitive indicator of early PD impairment and disability., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Objective: To compare the efficacy of treadmill exercises and stretching and resistance exercises in improving gait speed, strength, and fitness for patients with Parkinson disease., Design: A comparative, prospective, randomized, single-blinded clinical trial of 3 types of physical exercise., Setting: The Parkinson's Disease and Movement Disorders Center at the University of Maryland and the Baltimore Veterans Affairs Medical Center, Geriatric Research Education and Clinical Center., Patients: A total of 67 patients with Parkinson disease who had gait impairment were randomly assigned to 1 of 3 arms of the trial. INTERVENTIONS; (1) A higher-intensity treadmill exercise (30 minutes at 70%-80% of heart rate reserve), (2) a lower-intensity treadmill exercise (50 minutes at 40%-50% of heart rate reserve), and (3) stretching and resistance exercises (2 sets of 10 repetitions on each leg on 3 resistance machines [leg press, leg extension, and curl]). These exercises were performed 3 times a week for 3 months., Main Outcome Measures: The primary outcome measures were gait speed (6-minute walk), cardiovascular fitness (peak oxygen consumption per unit time [$$ VO2], and muscle strength (1-repetition maximum strength)., Results: All 3 types of physical exercise improved distance on the 6-minute walk: lower-intensity treadmill exercise (12% increase; P=.001), stretching and resistance exercises (9% increase; P<.02), and higher-intensity treadmill exercise (6% increase; P=.07), with no between-group differences. Both treadmill exercises improved peak $$ VO2 (7%-8% increase; P<.05) more than did the stretching and resistance exercises. Only stretching and resistance improved muscle strength (16% increase; P<.001)., Conclusions: The effects of exercise were seen across all 3 exercise groups. The lower-intensity treadmill exercise resulted in the greatest improvement in gait speed. Both the higher- and lower-intensity treadmill exercises improved cardiovascular fitness. Only the stretching and resistance exercises improved muscle strength. Therefore, exercise can improve gait speed, muscle strength, and fitness for patients with Parkinson disease. The combination of treadmill and resistance exercises may result in greater benefit and requires further investigation.

Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. An international consortium of behavioral neurology, neuropsychology, and movement disorders specialists developed new criteria based on consensus and a systematic literature review. Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. Combined with consensus, 4 CBD phenotypes emerged: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS). Clinical features of CBD cases were extracted from descriptions of 209 brain bank and published patients, providing a comprehensive description of CBD and correcting common misconceptions. Clinical CBD phenotypes and features were combined to create 2 sets of criteria: more specific clinical research criteria for probable CBD and broader criteria for possible CBD that are more inclusive but have a higher chance to detect other tau-based pathologies. Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset ≥ 50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype. Future validation and refinement of the proposed criteria are needed.

Movement disorders uncommonly require emergent intervention; however, there are acute/subacute clinical settings in which the neurologist is consulted for recommendations about the diagnosis and management of a movement disorder. In these circumstances the neurologist must be comfortable with the diagnostic evaluation and be prepared to properly manage the patient. This article focuses on diagnosis and management of acute-onset movement disorders occurring secondary to prescription drug use, illicit drug abuse, and drug withdrawal syndromes. In addition, drug-induced emergencies occurring in patients with movement disorders are reviewed., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Hemiballism is a rare movement disorder characterized by a high amplitude movement of an entire limb or limbs on one side of the body. The acute development of hemiballismus is often caused by focal lesions in the contralateral basal ganglia and STN. Many etiologies exist for this rare disorder with vascular causes and nonketotic hyperglycemia being the most common. Clearer understanding of the pathophysiology of hemiballism has led to important insights into the function and interaction of structures within the basal ganglia. Newer models of basal ganglia function have been proposed based on the study of the pathophysiology of hemiballism. Prognosis is favorable for most patients with complete resolution with or without treatment. Medical and surgical treatments are often successful in reducing or completely ameliorating the movements in those patients with more severe movements., (Published by Elsevier Ltd.)

Accumulation of alpha-synuclein is a pathological feature in several neurological diseases. Its characterization has allowed for a re-grouping of diseases according to the expected pathology. The clinical syndrome of PD can now be classified into forms with and without alpha-synuclein pathology. DLB and PDD are synucleinopathies, and MSA shows alpha-synuclein pathology with glial inclusions. ADHD symptoms commonly occur in persons that will subsequently develop DLB. A similar phenomenon may be the early personality changes and frontotemporal atrophy in patients with SNCA multiplication. RLS is not known to have alpha-synuclein pathology, but as PD and ADHD, involves a hypodopaminergic state. Furthermore, PD and RLS co-occur in families in a way that suggests common inheritance. A proportion of patients with ET have brainstem Lewy body pathology. Gaucher disease and other lysosomal storage disorders also have alpha-synuclein pathology. Alpha-synuclein is a naturally unfolded protein. Non-fibrillar oligomeres may be the toxic species, and Lewy body formation may in fact be protective. Inhibiting alpha-synuclein toxicity seems to be an attractive novel treatment strategy and several approaches are being developed. When such treatments become available, clinicians will need to be familiar with the clinical features that distinguish the synucleinopathies from their look-alikes., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Psychogenic movement disorders are common, but the diagnosis may be difficult. Visual appearance alone is typically not sufficient to make a diagnosis, but such information is certainly important. That a movement is bizarre can be helpful, but still must be considered thoughtfully since organic movement disorders can have endless variety. The diagnosis should rest on positive findings such as paroxysmal nature, maximum severity at or near onset, variability of tremor direction, frequency and amplitude, entrainment of tremor, distractability and suggestibility, and wildly swaying gait and balance problems with no falling. Psychogenic parkinsonism often poses a problem because of the relatively high frequency of overlap of psychogenic and organic disease. In regard to psychogenic parkinsonism, there are special features to look for. There might be tremor with kinetic movement as well as rest and posture, and finger tremor might be absent. With sequential movements, the sequence effect is typically lacking. Extreme slowness and grunting with great effort may be seen. Improvement in arm swing while running, a feature of organic parkinsonism, may not be seen., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Dystonia in association with peripheral trauma is a well-described clinical syndrome. The syndrome goes by many names--"traumatic" dystonia, "fixed" dystonia, peripherally induced dystonia, or complex region pain syndrome (CRPS) dystonia. We reviewed the role of peripheral trauma in the development of dystonia, focusing on 4 subtypes--cervical dystonia, focal limb dystonia, CRPS dystonia, and psychogenic dystonia. We show that peripheral trauma inducing, provoking, or precipitating structural changes within the CNS leading to dystonia is not an accepted concept, and current evidence supporting a pathophysiologic mechanism is virtually nonexistent. A better approach to this clinical syndrome is to define it as fixed abnormal posturing that is most commonly psychogenic. While symptomatic treatment of pain and spasms with medication can be beneficial, early psychological evaluation and patient-specific treatment is important. Modalities such as physical and occupational therapy should be utilized early. Finally, it should be emphasized that like many psychogenic movement disorders, it remains a highly disabling and distressing disorder.

Although movement disorders do not usually present as neurologic emergencies, there are times when the abrupt onset of an unusual movement abnormality results in emergency department or intensive care unit consultations. Part 1 of this review discussed hypokinetic movement disorders emergencies. Part 2 provides a diagnostic approach to the recognition and treatment of hyperkinetic movement disorders emergencies by identifying phenomenology and reviewing common etiologies.

Movement disorders usually do not require emergent intervention; nevertheless, there are acute/subacute clinical settings in which the neurologist is consulted. It is in these circumstances that the neurologist must be prepared to accurately diagnose and properly treat the patient. We have reviewed the literature regarding movement disorder emergencies and divided them into hypokinetic (part 1) and hyperkinetic (part 2) presentations. In part 1, drug-induced syndromes including neuroleptic malignant syndrome, parkinsonism hyperpyrexia syndrome, and serotonin syndrome will be discussed. Emergency complications related to the management of Parkinson disease, including falling, motor fluctuations, and psychiatric issues, will also be reviewed.

Objective: To assess potential racial and socioeconomic disparities in patients with parkinsonism treated at a tertiary Movement Disorders Center., Methods: Patients with parkinsonism were evaluated for demographics (age, race, annual income, and educational level), medical comorbidities, medication regimen, disability (Older Americans Resources and Services subscale), presence of Parkinson disease, and disease severity (Unified Parkinson Disease Rating Scale). Disability and disease severity measures were compared by race, income, and educational level using analysis of variance for continuous variables and χ(2) tests for dichotomous variables., Results: The sample included 1159 patients with parkinsonism (93.4% white, 6.1% African American, 61.2% who earned more than $50,000 annually, 62.7% who completed college, and 79.2% with a diagnosis of Parkinson disease). Cross-sectional analyses by race, income, and educational level showed greater disability and disease severity in African American compared with white patients (African American vs white Older Americans Resources and Services subscale total score, 29.8 vs 25.3, P = .005; Unified Parkinson's Disease Rating Scale total score, 53.0 vs 42.8; P < .001). African Americans were less likely to be prescribed dopaminergic medications, particularly newer agents (African Americans 20.6% vs whites: 41.1%; P = .01). Lower income and lower educational level were independently associated with greater disease severity and disability (P < .003)., Conclusion: Racial and socioeconomic disparities exist among patients with parkinsonism being treated at a tertiary Movement Disorders Center. African Americans and those with lower socioeconomic status have greater disease severity and disability than whites. These disparities may be because of problems in diagnosis, access to care, physician referrals, and patient attitudes regarding the appropriate threshold for seeking treatment at a specialized center. Understanding and correction of these disparities may improve outcomes.

"Dystonia" is the term used to describe abnormal movements consisting of sustained muscle contractions frequently causing twisting and repetitive movements or abnormal postures. Dystonia is classified partly by age at onset because this helps guide the diagnostic work-up and treatment decisions. This chapter focuses on early-onset (<26 years old) primary dystonia. The history, clinical features, genetics, pathophysiology, diagnosis, and treatment of early-onset primary dystonia are discussed. Special emphasis is placed on DYT1 dystonia, the most common, autosomal-dominant, early-onset, primary dystonia. A diagnostic algorithm is proposed for gene-negative early-onset dystonia, and treatment recommendations for generalized, early-onset dystonia are made., (Copyright © 2011 Elsevier B.V. All rights reserved.)