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Parkinson's Disease View online at http://pier.acponline.org/physicians/diseases/d243/d243.html Module Updated: 2013-01-24 CME Expiration: 2016-01-24 Author Melissa Gaines, MD Table of Contents 1. Diagnosis ..........................................................................................................................2 2. Consultation ......................................................................................................................6 3. Hospitalization ...................................................................................................................11 4. Therapy ............................................................................................................................12 5. Patient Counseling ..............................................................................................................22 6. Follow-up ..........................................................................................................................23 References ............................................................................................................................28 Glossary................................................................................................................................37 Tables ...................................................................................................................................40 Figures .................................................................................................................................55 Quality Ratings: The preponderance of data supporting guidance statements are derived from: level 1 studies, which meet all of the evidence criteria for that study type; level 2 studies, which meet at least one of the evidence criteria for that study type; or level 3 studies, which meet none of the evidence criteria for that study type or are derived from expert opinion, commentary, or consensus. Study types and criteria are defined at http://smartmedicine.acponline.org/criteria.html Disclaimer: The information included herein should never be used as a substitute for clinical judgement and does not represent an official position of the American College of Physicians. Because all PIER modules are updated regularly, printed web pages or PDFs may rapidly become obsolete. Therefore, PIER users should compare the module updated date on the offical web site with any printout to ensure that the information is the most current available. CME Statement: The American College of Physicians is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing education for physicians. The American College of Physicians designates this enduring material for a maximum of 1 AMA PRA Category 1 CreditTM. Physicians should claim only credit commensurate with the extent of their participation in the activity. Purpose: This activity has been developed for internists to facilitate the highest quality professional work in clinical applications, teaching, consultation, or research. Upon completion of the CME activity, participants should be able to demonstrate an increase in the skills and knowledge required to maintain competence, strengthen their habits of critical inquiry and balanced judgement, and to contribute to better patient care. Disclosures: Melissa Gaines, MD, current author of this module, has no financial relationships with pharmaceutical companies, biomedical device manufacturers, or health-care related organizations. Deborah Korenstein, MD, FACP, Co-Editor, PIER, has no financial relationships with pharmaceutical companies, biomedical device manufacturers, or health-care related organizations. Richard B. Lynn, MD, FACP, Co-Editor, PIER, has no financial relationships with pharmaceutical companies, biomedical device manufacturers, or health-care related organizations. PIER is copyrighted ©2013 by the American College of Physicians. 190 N. Independence Mall West, Philadelphia, PA 19106, USA. Parkinson's Disease Top 1. Diagnosis Diagnose Parkinson's disease by documenting the presence of characteristic neurologic features. 1.1 Conduct a thorough history and neurologic exam to establish the diagnosis. Recommendations • Assess patients for the presence of distinguishing features: Onset at age >20 years Progressive symptomatology Rigidity (resistance to movement), tremor (typically a rest tremor), or slowness (bradykinesia) Absence of definable etiology of parkinsonism or absence of exclusionary features See table Differential Diagnosis of Parkinson's Disease for list of definable etiologies of parkinsonism See table Exclusionary Features for Diagnosis of Parkinson's Disease. • Note additional features supporting the diagnosis of Parkinson's disease: Definite clinical improvement with levodopa and/or a dopamine agonist Asymmetric onset with persistent asymmetry (i.e., the initially symptomatic side remains more severely affected as the disease progresses) Levodopa response for 5 years or more Presence of levodopa-induced dyskinesias (i.e., choreiform movements) • See table Mini-Mental State Exam. Evidence • The specificity and sensitivity of the clinical diagnostic criteria of Parkinson's disease are unknown. Parkinson's disease may be overdiagnosed at the expense of other degenerative neurologic conditions, which tend to be underdiagnosed (1; 2). • There are limited means to improve diagnostic accuracy, especially in light of the rarity of autopsies and the lack of readily accessible, in vivo biologic markers of disease. Nonetheless, clinical criteria for an assortment of degenerative parkinsonian conditions have been revised on the basis of the limited clinicopathologic studies in the literature (Parkinson's disease: 3; multiple system atrophy: 4; progressive supranuclear palsy: 5; 6). • A comprehensive review of Parkinson's disease is also available (7; 8). • Consensus guidelines for the diagnosis of Parkinson's disease have been released by the American Academy of Neurology (9). Rationale • Inclusionary and exclusionary clinical features have been selected based on their correlation with postmortem diagnosis of Parkinson's disease or other parkinsonian conditions. 1.2 Screen patients for factors that place them at high risk for developing Parkinson's disease. Recommendations • Obtain a thorough family history for Parkinson's disease. • Assess long-term exposure to suspect chemicals, such as manganese, carbon monoxide, carbon disulfide, MPTP, and cyanide. PIER is copyrighted ©2013 by the American College of Physicians. 190 N. Independence Mall West, Philadelphia, PA 19106, USA. Page 2 of 55 Parkinson's Disease • Determine whether patients meet criteria for REM sleep behavioral disorder or have excessive daytime somnolence. • Evaluate patients for depression and anxiety disorders. • See table Differential Diagnosis of Parkinson's Disease. Evidence • There is evidence that gene mutations may play a role in causing juvenile and early-onset (10) and autosomal-dominant (11) Parkinson's disease. • A diagnosis of REM sleep behavioral disorder has been shown to be associated with later emergence of Parkinson's disease in more than one-third to one-half of patients studied. This relationship may be mediated in part by alterations in striatal dopamine neurons or a specific loss of atonia (12; 13; 14). • Cohort studies have suggested that persons who suffer from excessive daytime somnolence may be at risk for future development of Parkinson's disease (15). • A 2002 retrospective cohort study showed that depressed patients were at significantly greater risk for developing Parkinson's disease than nondepressed patients (16). Rationale • Although there are no treatments that prevent Parkinson's disease, efforts are under way to identify factors that increase risk so that patients can be diagnosed and treatment can be initiated as early as possible. Comments • A review of medical records in 2249 patients with Parkinson's disease or parkinsonism with onset at or before age 50 found that physicians, dentists, farmers, lawyers, scientists, clergy, and computer programmers and technicians were overrepresented. The significance of these findings is unclear (17). Although welders were previously thought to be at increased risk (18), a large database study did not support the association (19). 1.3 Recognize that the diagnosis of Parkinson's disease is made clinically because there is no readily available diagnostic test. Recommendations • Perform a comprehensive neurologic exam: Mini-mental status exam: an abnormal exam early in the course suggests an alternative diagnosis Supine and standing heart rate and blood pressure: orthostatic hypotension may occur in advanced disease or may be due to blood pressure medications Eye exam: Kayser-Fleischer rings are seen in Wilson's disease Eye movements: impairments in vertical eye movements suggest an alternative diagnosis Motor exam: increased tone (in patients with Parkinson's disease) Reflexes: are normal Sensory: an abnormal sensory exam may occur in parkinsonism due to other causes than Parkinson's disease Coordination exam: impaired coordination suggests alternative diagnoses Tremor assessment: an action and postural tremor Stance and gait assessment: reduced stride length and posture stooped, can have asymmetric reduced arm swing • Perform MRI, CT scan, or both if the diagnosis is uncertain. • In atypical cases of parkinsonism, or possibly in those with symptom onset before age 50, consider an MRI brain scan. 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