Continuum: Lifelong Learning in Neurology

Movement disorders that are infrequently seen in clinical practice can be difficult to recognize and accurately diagnose. Familiarity with these disorders can help the clinician distinguish them from more common movement disorders, such as Parkinson disease, which is associated with a significantly different prognosis and treatment approach. Rare disorders with predominate parkinsonism include multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration, whereas Huntington disease is the primary movement disorder characterized by chorea. This chapter will provide an overview of the diagnostic, pathologic, and imaging advances from recent literature as well as discuss current treatments for these rare movement disorders.(C) 2010 American Academy of Neurology

Dopaminergic agents remain the principal treatments for patients with Parkinson disease (PD). In many patients, however, a combination of relatively resistant motor symptoms, motor complications such as dyskinesias, or nonmotor symptoms such as dysautonomia may lead to substantial disability in spite of dopaminergic therapy. This chapter will review both dopaminergic and nondopaminergic therapies for motor and nonmotor symptoms in PD.Although the basic principles of pharmacotherapy for the motor symptoms of PD have largely remained unchanged over the past decade, several new therapies have become available to refine treatment. In addition, there has been an increasing interest in agents targeting nonmotor symptoms, such as dementia and sleepiness. As patients with PD live longer and acquire additional comorbidities, addressing these nonmotor symptoms has become increasingly important. In this chapter, the major antiparkinsonian dopaminergic compounds will be reviewed, followed by a patient-focused guide to implementation of these treatments as part of an overall management plan.(C) 2010 American Academy of Neurology

The therapeutic use of botulinum neurotoxin has exploded since the first US Food and Drug Administration indication was obtained in 1989, and today it represents the first-line therapy for several hyperkinetic movement disorders. Of the seven serotypes (A to G), types A and B have been approved for use in the United States. Two type A toxins, onabotulinumtoxinA (Botox) and abobotulinumtoxinA (Dysport), are available, and one type B toxin, rimabotulinumtoxinB (Myobloc) is available. The commercially available toxins differ by protein target, duration of action, and adverse event profile; no formula exists for interconversion. The clinical development of the toxin is outlined and methods for muscle targeting are compared. Treatment regimens should be designed to achieve a specific care or functional goal by interdisciplinary teams consisting of physicians, patients, caregivers, and therapists, when appropriate. We discuss dosing considerations and safety profiles in the context of hyperkinetic movement disorders commonly encountered by neurologists, including cervical dystonia, spasticity, pediatric spasticity, blepharospasm, focal limb dystonias, and essential tremor. Finally, the multiple illustrative cases sprinkled throughout the chapter demonstrate the highly individualized, goal-focused nature of treatment with neurotoxins.(C) 2010 American Academy of Neurology