Parkinsonian Syndromes: Clinical Recognition and Treatment (Movement Disorders 2013)

Educational Objectives

The goal of this program is to improve the diagnosis and management of Parkinson disease and atypical parkinsonian syndromes. After hearing and assimilating this program, the clinician will be better able to:

1. Differentiate Parkinson disease (PD) from the atypical parkinsonian syndromes.
2. Identify the characteristic features of progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration.
3. Manage parkinsonian symptoms while minimizing adverse effects.

Summary

Parkinsonism: Characterized by bradykinesia, hypokinesia, rigidity, tremor at rest, and/or postural instability; in addition to motor dysfunction, cognitive, autonomic, and sleep disturbances may be substantial and associated with decreased quality of life.

Bradykinesia and hypokinesia: Assessment — patient taps thumb and finger together or taps heel or toe against floor; signs — slow movements; decreasing amplitude of movements; gait — unstable gait common in patients with atypical parkinsonian disorders; wide-based gait suggests cerebellar involvement.

Diagnosing parkinsonian disorders: Primarily clinical diagnosis; although DaTscan may support diagnosis (by eg, low levels of dopamine in striatum), it cannot distinguish Parkinson disease (PD) from atypical parkinsonian disorders nor distinguish among atypical parkinsonian disorders.

Clinical diagnosis: In patient without characteristic signs of atypical parkinsonian disorder, strong response to levodopa therapy confirms diagnosis of PD; features that suggest diagnosis other than PD (eg, atypical parkinsonian disorder) include gait disturbance, oculomotor disturbance, orthostatic hypotension, dementia, and hallucinations, especially when these occur early in disease course.

Implications of diagnosis: Management — levodopa effective in PD but not in most atypical parkinsonian disorders; deep brain stimulation (DBS) appropriate in patients with PD and significant motor problems but not helpful in most patients with atypical parkinsonism; prognosis — when treated, patients with PD often have normal life expectancies, but life expectancy often reduced in patients with atypical parkinsonian disorders.

Progressive Supranuclear Palsy (PSP)

Presentation: Richardson syndrome — most common presentation; characteristic features occurring early in disease course include postural instability, gait disturbance, and falls; other early features include dysarthria and dysphagia; supranuclear vertical gaze palsy — preceded by slowing of vertical saccades and decreased vertical optokinetic nystagmus (evaluated by observing eye movement as patient quickly shifts gaze between two targets), at which time, diagnosis of PSP can be made.

Other presentations: One-third of patients with PSP have presentation similar to that of PD, with unilateral parkinsonism that initially responds to levodopa therapy; patients later develop atypical features and no longer respond well to levodopa; important to evaluate eye movements over time, especially in patients whose responsiveness to levodopa diminishes; frozen gait variant — akinetic freezing of gait occurs without other abnormalities in movement (although dysarthria may occur); autopsy studies show PSP pathology, but life expectancy higher than in patients with other forms of PSP; management focuses on physical therapy.

Pathology: Aggregation of tau in neurons and glia in brainstem and midbrain (eg, substantia nigra, superior colliculus, periaqueductal gray, basal ganglia, subthalamic nucleus, globus pallidus); involvement of frontal cortex may occur later in disease process; natural history — changes occur slowly and progressively; impairment develops in motor and nonmotor systems; aspiration pneumonia (result of dysphagia) most common cause of death; average duration of survival after diagnosis, ≈7 years.

Management: No effective pharmacotherapy available; mainstay of management includes physical therapy (eg, gait training) and strategies to avoid complications (eg, aspiration).

Multiple System Atrophy (MSA)

Presentation: Autonomic disturbances precede development of parkinsonism and gait problems; important to ask about symptoms of autonomic dysfunction because patients may not volunteer information; other characteristic features — cerebellar features (eg, dysmetria, wide-based gait); stimulus-sensitive myoclonus; stridor may result from dystonia of vocal cords; progression — rapid.

Pathology: Aggregation of α-synuclein in oligodendroglia occurs in MSA, PD, and Lewy body dementia; distinguishing features of patients with MSA include REM behavior disorder and early autonomic disturbances.

Management: Parkinsonism — patients with α-synucleinop­athies may respond to dopaminergic therapy; ≥30% of patients with MSA respond well, and as many as 50% have partial response; autonomic symptoms — typically responsive to standard therapies; use of levodopa or other dopamine agonists often worsens orthostatic hypotension.

Corticobasal Syndrome

Presentation: Features include unilateral apraxia, ideomotor apraxia, myoclonus, dystonia, and alien limb phenomenon; parkinsonism does not respond to dopaminergic therapy; nonmotor features include cortical sensory deficits (eg, sensory neglect), visuospatial deficits, progressive nonfluent aphasia, and frontotemporal dementia (primarily apathetic); life expectancy — decreased.

Pathology: Corticobasal syndrome caused by multiple underlying pathologies, including corticobasal degeneration (CBD; most common pathology), PSP, Alzheimer disease, Pick disease, and Creutzfeldt-Jakob disease.

Management: No effective treatments available; potential therapeutic targets include tau protein, but trials of glycogen synthase kinase-3 beta (GSK-3β) inhibitors found no benefit.

Vascular Parkinsonism

Etiology and presentation: Multiple strokes may lead to development of parkinsonian features, especially affecting lower limbs and gait.

Management: Primarily involves management of cardiovascular risk factors and prevention of subsequent stroke; occasionally, patients respond to levodopa therapy.

Readings

Williams DR, Litvan I. Parkinsonian syndromes: clinical recognition and treatment. Continuum (Minneap Minn) 2013;19(5).

Disclosures

For this program, the following was disclosed: Dr. Litvan has served as a member of the Abbot/Abbvie, Biogen, Bristol-Myers-Squibb, and Pfizer scientific advisory boards and was a consultant for Novartis. Unlabeled Use of Products/Investigational Use Disclosure: Dr. Litvan reports nothing to disclose. To view disclosures of planning committee members with relevant financial relationships, visit: audiodigest.org/continuumaudio/committee. All other members of the planning committee report nothing to disclose.

Acknowledgements

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Audio Digest Foundation is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. Audio Digest Foundation designates this activity for 0 CE contact hours.

Lecture ID:

CA021701

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

To earn CME/CE credit for this course, you must complete all the following components in the order recommended: (1) Review introductory course content, including Educational Objectives and Faculty/Planner Disclosures; (2) Listen to the audio program and review accompanying learning materials; (3) Complete posttest (only after completing Step 2) and earn a passing score of at least 80%. Taking the course Pretest and completing the Evaluation Survey are strongly recommended (but not mandatory) components of completing this CME/CE course.

Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.