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Guest Editorial |
Bradley C. Hiner, MD, Neurologic Director, Deep Brain Stimulation Program, Associate Professor, Medical College of Wisconsin, Milwaukee, Wisconsin USA
Reprint Requests: Bradley C. Hiner, MD, Neurologic Director, DBS Program, Associate Professor, Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee,WI, 53226, Email: bhiner{at}mcw.edu
Key Words: Parkinsons disease Atypical parkinsonian disorders Differential diagnosis Deep brain stimulation Growth hormone test
Parkinsons disease (PD) is a common disorder that should be, and usually is, easy to diagnose. The clinical features first described by James Parkinson in 1817 continue to apply today. The most widely accepted modern version is from the UK Parkinsons Disease Society Brain Bank1 whose criteria for a diagnosis of Parkinsons disease are bradykinesia (slowness of initiation of voluntary movement with progressive reduction in speed and amplitude of repetitive actions) and at least one of the following:
muscular rigidity on passive range of motion,
46 Hz resting tremor,
postural instability not caused by primary visual, vestibular, cerebellar or proprioceptive function. A variety of exclusion criteria (e.g., history of repeated strokes, repeated head trauma, encephalitis, neuroleptic use and abnormal neuroimaging) as well as supportive criteria (e.g., unilateral onset and levodopa responsiveness) can help to weaken or strengthen the presumptive diagnosis. Nevertheless, clinicopathologic studies have demonstrated that even in the best of hands the correct diagnosis is missed in approximately 20% of cases.2,3 Disorders like progressive supranuclear palsy and the multiple systems atrophy syndromes (i.e., striatonigral degeneration, olivopontocerebellar atrophy, Shy-Drager syndrome, corticobasal degeneration) make up the remaining 20% that are often misdiagnosed.
The importance of making the correct diagnosis is at least three-fold:
Currently, we have no reliable adjunctive tools to help us accurately distinguish the atypical parkinsonian disorders from idiopathic PD. Standard magnetic resonance imaging or computed tomography studies have limited usefulness. More expensive and often research-based technologies like positron emission tomography, single photon emission computed tomography scans or functional magnetic resonance imaging studies do not yet have a practical place in making these diagnoses; blood work and cerebral spinal fluid studies are currently of no value. A good history and physical examination, along with assessment of levodopa responsiveness, remain our strongest tools.
Nevertheless, in this issue of Clinical Medicine & Research, Pellecchia and colleagues4 review data which provocatively suggest that indeed there may be a relatively simple adjunctive test to assist in differential diagnosis of parkinsonian disorders: measuring serum growth hormone (GH) levels in response to challenge with clonidine or arginine. These agents both act physiologically as GH secretagogues, and while idiopathic PD patients demonstrate essentially normal responses over the course of 1 hour, the atypical syndromes do not show the expected rise in GH. The literature is still small and somewhat controversial, but the review by Pellecchia et al4 helps shed light on an area of marked importance in neurology and will hopefully stimulate future studies. As in most things medical: more work is required.
[See related article pp. 322325]
References
Related articles in CM&R:
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