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The Evolution of Diagnosis in Early Parkinson Disease
Joseph Jankovic, MD;
Ali H. Rajput, MD;
Michael P. McDermott, PhD;
Daniel P. Perl, MD;
for the Parkinson Study Group
Arch Neurol. 2000;57:369-372.
Context Since there is no diagnostic biological marker for Parkinson disease (PD), the diagnosis is based on the results of clinical assessment. The accuracy of diagnosis improves with time and repeated assessments. Studies that require only inclusion of early cases of PD present a diagnostic challenge. Previous studies concluded that initial diagnoses of PD made by general neurologists were incorrect in 24% to 35% of the cases when patients were examined at autopsy. Experts in movement disorders are expected to have greater accuracy of initial diagnosis of PD.
Objective To determine the evolution of clinical diagnosis in patients with early PD made initially by experts in PD.
Design Eight hundred patients with mild parkinsonian symptoms (Hoehn and Yahr stage 1 or 2) who received a diagnosis of PD less than 5 years before the beginning of the study were included in the original Deprenyl and Tocopherol Antioxidative Therapy for Parkinson's Disease study. These patients were followed up prospectively with repeated clinical assessments. The following clinical criteria were used to reassess the initial diagnosis: investigator's confidence in the diagnosis of PD, presence of atypical clinical features, findings of imaging studies, response to levodopa, and results of autopsy examinations.
Results The mean ± SD duration of illness in the 800 cases at enrollment was 2.2 ± 1.3 years, and the mean ± SD Hoehn and Yahr stage was 1.6 ± 0.5. The mean ± SD follow-up was 6.0 ± 1.4 years (range, 0.2-7.6 years). In 5 cases, PD was not confirmed at autopsy, and in 15 patients, the results of imaging studies indicated the presence of other pathological conditions. Of the 550 cases treated with levodopa, 49 (8.9%) had little or no improvement; 6 of these cases overlap with either autopsy or imaging study exclusion criteria. Two other cases had at least 4 of the 6 atypical clinical features arguing against the diagnosis of PD. Thus, of the 800 patients, 65 (8.1%) did not have PD according to the study criteria. Compared with those patients with the final diagnosis of PD, in the diagnoses of 60 patients without autopsy, the duration of symptoms (mean ± SD, 7.2 ± 2.0 years vs 8.3 ± 1.9 years; P<.001) and the duration of follow-up (5.3 ± 1.6 years vs 6.1 ± 1.3 years; P<.001) were shorter.
Conclusions We found that 65 (8.1%) of patients initially diagnosed as having PD were later found to have an alternate diagnosis based on multifactorial clinical diagnostic criteria. This alternate diagnosis indicated that experts in PD changed their diagnoses infrequently during the 7.6-year follow-up.
From the Department of Neurology, Baylor College of Medicine, Houston, Tex (Dr Jankovic); Department of Medicine, Royal University Hospital, University of Saskatchewan, Saskatoon (Dr Rajput); Department of Biostatistics, University of Rochester School of Medicine and Dentistry, Rochester, NY (Dr McDermott); and Department of Pathology (Neuropathology), Mount Sinai School of Medicine, New York, NY (Dr Perl). A complete list of the members of the Parkinson Study Group has been published previously (Arch Neurol. 1995;52:565-570).
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