Dopa-responsive dystonic camptocormia.
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Biomedical subjects
Publications and source records attributed to Jay A Van Gerpen.
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BACKGROUND: The threat of levodopa-induced dyskinesias often influences early treatment decisions in those with Parkinson disease. OBJECTIVE: To determine the long-term risks of levodopa-associated dyskinesias of any severity, dyskinesias sufficient to require medication adjustment, and dyskinesias failing medication adjustments. DESIGN: The medical records linkage system of the Rochester Epidemiology Project was used to identify all incident Parkinson disease patients treated with levodopa (1976-1990). All records were independently reviewed by 2 neurologists who recorded demographic and drug data, dates when dyskinesias were initially identified, and dates when dyskinesias were sufficient to require medication changes; dyskinesias not controlled by drug adjustments were also tabulated. RESULTS: We identified 126 incident Parkinson disease patients treated with levodopa for at least 2 months. By Kaplan-Meier analysis, the estimated rate of dyskinesias was 30% by 5 treatment years and 59% by 10 years. However, the rate of dyskinesias requiring medication adjustment was estimated to be only 17% by 5 years and 43% by 10 years. At 10 treatment years, the rate of dyskinesias that could not be controlled with medication adjustments was estimated at only 12%. An increased risk was associated with younger age and higher initial levodopa dose, but not with sex. CONCLUSIONS: Levodopa-associated dyskinesias can be expected to develop in nearly 60% of patients in our community after 10 years, but these will be severe enough to require medication adjustments in only 43% of patients. At 10 treatment years, nearly 90% of these patients can expect to be spared dyskinesias that could not be controlled by drug adjustments. This population-based study suggests dyskinesia risk may not be a major concern for most Parkinson disease patients.
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We assessed the 5-year risk of levodopa-induced dyskinesias by age of Parkinson's disease (PD) onset in a population-based cohort. The medical records linkage system of the Rochester Epidemiology project was used to identify residents of Olmsted County, Minnesota, with incident PD for the period 1976 through 1990. In this population-based study, the 5-year dyskinesia incidence declined with age. With PD onset after 70 years of age, the incidence was 16%. With onset from 40 to 59 years of age, the incidence was higher at 50%. These incidence data may overestimate the functional importance, because patients with minimal or reversible dyskinesias were counted.
CONTEXT: The neurologic manifestations, laboratory findings, and outcome of patients with West Nile virus (WNV) infection have not been prospectively characterized. OBJECTIVE: To describe prospectively the clinical and laboratory features and long-term outcome of patients with neurologic manifestations of WNV infection. DESIGN, SETTING, AND PARTICIPANTS: From August 1 to September 2, 2002, a community-based, prospective case series was conducted in St Tammany Parish, La. Standardized clinical data were collected on patients with suspected WNV infection. Confirmed WNV-seropositive patients were reassessed at 8 months. MAIN OUTCOME MEASURES: Clinical, neurologic, and laboratory features at initial presentation, and long-term neurologic outcome. RESULTS: Sixteen (37%) of 39 suspected cases had antibodies against WNV; 5 had meningitis, 8 had encephalitis, and 3 had poliomyelitis-like acute flaccid paralysis. Movement disorders, including tremor (15 [94%]), myoclonus (5 [31%]), and parkinsonism (11 [69%]), were common among WNV-seropositive patients. One patient died. At 8-month follow-up, fatigue, headache, and myalgias were persistent symptoms; gait and movement disorders persisted in 6 patients. Patients with WNV meningitis or encephalitis had favorable outcomes, although patients with acute flaccid paralysis did not recover limb strength. CONCLUSIONS: Movement disorders, including tremor, myoclonus, and parkinsonism, may be present during acute illness with WNV infection. Some patients with WNV infection and meningitis or encephalitis ultimately may have good long-term outcome, although an irreversible poliomyelitis-like syndrome may result.
Acute weakness associated with West Nile virus (WNV) infection has previously been attributed to a peripheral demyelinating process (Guillain-Barré syndrome); however, the exact etiology of this acute flaccid paralysis has not been systematically assessed. To thoroughly describe the clinical, laboratory, and electrodiagnostic features of this paralysis syndrome, we evaluated acute flaccid paralysis that developed in seven patients in the setting of acute WNV infection, consecutively identified in four hospitals in St. Tammany Parish and New Orleans, Louisiana, and Jackson, Mississippi. All patients had acute onset of asymmetric weakness and areflexia but no sensory abnormalities. Clinical and electrodiagnostic data suggested the involvement of spinal anterior horn cells, resulting in a poliomyelitis-like syndrome. In areas in which transmission is occurring, WNV infection should be considered in patients with acute flaccid paralysis. Recognition that such weakness may be of spinal origin may prevent inappropriate treatment and diagnostic testing.
BACKGROUND: We conducted a population-based case-control study to investigate the association of Parkinson's disease (PD) with preceding nonfatal cancer. METHODS: We used the medical records-linkage system of the Rochester Epidemiology Project to identify all incident cases of PD in Olmsted County, MN (1976-1995). Each case was matched by age and sex to a general population control. We ascertained cancer diagnoses through medical records abstraction. RESULTS: The frequency of any cancer was lower in cases (19.4%) than in controls (23.5%) (OR = 0.79; 95% CI = 0.49-1.27). This pattern was more pronounced in women than in men, and in patients age 71 years or younger at onset of PD than in older patients. We found an interaction between smoking and smoking-related cancers in their association with PD. Bladder cancer (OR = 0.22; 95% CI = 0.03-2.24) and breast cancer (OR = 0.20; 95% CI = 0.02-1.71) were less frequent in PD cases than in controls, whereas prostate cancer was more frequent in PD cases than in controls (OR = 1.80; 95% CI = 0.60-5.37). However, these results are based on small numbers. CONCLUSIONS: We did not find a strong association between PD and preceding nonfatal cancer. There were suggestive trends in analyses stratified by sex and age at onset of PD, and for specific cancers related to smoking or hormonal factors.