Among promising biological markers proposed for Parkinson's disease (PD) and other disorders related to Lewy bodies, plasma alpha-synuclein assay has provided conflicting results mainly owing to the various laboratory assay techniques used and protein forms assayed. In this observational and exploratory cross-sectional study, using an immunoenzymatic technique, we assayed and compared total plasma alpha-synuclein concentrations in 69 patients with PD and 110 age-matched healthy control subjects. Two previously unreported findings concerned gender. First, plasma alpha-synuclein concentrations measured in the more advanced parkinsonian disease stages decreased in men, but not in women. Second, again only in men, plasma alpha-synuclein concentration was associated with cognitive impairments, hallucinations, and sleep disorders. These findings underline the gender-related differences in parkinsonian patients and indicate plasma alpha-synuclein expression as a potential biological marker for PD progression in men.
Parkinson's disease (PD) is a neurodegenerative disorder affecting not only the motor system but also the cognitive and behavioral domains. Although there are many studies addressing the issue of cognition, a universally recognized method to diagnose patients with dementia is still lacking. The aim of this study was to determine which neuropsychological test is the most reliable in the diagnosis of dementia in PD and to establish if mini mental state examination (MMSE) is enough to detect this condition. We studied 200 consecutive PD patients through an extensive neuropsychological battery, clinical evaluation and brain magnetic resonance imaging over a period of 4 years. A logistic regression model was used to evaluate the interplay between possible risk factors and the accuracy of different neuropsychological tests. PD patients with dementia performed significantly worse in all the tests as compared to patients with PD alone: phonological verbal fluency, attentional matrices, Rey auditory verbal learning test and digit span were the most useful tools. Age and disease duration were correlated with cognitive impairment. No relevant differences were detected in phenotype, affected body side at onset, levodopa equivalent daily dose or neuroimaging findings (except for the occurrence of atrophy). Despite reasonable accuracy of MMSE (90%), its positive predictive value is only 74%. Using at least 3 neuropsychological tests, among those more significant detected with logistic regression analysis, the positive predictive value rises to 91%. In conclusion, the use of an extensive neuropsychological battery is still recommended in the diagnosis of dementia in PD.
Arnold-Chiari type I malformations usually manifest clinically with short-lasting headaches typically involving the occipital-nuchal region and precipitated by the Valsalva maneuver, coughing, sudden postural change, or physical exertion. We describe the case of an adult patient who presented with symptomatic trigeminal neuralgia caused by an Arnold-Chiari type I malformation. Unlike previous cases, the malformation involved the trigeminal ophthalmic division alone.
Bilateral striopallidodentate calcification, usually termed Fahr's disease, can give rise to various clinical manifestations including hyperkinetic movement disorders or a hypokinetic Parkinsonian syndrome, behavioural and mood changes, cognitive deficits and even frank dementia. We describe four patients all of whom underwent a detailed scintigraphic, neuroradiological and clinical work-up: two had primary, sporadic Fahr's disease and two had Fahr's disease secondary to hypoparathyroidism. The neuroradiological and clinical studies disclosed similar anatomical and pathological changes in the four patients but variable and sometimes unexpected clinical manifestations. Both patients with primary forms had hypokinetic Parkinsonian syndrome, both patients with secondary forms had hyperkinetic movements. Dopamine autotransporter scan brain scintigraphy disclosed an unexpected unilateral putamen involvement despite substantially symmetric calcifications.
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