The C9ORF72 hexanucleotide expansion is a major pathological expansion pattern found in patients with frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (C9FTD/ALS). We describe a ...patient in whom early clinical evaluation, MRI and fluorodeoxyglucose (FDG) positron emission tomography (PET) findings failed to definitively differentiate between FTD and Alzheimer's disease (AD), whereas
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C-Pittsburgh compound B (PiB) PET was negative for amyloid pathology. He later developed ALS symptoms, and post mortem neuropathological findings were diagnostic of FTD-ALS, while no findings suggested AD. His sister was diagnosed with FTD, and the C9ORF72 expansion was detected in both siblings. We conclude that
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C-PiB PET imaging may help the early differential diagnosis between AD and FTD, including C9FTD/ALS.
The aim of this study was to examine the neural substrates of multiobject naming by positron emission tomography in normals. Multiobject naming is used in a technique called contextual priming (CP) ...to elicit contextual effects on picture naming through systematic manipulation of the relatedness of to-be-named pictures in an array. Inhibitory and facilitatory effects of CP on naming have been used both to highlight the inner dynamics of the normal word retrieval system and to treat naming difficulties caused by acquired anomia. Because of the complexity of the CP technique, it is unclear whether it merely reflects lexical retrieval or whether it also recruits general cognitive resources such as attentional control and response selection to a significant degree. When compared with a baseline situation in which colored circles were named, multiobject naming elicited increased blood flow in the occipital lobe and in the left inferior temporal and fusiform gyri. These areas have been related to visual pattern recognition and semantic access, and the pattern of activation is in line with previous functional imaging studies on single picture naming. In conclusion, multiobject naming does not appear to activate brain regions beyond those needed for single object naming. These findings thus provide independent evidence for the claim that CP is a valid paradigm for the study of word production proper.
The most common cause of monogenic hereditary Parkinson's disease is a mutation in the PARK2 gene. Early onset, slow progression, dystonia, and good response to levodopa are typical of the disease ...phenotype. Finnish PARK2 patients have not been described previously. We describe two patients, in whom pathogenic mutations in the PARK2 gene were the cause of parkinsonism.
Neurobiology of pathological gambling Kaasinen, Valtteri; Halme, Jukka; Alho, Hannu
Duodecim (Helsinki, Finland : 1961),
2009, Letnik:
125, Številka:
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Magazine Article
Approximately one third of problem gamblers in Finland suffer from pathological gambling. An essential factor affecting the genesis of pathological gambling is a dysfunction of the dopaminergic ...reward system. It may be associated with the pleasure arising from gambling along with the reward and expectance of reward. In Parkinsons's disease patients receiving dopaminergic medication, pathological gambling and disturbances of impulse control are more common than in the average population. Various psychosocial modes of treatment and medications have been developed for the treatment of pathological gambling, but based on current knowledge, none of them displays particular efficacy.
The treatment of Parkinson's disease may be initiated with dopamine agonist or MAO-B-inhibitor for people under 60-65 years of age. For older patients, the treatment may also be started with ...levodopa. If there are motor complications, such as on-off-symptoms, apomorphin injections can be beneficial in addition to other medications. In the case of difficult on-off-symptoms and dyskinesias in spite of optimal treatment, deep brain stimulation, duodenal levodopa infusion and apomorphine infusion should be considered. Rehabilitation can improve gait speed and balance, decrease falls and improve speech. However, with advancing disease the results are not maintained if trainino is discontinued.
The incidence of Creutzfeldt-Jakob disease (CJD) in Finland in 1974-1989 was reported to be 0.6/1 000 000. Our aim was to compare the current incidence of CJD in Finland with the earlier incidence ...and also study the diagnostics of the disease.
Register study of the Finnish CJD cases from 1997 to 2012 and the clinical data of CJD patients within the Hospital District of Southwest Finland from 2007 to 2013.
There were 119 cases. The average yearly incidence was 1.36-1.44/1 000 000.
Compared with the previous study, the incidence in Finland appears to have increased. The change is propably due to increased awareness and improved diagnostic methods.
