Abstract:
OBJECTIVE:To characterize the progression of the cognitive involvement in patients affected by myotonic dystrophy type 1 (DM1) by a longitudinal neuropsychological follow-up study. METHODS:In a previous study we documented an ageing-related decline of frontal and temporal cognitive functions in juvenile/adult forms of DM1, irrespectively of the n(CTG) in leukocytes and the severity of muscle weakness. Here we present the results of a neuropsychological follow-up study performed in 34 out of 70 DM1 patients previously studied. Patients were divided into four groups according to their genotype (E1:50-150; E2:150-500; E3:500-1000; E4: >1000 CTG). The neuropsychological test battery included MMSE, memory, linguistic, level, praxis, attentional and frontal-executive tasks. Statistical analysis was performed by One way MANOVA with repeated measures analysis and by Wilcoxon match paired test. RESULTS:The whole group of patients showed a significant deterioration in linguistic functions, together with a tendency towards decline in executive abilities, confirming a predominant involvement of cognitive functions subserved by fronto-temporal areas. We found no significant correlation between the progression of cognitive decline and the n(CTG) in leukocytes. Moreover, we observed that patients belonging to E2 group, with the highest mean age, got scores lower than E3 patients, with particular regard both to linguistic and executive tasks. CONCLUSIONS:These data support our previous hypothesis that the cognitive damage is confined to frontotemporal functions in adult DM1 patients, with a tendency towards a decline with aging.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Modoni A,Silvestri G,Vita MG,Quaranta D,Tonali PA,Marra Cdoi
10.1007/s00415-008-0017-5subject
Has Abstractpub_date
2008-11-01 00:00:00pages
1737-42issue
11eissn
0340-5354issn
1432-1459journal_volume
255pub_type
杂志文章abstract:BACKGROUND:Propranolol is recommended as first-line treatment for preventing migraine attacks; acupuncture has not been compared with propranolol in a head-to-head trial. OBJECTIVE:To compare acupuncture with propranolol using indirect treatment comparison meta-analysis. METHOD:We searched MEDLINE, EMBASE, and Cochra...
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pub_type: 杂志文章,meta分析
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pub_type: 杂志文章,多中心研究
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abstract::Botulinum neurotoxin (BoNT) is an effective treatment for cervical dystonia (CD). Long-term changes of several variables, including the dose of BoNT, in these patients is largely unknown. We reviewed the clinical charts of 275 patients with CD treated with BoNT type A (BoNT-A) for at least 5 years since 1989 at ten te...
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pub_type: 杂志文章,多中心研究
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pub_type: 杂志文章
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pub_type: 杂志文章,评审
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更新日期:2005-02-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0809-y
更新日期:2002-09-01 00:00:00
abstract::Moderate unilateral cerebral ischemia was produced by microembolism in 24 adult cats. Two million plastic microspheres with a diameter of 15 +/- 5 microns were injected into the left common carotid artery via the lingual artery. The physiological and metabolic responses to embolism were accessed by electrocorticograp...
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pub_type: 杂志文章
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更新日期:1980-01-01 00:00:00
abstract::Sexuality in patients with amyotrophic lateral sclerosis (ALS) has received little attention so far. Although sexual function is not directly affected by the disease process, several patients have reported problems within their sexual relationship. We performed a questionnaire survey to ascertain the extent and clinic...
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pub_type: 杂志文章
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pub_type: 杂志文章,评审
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pub_type: 杂志文章
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