Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Aug 2009
Cognitive determinants of social functioning after a first ever mild to moderate stroke at vocational age.
Social functioning is impaired in approximately two-thirds of stroke patients of vocational age, even several months after a first ever mild to moderate stroke. The known predictors of social functioning are initial stroke severity, anxiety and depression, and the Mini-Mental State Evaluation, suggesting that cognitive deficits contribute to post-stroke social dysfunctioning. The aim of this study was to evaluate whether cognitive domains correlated with social functioning and to determine the cognitive predictors of social dysfunctioning. ⋯ All cognitive domains were associated with social functioning, working memory being the main cognitive determinant. Our results suggest that cognitive impairment impacts on social dysfunctioning, which is known to be a component of quality of life.
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J. Neurol. Neurosurg. Psychiatr. · Aug 2009
Autosomal-dominant GTPCH1-deficient DRD: clinical characteristics and long-term outcome of 34 patients.
An autosomal dominantly inherited defect in the GCH1 gene that encodes guanosine triphosphate cyclohydrolase 1 (GTPCH1) is the most common cause of dopa-responsive dystonia (DRD). A classic phenotype of young-onset lower-limb dystonia, diurnal fluctuations and excellent response to levodopa has been well recognised in association with GCH1 mutations, and rare atypical presentations have been reported. However, a number of clinical issues remain unresolved including phenotypic variability, long-term response to levodopa and associated non-motor symptoms, and there are limited data on long-term follow-up of genetically proven cases. ⋯ The classic phenotype was most frequent (n = 23), with female predominance (F:M = 16:7), and early onset (mean 4.5 years) with involvement of legs. However, a surprisingly large number of patients developed craniocervical dystonia, with spasmodic dysphonia being the predominant symptom in two subjects. A subset of patients, mainly men, presented with either a young-onset (mean 6.8 years) mild DRD variant not requiring treatment (n = 4), or with an adult-onset (mean 37 years) Parkinson disease-like phenotype (n = 4). Two siblings were severely affected with early hypotonia and delay in motor development, associated with compound heterozygous GCH1 gene mutations. The study also describes a number of supplementary features including restless-legs-like symptoms, influence of female sex hormones, predominance of tremor or parkinsonism in adult-onset cases, initial reverse reaction to levodopa, recurrent episodes of depressive disorder and specific levodopa-resistant symptoms (writer's cramp, dysphonia, truncal dystonia). Levodopa was used effectively and safely in 20 pregnancies, and did not cause any fetal abnormalities.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2009
The Severity of Dependence Scale detects people with medication overuse: the Akershus study of chronic headache.
To evaluate the Severity of Dependence Scale (SDS) in people with primary chronic headache and analyse the pattern of medication overuse. ⋯ The SDS questionnaire detects medication overuse and dependency-like behaviour in persons with primary chronic headache.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2009
Comparative Study Clinical TrialBrain penetration effects of microelectrodes and DBS leads in STN or GPi.
To determine how intraoperative microelectrode recordings (MER) and intraoperative lead placement acutely influence tremor, rigidity, and bradykinesia. Secondarily, to evaluate whether the longevity of the MER and lead placement effects were influenced by target location (subthalamic nucleus (STN) or globus pallidus interna (GPi)). ⋯ This study demonstrated significant acute intraoperative penetration effects resulting from MER and lead placement/collision in PD. Clinicians rating patients in the operating suite should be aware of these effects, and should consider pre- and post-lead placement rating scales prior to activating DBS. The collision/implantation effects were greater intraoperatively with STN compared with GPi, and with greater disease duration there was a larger effect.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2009
Multicenter StudyFunctional and cognitive criteria produce different rates of mild cognitive impairment and conversion to dementia.
To compare rates of mild cognitive impairment (MCI) and rates of progression to dementia using different MCI diagnostic systems. ⋯ Different criteria produce different MCI rates and different conversion rates to dementia. Although a higher percentage of MCI was identified by CDR than NP, a higher percentage of NP MCI progressed to dementia. These findings suggest that the CDR is sensitive to subtle changes in cognition not identified by the NP algorithm but is also sensitive to demographic and clinical factors probably leading to a greater number of false positives. These results suggest that identifying all individuals with CDR scores of 0.5 as Alzheimer's disease is not advisable.