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The identification of a truncating mutation in a patient with ID, severe microcephaly, epilepsy, and growth retardation, combined with its dual function in regulating the neural proliferation/neuronal differentiation, adds DYRK1A to the list of genes responsible for such a phenotype. ID, microcephaly, epilepsy, and language delay are the more specific features associated with DYRK1A abnormalities. DYRK1A studies should be discussed in patients presenting such a phenotype.

Background and Purpose-Comparison of incidence and case-fatality rates for stroke in different countries may increase our understanding of the etiology of the disease, its natural history, and management. Within the context of an aging population and the trend for governments to set targets to reduce stroke risk and death from stroke, prospective comparison of such data across countries may identify what drives the variation in risk and outcome. Methods-Population-based stroke registers, using multiple sources of notification, ascertained cases of first in a lifetime stroke between 1995 and 1997 for all age groups. The study populations were in Erlangen, Germany; Dijon, France; and London, UK. Crude incidence rates were age-standardized to the European population for comparative purposes. Case-fatality rates up to 1 year after the stroke were obtained, and logistic regression adjusting for age group, sex, and pathological subtype of stroke was used to compare survival in the 3 communities. Results-A total of 2074 strokes were registered over the 3 years. The age-standardized rate to the European population was 100.4 (95% CI 91.7 to 109.1) per 100 000 in Dijon, 123.9 (95% CI 115.6 to 132.2) in London, and 136.4 (95% CI 124.9 to 147.9) in Erlangen. Both crude and adjusted rates were lowest in Dijon, France. The incidence rate ratio, with Dijon as the baseline comparison (1), was 1.21 (95% CI 1.09 to 1.34) in London and 1.37 (95% CI 1.22 to 1.54) in Erlangen (PϽ0.0001). There were significant differences in the proportion of the subtypes of stroke between populations, with London having lower rates of cerebral infarction and higher rates of subarachnoid hemorrhage and unclassified stroke (PϽ0.001). Case-fatality rates varied significantly between centers at 1 year, after adjustment for age, sex, and subtype of stroke (35% overall, 34% Erlangen, 41% London, and 27% Dijon; PϽ0.001). Conclusions-The impact of stroke is considerable, and the risk of stroke varies significantly between populations in Europe as does the risk of death. The striking differences in survival require clarification but lend weight to the evidence that stroke management may differ between northern and central Europe and influence outcome. (Stroke.

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Objectives-To analyse the clinical features induced by lenticular infarction found in 20 patients, and to analyse the radiological and clinical correlations. Methods-Eight women and 12 men, mean age 73 years, were included in this study, which was carried out from 1 January 1994 to 30 November 1996. They were characterised by the onset of a lenticular infarction, shown by CT and MRI. A complete neurological and neurocognitive examination, and photon emission computed tomography (SPECT), were performed in all the patients and there was a long clinical follow up. Results-Two distinct clinical syndromes were identified corresponding to the two anatomical areas of the lenticular nucleus: behavioural and cognitive disorders were associated with infarcts within the globus pallidus, whereas both motor disorders (dystonia) and cognitive disorders were associated with infarcts within the putamen. Outcome was excellent in all the patients for motor function, but slight cognitive disorders, problems with short term memory, and dysphasia persisted for several months. The size of the lesion did not explain these symptoms. By contrast, the slight reduction in cerebral blood flow found in the adjacent frontotemporal area may explain them by a deaVerentation or a diaschisis phenomenon. Conclusion-It is possible to identify the clinical symptoms of a single lesion in the pallidus nucleus and in the putaminal nucleus, in which behavioural, cognitive, and movements disorders are important. After an acute and spectacular onset, outcome is in general excellent. A disease of the small arteries must be involved. (J Neurol Neurosurg Psychiatry 1997;63:611-615) Keywords: infarction: lentiform nucleus: motor disorders: cognitive disorders A knowledge of the functions of the basal ganglia is important.The lesions that damage the human brain are rarely restricted to a single anatomical structure. Published case reports of lenticular infarction often include patients whose infarcts were not limited to the lenticular nucleus.1 However, modern imaging techniques such as CT and MRI are able to identify lesions restricted to the lenticular nucleus.We present the clinical findings, the radiological features, the aetiologies, and the long term prognosis of 20 patients with an isolated lenticular infarct established by CT and MRI. MethodsWe studied all patients with an acute lenticular infarct-diagnosed by both CT and MRIwho were admitted to the neurology department between 1 January 1994 and 30 November 1996 (eight women and 12 men). We excluded patients with previous strokes or with associated infarcts in the internal capsule, the caudate nucleus, or the thalamus. All patients were studied by all of us and they had the same investigations including standard blood tests, ECG, transoesophageal echography (TEE), Doppler ultrasound, CT at day 1 and day 10, MRI at day 7, and SPECT at day 10.Brain CT and MRI were performed using orbitomeatal horizontal sequences and coronal sequences also for MRI. An ischaemic stroke was defined by a hypode...

Background and Purpose-The changing incidence of ischemic stroke is of major concern in view of its public health impact, to define the population concerned, to identify risk factors, and to set up health-care systems. The aim of this study was to evaluate the time trends associated with the incidence of all the subtypes of ischemic stroke and transient ischemic attacks in a well-defined population for 10 years. Methods-Since 1985, a population registry has recorded each patient living in Dijon (France) who suffered from a cerebrovascular disease (CVD) regardless of the type of management. This study involved all patients suffering from their first ischemic stroke and their first transient ischemic attacks (TIAs) during 1 calendar year between January 1, 1985 and December 31, 1994. The incidence changes according to age, sex, and type of cerebral ischemic event (cortico-subcortical infarct, lacunar infarct, and TIA) were studied on the basis of their annual variations. Results-During the 10-year study period, 834 cortico-subcortical infarcts (52.1%), 296 lacunar infarcts (18.5%), 369 TIAs (23.1%), and 101 undetermined ischemic strokes (6.3%) were collected. The incidence of all ischemic events was relatively stable in both sexes over the 10-year period. However, the incidence rates differed according to age and type of ischemia. An increased incidence of cerebral cortico-subcortical infarct was observed in patients older than 75 years of age (ϩ5.45% annual change [AC] in men, PϽ0.05; ϩ5.09% AC in women, NS). In parallel, a higher proportion of emboligenic cardiac arrhythmias was observed in these patients (PϽ0.001). The incidence of lacunar infarcts tended to decrease, regardless of age but mainly in men under younger than 75 years of age (Ϫ12.74% AC in men, NS; ϩ0.31% AC in women, NS). The incidence of TIAs was relatively stable in both sexes. Because our population consisted of a large number of elderly subjects, the increase in cardioembolic causes could partially explain the increased incidence of cerebral cortico-subcortical infarcts in patients older than 75 years of age. Conclusions-These preliminary data emphasize the importance of stroke surveillance in considering the variations of the different mechanisms of ischemic cerebrovascular disease. Although the incidence of TIA is stable and the incidence of lacunes tends to decrease in men, mainly before 75 years of age, we emphasize the rise of the crude incidence of cortico-subcortical infarcts in men older than 75 years of age, induced by an increase in cardioembolic causes. (Stroke. 1999;30:371-377.)

Two thousand three hundred and eighty-nine patients with first-ever stroke were registered in the population-based Dijon Stroke Registry over an 11-year period. There was a history of migraine in 49 cases (2%), with a majority of women (2.8% versus 1.1% men) with the following distribution: 27 cases among 1,380 large-artery cerebral infarctions (1.9%), 6 cases among 358 small-artery cerebral infarctions (1.6%), 6 cases among 412 cerebral infarctions due to cardiac embolism (1.4%), 7 cases among 191 cerebral hemorrhages (3.6%) and 3 cases among 47 subarachnoid hemorrhages (6.3%). The male/female ratio was 0.58 for the 49 strokes with a history of migraine versus 1.27 for the 2,340 strokes with no history of migraine. Twelve migraine-induced ischemic strokes occurred with an infarction of the posterior area of the brain in young patients. The annual incidence was 0.80/100,000/year (confidence interval, CI = 0.37–1.57) with a predominance of women (1.02/100,000/year, CI = 0.52–1.25; men: 0.57/100,000/year; CI = 0.28–1.04). We conclude that a history of migraine is more frequent in women, in particular in those with hemorrhagic strokes, and that the incidence of migraine-induced stroke in our population-based study is higher in women, although it remains low.