Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology remains unknown, recent genetic studies identified RNF213 in the 17q25-ter region as an important susceptibility gene of MMD among East Asian populations. Possibly because of genetic differences, MMD is relatively common in people living in East Asian countries such as Korea and Japan, compared to those in the Western Hemisphere. The prevalence of MMD appears to be slightly lower among Chinese, compared to Koreans or Japanese. There are two peaks of incidence with different clinical presentations, at around 10 years and 30-40 years. The peak appears to occur later in women than men. In children, ischemic symptoms, especially transient ischemic attacks, are predominant. Intellectual decline, seizures, and involuntary movements are also more common in this age group. In contrast, adult patients present with intracranial hemorrhage more often than pediatric patients. In patients with MMD, intracerebral hemorrhage is more often accompanied by intraventricular hemorrhage than in patients with hypertensive intracerebral hemorrhage. These different age peaks and different clinical presentations in each age group are also observed in MMD patients in the USA. Catheter angiography is the diagnostic method of choice. Magnetic resonance (MR) angiography and computed tomographic angiography are noninvasive diagnostic methods. High-resolution vessel wall MR imaging also helps diagnose MMD by revealing concentric vessel wall narrowing with basal collaterals.

Although there have been attempts to make clinical–MRI correlation in patients with lateral medullary infarction (LMI), studies with a large number of patients are unavailable. In this study, clinical features, MRI findings and angiogram results of 130 acute, consecutive patients with pure LMI were studied and correlated. MRI‐identified lesions were classified rostro‐caudally as rostral, middle and caudal, and horizontally as typical, ventral, large, lateral and dorsal. The distribution of horizontal subtypes was significantly different (P <0.001) among three rostro‐caudal lesions in that rostral lesions tend to be ventral types and caudal lesions are lateral types. Patients with rostrally located lesions had dysphagia, facial paresis (P < 0.01, each) and dysarthria (P < 0.05) significantly more often, and severe gait ataxia and headache (P < 0.05, each) less often than those with caudal lesions. The frequencies of dysphagia (P < 0.01), dysarthria (P < 0.01) and bilateral trigeminal sensory pattern (P < 0.05) were significantly different among horizontal subtypes in that these symptoms were frequent in patients with ‘large type’ as compared with those with lateral type lesions. Angiograms performed in 123 patients showed vertebral artery (VA) disease in 67% and posterior inferior cerebellar artery (PICA) disease in 10%. The presumed pathogenetic mechanisms included large vessel infarction in 50%, arterial dissection in 15%, small vessel infarction in 13% and cardiac embolism in 5%. Dissection occurred more often in patients with caudal (versus rostral) lesions (P < 0.01), whereas dorsal type infarcts (versus other types) were related more often to cardiogenic embolism and normal angiogram findings (P < 0.05, each). Patients with isolated PICA disease (versus those with VA disease) more often had cardiogenic embolism (P < 0.05) and less often had dissection (P < 0.01). It is concluded that rostro‐caudal and horizontal classification of MRI helps us to understand the clinical and, partly, the aetiopathogenetic aspect of the heterogeneous LMI syndrome.

Background and Purpose—: The aim of this study was to investigate differences in risk factors and stroke mechanisms between intracranial atherosclerosis (ICAS) and extracranial atherosclerosis (ECAS) and between anterior and posterior circulation atherosclerosis. Methods—: A multicenter, prospective, Web-based registry was performed on atherosclerotic strokes using diffusionweighted magnetic resonance imaging and magnetic resonance angiography. Stroke mechanisms were categorized as artery-to-artery embolism, in situ thrombo-occlusion, local branch occlusion, or hemodynamic impairment. Results—: Onethousand patients were enrolled from 9 university hospitals. Age (odds ratio [OR], 1.033; 95% confidence interval [CI], 1.018–1.049), male gender (OR, 3.399; 95% CI, 2.335–4.949), and hyperlipidemia (OR, 1.502; 95% CI, 1.117–2.018) were factors favoring ECAS (vs ICAS), whereas hypertension (OR, 1.826; 95% CI, 1.274–2.618; P =0.001) and diabetes mellitus (OR, 1.490; 95% CI, 1.105–2.010; P =0.009) were related to posterior (vs anterior) circulation diseases. Metabolic syndrome was a factor related to ICAS (vs ECAS) only in posterior circulation strokes (OR, 2.433; 95% CI, 1.005–5.890; P =0.007). Stroke mechanisms included arterytoartery embolism (59.7%), local branch occlusion (14.9%), in situ thrombo-occlusion (13.7%), hemodynamic impairment (0.9%), and mixed (10.8%). Anterior ICAS was more often associated with artery-to-artery embolism (51.8% vs 34.0%) and less often associated with local branch occlusion (12.3% vs 40.4%) than posterior ICAS ( P <0.001). Conclusions—: The prevalence of risk factors and stroke mechanisms differ between ICAS and ECAS, and between anterior and posterior circulation atherosclerosis. Posterior ICAS seems to be closely associated with metabolic derangement and local branch occlusion. Prevention and management strategies may have to consider these differences.

Kim BJ, et al. J Stroke. 2014 Jan;16(1):8-17. http://dx.doi.org/10.5853/jos.2014.16.1.8

Post-stroke mood and emotional disturbances are frequent and diverse in their manifestations. Out of the many post-stroke disturbances, post-stroke depression, post-stroke anxiety, post-stroke emotional incontinence, post-stroke anger proneness, and post-stroke fatigue are frequent and important symptoms. These symptoms are distressing for both the patients and their caregivers, and negatively influence the patient's quality of life. Unfortunately, these emotional disturbances are not apparent and are therefore often unnoticed by busy clinicians. Their phenomenology, predicting factors, and pathophysiology have been under-studied, and are under-recognized. In addition, well-designed clinical trials regarding these symptoms are rare. Fortunately, these mood and emotional disturbances may be treated or prevented by various methods, including pharmacological therapy. To administer the appropriate therapy, we have to understand the phenomenology and the similarities and differences in the pathophysiological mechanisms associated with these emotional symptoms. This narrative review will describe some of the most common or relevant post-stroke mood and emotional disturbances. The phenomenology, factors or predictors, and relevant lesion locations will be described, and pharmacological treatment of these emotional disturbances will be discussed based on presumable pathophysiological mechanisms.

Background and Purpose— The efficacy and safety of the selective serotonin reuptake inhibitor fluoxetine have rarely been studied in the treatment of poststroke emotional disturbances. Methods— Stroke patients (152) who had poststroke depression (PSD), emotional incontinence (PSEI), or anger proneness (PSAP) were studied. PSD was evaluated by Beck Depression Inventory and Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition , PSEI by Kim’s criteria, and PSAP was assessed by Spielberger Trait Anger Scale. Subjects were randomly given either fluoxetine 20 mg/day (n=76) or placebo (n=76) for 3 months. Follow-up evaluations were done 1, 3, and 6 months after the beginning of the treatment. The primary outcome measurement was the scores of emotional disturbances at each follow-up assessment. The secondary outcome measurements were the percentage changes of the scores and the subjective responses of the patients. Results— Although patients in the fluoxetine group more often dropped out because of adverse effects, fluoxetine administration was generally safe. Fluoxetine significantly improved PSEI and PSAP, whereas no definitive improvement of PSD was found. Improvement of PSAP was noted even at 3 months after the discontinuation of the treatment. Conclusions— Fluoxetine is efficacious in the treatment of PSEI and PSAP. Its effect on PSD is not solidly confirmed.