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Objectives: The present study aimed to examine the effectiveness of proton magnetic resonance spectroscopy (1HMRS) in determining the progression of neurological symptoms resulting in acute ischemic stroke in patients with lenticulostriate artery (LSA) infarction., Materials and Methods: 1HMRS was performed within 72 h after neurological symptom onset. Voxel of interest was placed in tissue that included the pyramidal tract and identified diffusion weighted echo planar spin-echo sequence (DWI) coronal images. Infarct volume in DWI was calculated using the ABC/2 method. 1HMRS data (tNAA, tCr, Glx, tCho, and Ins) were analyzed using LCModel. Progressive neurological symptoms were defined as an increase of 1 or more in the NIHSS score. Patients who underwent 1HMRS after progressive neurological symptoms were excluded., Results: In total, 77 patients were enrolled. Of these, 19 patients had progressive neurological symptoms. The patients with progressive neurological symptoms were significantly more likely to be female and had higher tCho/tCr values, higher rates of axial slices ≥ 3 slices on DWI, higher infarct volume on DWI, higher maximum diameter of infarction of axial slice on DWI, and higher SBP on admission compared to those without. Multivariable logistic analysis revealed that higher tCho/tCr values were independently associated with progressive neurological symptoms after adjusting for age, sex, and initial DWI infarct volume (tCho/tCr per 0.01 increase, OR 1.26, 95% CI 1.03-1.52, P = 0.022)., Conclusions: Increased tCho/tCr score were associated with progressive neurological symptoms in patients with LSA ischemic stroke. Quantitative evaluation of 1HMRS parameters may be useful for predicting the progression of neurological symptoms., Competing Interests: Declaration of Competing Interest Dr. Maruyama received research support from Eisai Co., Ltd, Pfizer Inc., Takeda Pharmaceutical Company Limited, Otsuka Pharmaceutical Co., Ltd, Nihon Pharmaceutical Co., Ltd., Shionogi Co., Ltd., Teijin Pharma Limited, Fujifilm Holdings Corporation, Boehringer Ingelheim, Sumitomo Dainippon Pharma Co., Ltd., Nihon Medi-Physics Co., Ltd., Bayer AG, Merck Sharp & Dohme Corp., Daiichi Sankyo Company, Ltd., Kyowa Hakko Kirin Co., Ltd., Sanofi S.A., Novartis International AG, Kowa Pharmaceutical Co., Ltd., Astellas Pharma Inc., Tsumura & Co., Japan Blood Products Organization, Mitsubishi Tanabe Pharma Corporation, and Mylan N.V. The other authors declare they have no conflicts of interest., (Copyright © 2021 Elsevier Inc. All rights reserved.)

A 65-year-old woman presented to the emergency department with sudden onset of left-sided weakness, headache and vomiting. A cerebral CT showed an acute intracerebral haemorrhage involving the right caudate nucleus and lentiform nucleus with mild midline shift and intraventricular extension. CT angiography did not reveal aneurysm or other vascular anomaly. Conventional cerebral angiography demonstrated a 3 mm right medial lenticulostriate branch aneurysm, arising from the right anterior cerebral artery (ACA). Endovascular treatment was performed from the left internal carotid via the anterior communicating artery into the right ACA. Complete occlusion was achieved with injection of N-butyl-2-cyanoacrylate. The patient had neurological rehabilitation during hospitalisation followed by outpatient physical therapy. Two years later, clinical follow-up demonstrated excellent recovery., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

We describe herein an extremely severe case of Aicardi-Goutières syndrome 7 (AGS7). The female patient was the daughter of nonconsanguineous parents and developed cardiomegaly, pericardial effusion, splenomegaly, and intracranial calcification during the fetal period. Because her cardiotocogram showed a non-reassuring fetal status, she was delivered at 29 weeks and 4 days of gestation by an emergency cesarean section. After birth, she suffered from respiratory distress, pulmonary hypertension, refractory fever, recurrent thrombocytopenia, and abdominal distention caused by hepatomegaly and ascites. She showed a lenticulostriate vasculopathy, which was compatible with the fetal intracranial calcification. Despite various intensive care procedures, she died of gradually progressive pulmonary hypertension at 3 months of age. After her death, whole exome sequencing on the patient and the parents was performed and revealed a novel, de novo, heterozygous mutation in the IFIH1 gene (IFIH1:NM_022168:exon12:c.2439A > T:p.Glu813Asp). On the basis of the mutation and the clinical features, the diagnosis was AGS7. Although AGS7 has been regarded as a relatively mild subtype of Aicardi-Goutières syndrome, this case indicates that the c.2439A > T variant of AGS7 can be fatal in early infancy., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Objective  The objective of this study was to perform a systematic review of all studies that report neurodevelopmental outcomes at 12 months' corrected age or later for preterm infants (< 32 weeks) who are diagnosed with lenticulostriate vasculopathy (LSV) on cranial ultrasound. Study Design  This is a systematic review. Results  We identified 501 unique publications of which 3 met all prespecified eligibility criteria. Neurodevelopmental outcome data were available for 44 preterm infants with LSV. There were mixed results from the studies: the first reported impairments of mental development; the second reported impairments of motor development only; and finally, the third reported that three of three preterm infants with LSV had mild or moderate developmental delay. Conclusion  The available results raise some concern for future impairments in preterm infants with LSV, but they are conflicting and inconclusive. There is insufficient evidence about the neurodevelopmental implications of LSV in preterm infants to inform counseling of parents., Competing Interests: Conflict of Interest: None. Funding None., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Objective: Hypertension can alter the vascular structure, mechanics, and function of small arteries and arterioles. It remains unknown whether microvascular changes are associated with brain metabolism. The purpose of this study was to analyze the correlation between the reduction in small arteries and changes in brain metabolism in patients with hypertension., Subjects and Methods: The study population comprised 50 patients with hypertension and 50 volunteers without hypertension. The two groups underwent 3-T 3D time-of-flight MR angiography, and the numbers of lenticulostriate arteries (LSAs) were determined for both groups. Single-voxel proton MR spectroscopic data on the basal ganglia regions were also acquired. The ratios of N-acetylaspartate to creatine (NAA/Cr), myo-inositol to creatine (Mi/Cr), and choline to creatine (Cho/Cr) were measured. Statistical analysis was performed to evaluate the differences between the two groups with respect to metabolite ratios., Results: The average total number of LSA stems on both sides in patients with hypertension was 5.12 ± 0.98 compared with 6.10 ± 0.95 in volunteers without hypertension (p < 0.0001). The NAA/Cr ratio decreased according to a reduction in the number of LSAs in the hypertension group, which was significantly reduced when the number of LSAs was 3 or fewer., Conclusion: Hypertension can lead to a statistically significant reduction in NAA/Cr ratio in the basal ganglia regions when the number of LSAs decreases to a certain extent. Reduced numbers of LSAs correlated with brain metabolism changes caused by hypertension, which can provide important insights for understanding the pathophysiologic mechanism of hypertension and may be valuable in evaluating this disease.

Aim: This study investigated the relationship between lenticulostriated vasculopathy (LSV) and hearing loss in 141 infants with congenital cytomegalovirus (cCMV) infection., Methods: We included all infants with cCMV infection who were followed in our clinic for more than a year with only LSV signs of brain involvement on initial brain ultrasound. Group one comprised 13 infants with no hearing impairment at birth who were not treated with gan/valganciclovir during 2006-2009. Group two was 51 infants with LSV and no hearing impairment who had been treated since mid-2009. Group three was 25 infants born with LSV and hearing loss, who had been treated from birth. Group four was 52 control infants born during the same period with asymptomatic cCMV. Hearing tests were performed during the neonatal period and every four to six months until four years of age., Results: Hearing deterioration was more extensive in group one (85%) than in group two (0%, p < 0.001) and the asymptomatic group (10%, p < 0.001) and occurred more often in group four (10%) than in group two (0%, p = 0.008)., Conclusion: Lenticulostriated vasculopathy was common in infants with cCMV infection and may serve as a sign of central nervous system involvement and further hearing deterioration. Antiviral treatment may be prudent in such infants., (©2015 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Background: Aneurysms involving the lenticulostriate artery (LSA) are rare. The majority of LSA aneurysms reported are idiopathic. We present 2 paediatric cases of dissecting distal LSA aneurysm. An 8-year-old girl and 9-year-old boy presented with basal ganglia infarction. History and imaging identified dissection as the most likely aetiology. The clinico-radiological features, aetiology and management involving are discussed. Conservative treatment with careful clinico-radiological monitoring may be a feasible therapeutic strategy., (© The Author(s) 2014.)

We present a case of a late preterm baby with respiratory distress syndrome (RDS), prolonged jaundice and congenital hypothyroidism. The infant developed late lenticulostriate vasculopathy (LSV). LSV was previously described in association with various neurodevelopmental abnormalities and in this case would have been missed by the current US brain screening recommendations for newborns.

Septic cavernous sinus thrombosis is a rare clinical condition. Although Staphylococcus aureus is the most common pathogen causing septic cavernous sinus thrombosis [CST], it is an uncommon cause of meningitis. We report the first case of CST with meningitis in Hyderabad, Andhra Pradesh, caused by community acquired epidemic strain of Methicillin resistant staphylococcus aureus [MRSA], in a previously healthy individual with no risk factors. The patient recovered completely following treatment with Vancomycin. We consecutively reviewed all cases of community acquired staphylococcus aureus [CA-MRSA] with central nervous system involvement available in literature.

Infections involving endovascular devices are rare and, to our knowledge, only three cases of infection with an inserted carotid stent have ever been reported. A 68-year-old man underwent carotid artery stenting (CAS) of the left proximal internal carotid artery. Two days after CAS the patient developed a high fever and investigation showed that the inserted carotid stent was infected. The infection could not be controlled despite adequate antibiotic therapy. Eventually a rupture of the carotid artery occurred and the patient underwent emergency resection of the left carotid bifurcation in addition to stent removal and reconstruction with a saphenous vein interposition graft. The patient recovered fully without any neurological sequelae.

Background: Capsular warning syndrome (CWS) is rare (1.5% of TIA presentations) but has a poor prognosis (7-day stroke risk of 60%). Up to date, the exact pathogenic mechanism of CWS has not been fully understood. We report the clinical presentations and high-resolution MRI (HR MRI) findings of two cases with capsular warning symptoms., Case Presentation: Case 1 was a 63-year-old man with a history of hypertension with recurrent episodes of left hemiparesis and dysarthria lasting 10 ~ 30 minutes. Case 2 was a 54-year-old woman with repetitive episodes of transient left hemiparesis and dysarthria lasting about 10 minutes. Capsular infarctions on DWI were demonstrated in the territory of a lenticulostriate artery in both 2 patients. HR MRI disclosed atherosclerotic plaques on the ventral wall of the MCA where enticulostriate arteries were arisen from, although traditional digital subtraction angiography showed normal. Aggressive medical therapy with dual antithrombotic agents and statin was effective in these two cases., Conclusion: Our HR MRI data offer an insight into the pathophysiology of CWS which might be caused by atherosclerotic plaque in non-stenotic MCA wall. HR MRI might be a useful modality for characterizing atherosclerotic plaques in the MCA and detecting the pathophysiology of the CWS.

We describe a case of bilateral caudate nucleus infarction caused by cardioembolic stroke associated with a variant circle of Willis. The patient was an 81-year-old man with atrial fibrillation who presented with a sudden disturbance of consciousness. When he became more alert a few days later, he was abulic with no spontaneous speech or activity. A magnetic resonance imaging scan of the brain revealed cerebral infarction of bilateral caudate nucleus heads and the left frontal lobe. The left A1 segment was absent on 3-dimensional computed tomography angiography. One year later, abulia had completely resolved. Bilateral caudate nucleus infarction with variant circle of Willis is rare., (Copyright © 2012 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

A 57-year-old man was admitted because of left hemiparesis. A magnetic resonance imaging scan of the brain revealed a recent infarct from the dorsal side of the right putamen to the corona radiata. Three hours after onset, he complained of rotational vertigo. Truncal ataxia was also found on standing a few days later, which persisted until the chronic phase. Repeat magnetic resonance imaging scans on days 2 and 11 revealed no additional lesions. The present case indicates that rotational vertigo might result from a small, supratentorial, subcortical lesion., (Copyright © 2012 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Brain microbleeds (BMBs) can be detected on the gradient-echo T2*-weighted magnetic resonance imaging and are considered a risk factor for cognitive impairment and intracerebral hemorrhage. Detailed radiologic findings on the etiology of BMBs and their changes remain scarce. We present a case of subacute change in a BMB in the basal ganglia that mimicked a subacute lacunar infarct. Our findings underscore the need for physicians to be careful to not erroneously diagnose BMBs as lacunar infarctions and prescribe unnecessary antiplatelet medication., (Copyright © 2012 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Lenticulostriate vasculopathy (LSV) is a sonographic finding in infancy with obscure etiology and variable diagnostic and prognostic significance. Ischemic infarct in the territory of the lenticulostriate vessels after mild head trauma is a rare pathology. There are no publications on LSV followed by ischemic infarct. We present the case of an 8-month-old boy who suffered mild head trauma and developed an ischemic brain infarct in the territory of preexisting LSV. It is speculated that LSV might be a predisposing factor for ischemic brain infarct after mild head trauma in infants., (Copyright © 2012 Wiley Periodicals, Inc.)

Background: Small vessel diseases have been studied noninvasively with magnetic resonance imaging. Direct observation or visualization of the connected microvessel to the infarct, however, was not possible due to the limited resolution. Hence, one could not determine whether vessel occlusion or abnormal narrowing is the cause of an infarct., Methods: In this report, we demonstrate that the small vessel related to the infarct can be detected using ultra-high-field (7 T) magnetic resonance imaging and a three dimensional image analysis and modeling technique for microvessels, which thereby enables us to quantify the vessel morphology directly, that is, visualize the vessel that is related to the infarct. We compared vessels of selected stroke patients, who had recovered from stroke, with vessels from typical stroke patients, who had after effects like motor weakness, and age-matched healthy subjects to demonstrate the potential of the technique., Results: The experimental results show that typical stroke patients had overall degradation or loss of small vessels, compared with the selected patients as well as healthy subjects. The selected patients, however, had only minimal loss of vessels, except for one vessel located close to the infarct area., Conclusions: These preliminary results demonstrated that 7 T magnetic resonance imaging together with a three dimensional image analysis and modeling technique could provide information for detection of the vessel related to the infarct. In addition, three dimensional image analysis and modeling of vessels could further provide quantitative information on the microvessel structures comprising diameter, length and tortuosity., (© 2011 The Authors. International Journal of Stroke © 2011 World Stroke Organization.)

Object: Gamma Knife surgery (GKS) has emerged as the treatment of choice for small- to medium-sized cerebral arteriovenous malformations (AVMs) in deep locations. The present study aims to investigate the outcomes of GKS for AVMs in the basal ganglia and thalamus., Methods: Between 1989 and 2007, 85 patients with AVMs in the basal ganglia and 97 in the thalamus underwent GKS and were followed up for more than 2 years. The nidus volumes ranged from 0.1 to 29.4 cm(3) (mean 3.4 cm(3)). The mean margin dose at the initial GKS was 21.3 Gy (range 10-28 Gy). Thirty-six patients underwent repeat GKS for residual AVMs at a median 4 years after initial GKS. The mean margin dose at repeat GKS was 21.1 Gy (range 7.5-27 Gy)., Results: Following a single GKS, total obliteration of the nidus was confirmed on angiograms in 91 patients (50%). In 12 patients (6.6%) a subtotal obliteration was achieved. No flow voids were observed on MR imaging in 14 patients (7.7%). Following single or repeat GKS, total obliteration was angiographically confirmed in 106 patients (58.2%) and subtotal obliteration in 8 patients (4.4%). No flow voids on MR imaging were observed in 18 patients (9.9%). The overall obliteration rates following one or multiple GKSs based on MR imaging or angiography was 68%. A small nidus volume, high margin dose, low number of isocenters, and no history of embolization were significantly associated with an increased rate of obliteration. Twenty-one patients experienced 25 episodes of hemorrhage in 850 risk-years following GKS, yielding an annual hemorrhage rate of 2.9%. Four patients died in this series: 2 due to complications of hemorrhage and 2 due to unrelated diseases. Permanent neurological deficits caused by radiation were noted in 9 patients (4.9%)., Conclusions: Gamma Knife surgery offers a reasonable chance of obliterating basal ganglia and thalamic AVMs and does so with a low risk of complications. It is an optimal treatment option in patients for whom the anticipated risk of microsurgery is too high.

Background and Purpose: This study investigated whether incompleteness of the anterior part of the circle of Willis affects the occurrence of lacunes in the basal ganglia., Methods: One thousand and seventy-seven healthy individuals examined by magnetic resonance (MR) imaging and MR angiography were divided into eight subgroups according to our new classification., Results: Logistic regression analysis demonstrated that healthy individuals with incompleteness of the anterior circle of Willis had significantly higher frequency of lacunes [odds ratio (OR): 2.121, 95% confidence interval (CI): 1.477-3.108; or OR: 2.46, 95% CI: 1.377-4.384 in cases without or with fetal type posterior communicating artery, respectively] and higher numbers of lacunes (P < 0.001 or P < 0.001 in cases without or with fetal type posterior communicating artery, respectively) compared to patients with complete circle of Willis., Conclusions: Incompleteness of the anterior part of the circle of Willis significantly affected the occurrence of lacunes., (© 2011 The Author(s). European Journal of Neurology © 2011 EFNS.)

Background: Small deep brain infarcts are often caused by two different vascular pathologies: 1. atheromatous occlusion at the orifice of large caliber penetrating arteries termed branch atheromatous disease (BAD) and 2. lipohyalinotic degenerative changes termed lipohyalinotic degeneration (LD). We herein analyze and describe the characteristics of these 2 different pathologies., Methods: We studied 394 patients with penetrating artery territory infarcts in the territories of the lenticulostriate arteries and anterior pontine arteries. Radiologically defined BAD of the lenticulostriate arteries was defined as infarcts with size more than 10mm in diameter on axial slice and visible for 3 or more axial slices, and that of the anterior pontine arteries was defined as unilateral infarcts extending to the basal surface of the pons. Within each of the 2 territory groups, differences between BAD and LD were compared., Results: Ninety five patients in the lenticulostriate arteries group (36.1%) and 78 patients in anterior pontine arteries group (59.5%) were classified as BAD. Initial NIHSS, incidence of progressive motor deficits and poor functional outcome were significantly higher and incidence of concomitant silent lacunar infarcts tended to be lower in BAD than LD. In logistic regression analysis, BAD compared with LD was independently associated with PMD, in lenticulostriate arteries group (OR: 4.21, p=0.0001) and in anterior pontine arteries group (OR: 5.32, p=0.0018)., Conclusions: Radiologically defined BAD and LD had different characteristics. BAD was significantly associated with progressive motor deficits and considered as a major vascular mechanism of progressive motor deficits in penetrating artery infarcts., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Background and Purpose: Little is known about the risk factors of dilated Virchow-Robin spaces (dVRS) and their relation with other markers of brain small vessel disease. We investigated both issues in a large population-based sample of elderly individuals., Methods: Severity of dVRS was semiquantitatively graded in both white matter and basal ganglia using high-resolution 3-dimensional MRI images taken from 1818 stroke- and dementia-free subjects enrolled in the Three-City Dijon MRI study. Multinomial logistic regression models were used to model the association of cardiovascular risk factors, APOE genotype, brain atrophy, and MRI markers of small vessel disease with the degree of dVRS., Results: Severity of dVRS was found to be strongly associated with age in both basal ganglia (degree 4 versus 1: OR, 2.1; 95% CI, 1.4 to 3.2) and white matter (OR, 1.5; 95% CI, 1.2 to 1.9). The proportion of hypertensive subjects increased with the degrees of dVRS in both basal ganglia (P = 0.02) and white matter (P = 0.048). Men presented a higher risk of severe dVRS in basal ganglia than women, particularly degree 4 (OR, 6.0; 95% CI, 1.8 to 19.8). The degree of dVRS was associated with the volume of white matter hyperintensities and the prevalence of lacunes, but not with brain atrophy., Conclusions: In this large cohort study of elderly subjects, the degree of dVRS appears independently associated with age, hypertension, volume of white matter hyperintensities, and lacunar infarctions. dVRS should be considered as another MRI marker of cerebral small vessel disease in the elderly with regional variations in their severity.

Parkinsonism is a syndrome that features bradykinesia (slowness of the initiation of voluntary movement) and at least 1 of the following conditions: rest tremor, muscular rigidity, or postural instability. Criteria for the clinical diagnosis of vascular parkinsonism (VP) have been proposed, which are derived from a postmortem examination study. Computed tomography and magnetic resonance imaging can support this clinical diagnosis with positive imaging findings. Dopamine transporter single-photon emission computed tomography may also be of help to distinguish VP from Parkinson disease and other parkinsonisms.

Objective: To assess for lenticulostriate vasculopathy (LSV) on cranial ultrasound (cUS) scans of very preterm infants: incidence and aetiology, evolution during neonatal period, association with clinical parameters, and MRI equivalent., Design: Prospective study., Setting: Tertiary neonatal referral centre., Patients: Very preterm infants (<32 weeks) underwent sequential cUS throughout the neonatal period and MRI around term age. cUS were evaluated for LSV and other changes, and MRI for changes in signal and myelination in deep grey matter. LSV was divided into early-onset (7 postnatal days). Perinatal clinical parameters were collected for all infants and compared between groups., Results: In 22/111 (20%) infants LSV was detected: early-onset in 5 and late-onset in 17. LSV mostly presented some weeks after birth and persisted for several months. There were no associations between LSV and other changes on cUS or deep grey matter changes on MRI. Infants with late-onset LSV were younger and smaller at birth than infants with early-onset LSV. Postmenstrual age at first detection was comparable for both LSV groups. There were no associations between LSV and perinatal clinical parameters, but infants with LSV had less episodes of hypotension than infants without LSV., Conclusions: LSV is a frequent finding on cUS in very preterm infants, but does not show on MRI. The postmenstrual age, rather than gestational and postnatal age, seems important in LSV development. LSV is not associated with clinical parameters. When encountered in otherwise healthy preterm infants, LSV is probably a benign temporary phenomenon.

The introduction of diagnostic criteria for vascular dementia has helped to re-define the impact of various subcortical neuropathologies on aging; however, state-of-the-art neuroimaging techniques and autopsy studies suggest that not all structural brain alterations associated with vascular dementia are exclusive to this neurodegenerative process alone. Thus, a detailed analysis of the cognitive phenotype associated with ischaemic vascular disease is key to our understanding of subcortical neuropathology and its associated behaviors. Over the past twenty years, we have operationally defined this cognitive phenotype using the Boston Process Approach to neuropsychological assessment. This has led to both an empirical, as well as a theoretical understanding of three core constructs related to the dysexecutive syndrome associated with ischaemic vascular disease affecting periventricular and deep white matter as well as subcortical structures connecting these regions with the prefrontal cortex. Thus, difficulties with mental set, cognitive control and mental manipulation negatively impact executive functioning. This review will outline the subtle markers underlying this prefrontal dysfunction, i.e., the dysexecutive phenotype, associated with ischaemic vascular disease and relate it to fundamental impairments of gating subserved by basal ganglia-thalamic pathways within and across various dementia syndromes.

The clinical significance of dilated Virchow-Robin spaces (dVRS) remains unclear and their impact on cognitive performances has only been reported in small sample studies. Our aim was to assess the association between severity of dVRS and risk of incident dementia and cognitive decline in an elderly cohort. The degree of dVRS in both white matter and basal ganglia were ranked using high-resolution 3D MRI in a population-based sample of 1,778 non-demented participants from 65 to 80 years of age, who had a cerebral MRI at baseline. Cognitive function was assessed and dementia was diagnosed during a 4-year follow-up period. Cox proportional hazard models were used to examine the association between dVRS degree on a four-level severity score and incident dementia. The relationship between dVRS degree and change in cognition was examined using linear mixed effect models. During 6,135 person-years of follow-up, 27 individuals developed dementia. The highest degree of dVRS was associated with a strong increase in the risk of incident dementia independently of other standard risk factors of dementia, both for dVRS in white matter (HR=9.8, 95% CI 1.7-55.3) and in basal ganglia (HR =5.8, 95% CI 1.2-28.4). After further adjustment on white matter hyperintensity volume and brain infarcts, this association remained significant for dVRS in white matter. Higher rate of cognitive decline was found to be related to high degree of dVRS in basal ganglia but not in white matter. These results need confirmation but they suggest that assessment of the severity of dVRS may help identify groups of individuals that are at increased risk of dementia.

Background: Congenital infections are associated with a wide spectrum of clinical symptoms, including lenticulostriate vasculopathy (LSV)., Objective: To determine the relationship between LSV and congenital infections, as diagnosed by TORCH serology and viral culture for cytomegalovirus (CMV)., Methods: All neonates with LSV admitted to our neonatal intensive-care unit from 2004 to 2008 were included in the study. Results of maternal and neonatal TORCH testing were evaluated., Results: During the study period, cranial ultrasound scans were performed in 2,088 neonates. LSV was detected in 80 (4%) neonates. Maternal and/or neonatal serological TORCH tests were performed in 73% (58/80) of cases. None of the mothers or infants (0 of 58) had positive IgM titres for Toxoplasma, rubella, CMV or herpes simplex virus. Additional urine culture for CMV was performed in 38 neonates. None of the infants (0 of 38) had a positive CMV urine culture test., Conclusions: Routinely applied efforts to diagnose congenital infections in cases presenting with LSV have a poor yield. Routine TORCH screening in neonates with LSV cases should only be regarded as mandatory once well-designed studies demonstrate a clear diagnostic benefit., (Copyright 2009 S. Karger AG, Basel.)

Hypertension, a major risk factor for stroke, is associated with altered arterial anatomy and function; however, the limited resolution of current imaging techniques has restricted the in vivo study of microvascular changes in the brain. In this report, we quantitatively examined the lenticulostriate arteries in hypertensive patients using ultrahigh-field 7T MRI. We compared the number of stems and branches, curvature, and tortuosity of the lenticulostriate arteries by 3D time-of-flight magnetic resonance angiography among 20 hypertensive patients (mean age: 46.6+/-9.1 years) and 20 age-matched healthy subjects (mean age: 47.7+/-8.1 years). The average numbers of stems and branches in hypertensive patients were significantly less than those of healthy subjects (P<0.002). However, this difference was abolished in older volunteers (>45 years old), whereas the difference between young hypertensive patients (< or = 45 years old) and age-matched healthy controls was augmented by 55% for stems and 91% for branches (P=0.001). In comparison, there were no differences in the average curvature and tortuosity of the lenticulostriate arteries and no significant difference when corrected for smoking (P=0.064). In conclusion, our results showed that there was a substantial difference in the lenticulostriate arteries of hypertensive patients compared with healthy individuals when observed in vivo by ultrahigh-resolution 7T magnetic resonance angiography, and the difference was considerable in young subjects.

Objective: HIV enters the brain soon after infection causing neuronal damage and microglial/astrocyte dysfunction leading to neuropsychological impairment. We examined the impact of HIV on resting cerebral blood flow (rCBF) within the lenticular nuclei (LN) and visual cortex (VC)., Methods: This cross-sectional study used arterial spin labeling MRI (ASL-MRI) to measure rCBF within 33 HIV+ and 26 HIV- subjects. Nonparametric Wilcoxon rank sum test assessed rCBF differences due to HIV serostatus. Classification and regression tree (CART) analysis determined optimal rCBF cutoffs for differentiating HIV serostatus. The effects of neuropsychological impairment and infection duration on rCBF were evaluated., Results: rCBF within the LN and VC were significantly reduced for HIV+ compared to HIV- subjects. A 2-tiered CART approach using either LN rCBF < or =50.09 mL/100 mL/min or LN rCBF >50.09 mL/100 mL/min but VC rCBF < or =37.05 mL/100 mL/min yielded an 88% (29/33) sensitivity and an 88% (23/26) specificity for differentiating by HIV serostatus. HIV+ subjects, including neuropsychologically unimpaired, had reduced rCBF within the LN (p = 0.02) and VC (p = 0.001) compared to HIV- controls. A temporal progression of brain involvement occurred with LN rCBF significantly reduced for both acute/early (<1 year of seroconversion) and chronic HIV-infected subjects, whereas rCBF in the VC was diminished for only chronic HIV-infected subjects., Conclusion: Resting cerebral blood flow (rCBF) using arterial spin labeling MRI has the potential to be a noninvasive neuroimaging biomarker for assessing HIV in the brain. rCBF reductions that occur soon after seroconversion possibly reflect neuronal or vascular injury among HIV+ individuals not yet expressing neuropsychological impairment.

Purpose: To evaluate the capability of flow-sensitive black blood (FSBB) acquisition to visualize the lenticulostriate artery (LSA) in comparison with time-of-flight (TOF) angiography., Materials and Methods: Twenty-one healthy subjects (13 males and 8 females, 19-44 years old) were enrolled in this study after obtaining written informed consent. Magnetic resonance imaging (MRI) examinations were performed with FSBB and TOF to visualize the LSA using a 1.5T MRI unit. In FSBB acquisition a motion probing gradient of b = 4 sec/mm(2) was applied to dephase blood flow. Images were reconstructed into coronal sections and were evaluated in terms of number, length, and image quality at origins and distal areas of visualized LSA branches with a four-point scale., Results: In all, 145 LSA branches were visualized with FSBB and 66 branches with TOF. There was no LSA visualized only with TOF. In all evaluated terms, FSBB was significantly better than TOF., Conclusion: We could better visualize the LSA with FSBB than with TOF, both quantitatively and qualitatively. FSBB is a promising method, although it remains to be evaluated in clinical cases.

In hyperarginenemia, there is a defect in argininase enzyme, which is a catalyzer of urea cycle. Though the pathogenesis of neuronal damage in hyperargininemia is not clear, high serum and cerebrospinal fluid arginine levels can be directly related with neuronal damage. In this study, our aim was to assess brain magnetic resonance images and magnetic resonance spectroscopy (MRS) patterns of two siblings with hyperarginenemia. We acquired single voxel MRS from the white matter to show the myelination pattern and to figure out any abnormal peak of metabolite stored due to enzymatic defect. We observed mild cerebral and cerebellar atrophy and infarct at bilateral posterior putamen and insular cortex localization on conventional images and elevated choline/creatine ratios and abnormal peak at 3.8 ppm, most likely representing arginine deposition. To the best of our knowledge, this is the first article revealing the brain MRS pattern of hyperargininemia. We reported the clinical and imaging findings of patients and discuss the correlation.

Object: Lenticulostriate artery (LSA) aneurysms are rarely reported in the literature, making management decisions challenging. Conservative, endovascular, and surgical treatments have been described primarily through case reports and reports of individual authors' experiences. The purpose of this study is to report neurological outcomes in a single-institution experience of ruptured lenticulostriate aneurysms treated surgically., Methods: The authors have conducted a retrospective review of all cases involving patients with ruptured LSA aneurysms who presented to the Mt. Sinai Hospital neurosurgical service between September 2001 and January 2007., Results: Over 5.4 years, the authors treated 6 patients with 7 LSA aneurysms-6 ruptured and 1 unruptured. The Hunt and Hess grade on admission ranged from I to IV, with subarachnoid hemorrhage in 5 of the 6 patients. Catheter angiography confirmed the presence of the aneurysms, and all patients underwent a pterional craniotomy and clipping or resection of the aneurysm, performed by a single surgeon. Associated risk factors in our series of patients included hypertension, cocaine abuse, and intracranial occlusive disease suggestive of moyamoya disease. Two types of LSA aneurysms were identified. The mean size of the 6 ruptured aneurysms was 3.2 mm. The LSA was preserved in 3 of 6 patients, but LSA preservation did not correlate with development of a postoperative infarct, clinically or radiologically. In patients with ruptured aneurysms, the mean modified Rankin Scale score at discharge was 1.7. The 3 patients in whom the LSA was sacrificed had good outcomes, suggesting that loss of the artery is clinically well tolerated., Conclusions: This case series demonstrates that surgical treatment of ruptured LSA aneurysms can be an appropriate, effective, and safe therapy.

Emotional facial palsy (EFP) is a rare condition in which facial paresis is only apparent during reflex movements of the hemiface, such as smiling and laughter. We report the case of a 32-year-old man presenting with EFP as the main symptom of a small striatocapsular infarction. Our case strongly suggests that the anterior arm of the internal capsule is part of the corticonuclear tract that is involved in emotional facial motility.

Brain abscess is a rare but very dangerous neurosurgical lesion. Prompt diagnosis and emergency surgical evacuation are the hallmarks of therapy. Brain abscess following ischemic and hemorrhagic stroke is a rare entity. These cases are often preceded by episodes of bacteremia, sepsis, and local infection. The authors report the case of a 30-year-old woman who presented with a cerebral abscess at the site of a recent intraparenchymal hemorrhage.

Aneurysms of the distal lenticulostriate artery (LSA) are rare. Only 16 cases have been reported in the literature. Early detection and treatment of these aneurysms is also difficult because of their deep location, small size and angioarchitecture. We report two additional patients with aneurysms, arising from the distal LSA, who presented with deep intracerebral haemorrhage. The conclusions drawn from our experience and a comprehensive review of the literature include the following. (1) A distal LSA aneurysm should be considered in young (mean 38.5 years) and non-hypertensive (80%) patients with deep intracerebral haemorrhage. (2) These aneurysms are frequently very small (<5 mm). Therefore, they cannot be detected on initial angiograms in some cases. (3) These aneurysms have higher rates of associated vascular lesions. Deep intracerebral haemorrhage, even in those over the age of 50 years, can still be due to underlying, treatable structural abnormalities, and should not be dismissed as being a result of hypertension. In addition, a more comprehensive diagnostic approach seems to be warranted in younger patients and those without known hypertension.

The authors describe a rare case of idiopathic distal lenticulostriate artery (LSA) aneurysm in a 5-year-old boy who presented in the emergency department with a sudden onset of headache. Admission computed tomography scans revealed an intracerebral hemorrhage in the left caudate nucleus with intraventricular extension. Angiographic studies demonstrated a left medial LSA aneurysm. The patient underwent a left parasagittal frontal craniotomy, the lateral ventricle was accessed via the anterior transcallosal approach, and the aneurysm was removed after sectioning of the parent vessel. The child left the hospital after 5 days; at that time he was asymptomatic and without motor impairment. The optimum treatment of aneurysms involving small perforating arteries is controversial and depends mainly on the causative factors. The pathogenesis and treatment of these unusual aneurysms are discussed.

Objective: Lenticulostriate artery aneurysms are rare. When present, distal locations in and around the basal ganglia are more common and often present with intraparenchymal hemorrhage when ruptured. We present a very rare case of a ruptured proximal lenticulostriate fusiform aneurysm presenting with subarachnoid hemorrhage., Clinical Presentation: We report the case of a 31-year-old healthy man who presented after the sudden onset of headache, nausea, and lethargy without neurological deficits. Cranial computed tomographic scanning demonstrated diffuse subarachnoid hemorrhage, and a cranial computed tomographic angiogram demonstrated a vascular irregularity on the superior surface of the left distal M1 trunk of the middle cerebral artery. A cerebral angiogram demonstrated a left proximal lenticulostriate fusiform aneurysm without evidence of moyamoya-like vessels or vasculitis. No other pathology or infectious etiology was noted., Intervention: Endovascular therapy was deemed unsafe, and microsurgical exploration and intervention was the more favorable and safe approach. A standard left pterional craniotomy was performed and the afferent lenticulostriate vessel into the fusiform aneurysm was visualized. Temporary clips were applied to the proximal and distal M1 trunk and miniclips were applied across the afferent portion and fundus of the aneurysm, thus sacrificing the parent lenticulostriate artery. A postoperative computed tomographic scan demonstrated an area of hypodensity in the left basal ganglia. The patient's postoperative right facial and upper extremity weakness improved to normal several days after aneurysmal clipping., Conclusion: This is the first report of a ruptured proximal lenticulostriate artery fusiform aneurysm, which presented as subarachnoid hemorrhage in a healthy patient without an underlying vascular disease.

Background: Late-life depressive disorders have been linked to cerebrovascular disease (the vascular depression hypothesis). Treatment resistance may be associated with vascular-based lesions in the white matter and basal ganglia. Virchow-Robin spaces (VRS) are cerebrospinal fluid spaces associated with microangiopathy of small cerebral vessels. This study tested the hypothesis that dilation of Virchow-Robin spaces seen on Magnetic Resonance Imaging (MRI) is associated with treatment resistance in elderly depressed individuals., Methods: 50 patients with late-onset (age over 60 years) major depressive disorder (29 responders to monotherapy, 21 non-responders to monotherapy) and 35 normal volunteers were recruited. Assessment of deep white matter lesions [WML] and periventricular hyperintensities [PVH] (both with the Scheltens rating scale score, [Scheltens, P., Barkhof, F., Leys, D., et al. (1993) A semiquantative rating scale for the assessment of signal hyperintensities on magnetic resonance imaging. J Neurol Sci;114(1):7-12.]) and the severity of VRS dilatation (using a new scale) were scored from MRI images. Statistical group comparisons and multiple regression analyses were performed to quantify the relationship between imaging features and clinical outcome., Results: There was a trend for greater WML Scheltens scores in the monotherapy resistant group compared to responders and control subjects, but only using the basal ganglia VRS score was there a statistically significant difference. A score of 2 or greater on the VRS score was 80% sensitive and 62% specific in predicting non-response to antidepressant monotherapy. The VRS score accounted for 38% of the variance in the multiple regression model and the PVH score, which was an independent predictor of outcome, contributed another 6%., Limitations: Numbers are small and type II errors possible, especially for the Scheltens ratings. Treatment response was limited to response or non-response to monotherapy and was retrospectively derived. The VRS scale was originally designed for use in patients with vascular dementia and has not been used before in affective disorders. Although all depressed subjects were late-onset, it is possible that depression led to vascular disease rather than vice versa., Conclusion: VRS dilatation is common in diseases associated with microvascular abnormality, which is the presumed basis of vascular depression in the elderly. VRS score may be useful in determining which patients are less likely to respond to antidepressant monotherapy. Prospective studies of patients with a wider range of treatment responses are indicated.

Background: Gait variability is an index of how much gait parameters, such as step length, change from one step to the next. Gait variability increases with age and in individuals affected by cortical and subcortical neurodegenerative conditions, and it is associated with falls and incident mobility disability. The brain anatomical correlates of gait variability have not been studied in high-functioning community-dwelling older adults., Methods: Gait variability and brain MRIs were assessed in a cohort of 331 men and women (mean age = 78.3 years) free from stroke, dementia or Parkinson's disease. Gait variability was computed for spatial parameters (step length and step width) and for temporal parameters (stance time). Subclinical brain vascular abnormalities were measured on brain MRIs as infarcts and white matter hyperintensities., Results: Greater variability of step length was associated with greater prevalence of infarcts, including infarcts in the basal ganglia, and with greater white matter hyperintensities severity, independent of age, gender, cognitive function and cardiovascular disease. Weaker associations were found between the other variability measures and the MRI measures., Conclusion: In this group of older adults free from neurodegenerative diseases, a greater variability of step length was associated with greater burden of subclinical brain vascular abnormalities as defined by MRI., ((c) 2007 S. Karger AG, Basel.)

Purpose: We sought to determine whether an early CT ischemic lesion showing parenchymal hypoattenuation might be undetectable on diffusion-weighted imaging (DWI) in acute cerebral ischemia., Materials and Methods: We retrospectively evaluated CT and MR images of 70 consecutive patients with acute middle cerebral artery (MCA) infarction. All patients underwent CT and MR imaging within 6 hours of symptom onset. We determined the presence of reversed discrepancy (RD), defined as an early ischemic lesion showing parenchymal hypoattenuation on CT but no hyperintensity on DWI. CT Hounsfield units (HU), apparent diffusion coefficients (ADCs), and perfusion parameters were calculated for RD lesions., Results: RD was found in 9 (12.9%) patients and at basal ganglia (89%). The mean HU of RD lesion was lower than that of normal tissue (DeltaHU, 2.33 +/- 0.74, P < .001). RD lesions showed no significant decrease of ADC (ADC ratio, 0.97 +/- 0.07, P = .059) and cerebral blood flow (relative CBF, 0.87 +/- 0.20, P > 0.05). Delayed DWI hyperintensity occurred in 8 (88.8%) RD lesions, and all lesions progressed to infarction. In 6 (66%) of 9 patients with RD, Alberto Stroke Program Early CT scores of ischemic lesions were lower on CT than those on DWI., Conclusion: RD was uncommonly found mainly in basal ganglia, and all RD lesions progressed to infarction at follow-up. Early CT ischemic lesion showing parenchymal hypoattenuation may be undetectable on DWI, and DWI may underestimate extent of severe ischemic tissue in patients with acute MCA infarction.

A case of pure mutism without pseudobulbar palsy and other neurological findings resulting from simultaneous bilateral lenticulostriate artery territory infarction is presented. A 45-year-old woman suffered a transient ischemic attack with nonfluent aphasia and right hemiparesis. Six months later, she developed pure mutism without oral apraxia, pseudobulbar signs, and motor deficits. Magnetic resonance imaging revealed bilateral infarction in the lentiform nucleus regions. In the available data, there is only one report of simultaneous bilateral lenticulostriate infarction. To date, in all reported cases of mutism of subcortical vascular origin there are also various degrees of pseudobulbar signs and motor deficits and the responsible lesions are mostly consecutive. The case presented here is the first to show pure vascular mutism without other neurological findings.

Abscesses of the spleen are rare and, if untreated, lead to death. The usual mode of development is by hematogenous spread of infection. Treatment options are splenectomy, percutaneous drainage and in selected cases an antibiotic therapy. We report a case of a splenic abscess due to Streptococcus bovis treated with antibiotics only.

We report on a patient who experienced visual hallucinations and pseudohallucinations comprising palinopsia, peduncular hallucinosis, and oblique vision. The most probable etiologies of these phenomena are discussed with respect to clinical presentation and findings on computed tomography and magnetic resonance imaging., (Copyright (c) 2005 S. Karger AG, Basel.)