Utility of Magnetic Resonance Spectroscopy for the Progression of Neurological Symptoms in Lenticulostriate Artery Territory Infarction.

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.

Ruptured lenticulostriate artery aneurysm: a report of a case treated with endovascular embolisation.

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.

An extremely severe case of Aicardi-Goutières syndrome 7 with a novel variant in IFIH1.

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.

Intraventricular haemorrhage as the first manifestation of congenital Cytomegalovirus infection.

Congenital Cytomegalovirus infection (CCMV) is the most common intrauterine infection. Early diagnosis of CCMV is hindered by three factors: There is no screening programme for CMV infection in pregnant women; a high percentage of infections in neonates are asymptomatic; the clinical signs of CCMV infection are uncharacteristic. The aim of this article is to analyse the clinical picture and course of CCMV treatment in a 3-week-old newborn, analyse adverse events in 14-week-long antiviral therapy and also assess intraventricular bleeding as an early indicator for the diagnosis of CCMV.

Características de la enfermedad cerebrovascular extracraneal en el municipio de arroyo naranjo / Characteristics of the extra-cranial cerebrovascular disease in Arroyo Naranjo municipality

Introducción: La enfermedad cerebrovascular extracraneal en Cuba está subdiagnosticada, de ahí que se desconoce su prevalencia en población general.Objetivo: Determinar las características de la enfermedad cerebrovascular extracraneal en el municipio de Arroyo Naranjo.Métodos: Se realizó estudio descriptivo en las 26 personas diagnosticadas con enfermedad carotidea de una muestra de 200 individuos obtenida por método aleatorio simple de los 208 554 residentes del municipio Arroyo Naranjo. La enfermedad se confirmó por eco-doppler carotideo. Las variables fueron: edad, sexo, porcentaje de estenosis, localización, tipo de placa, factores de riesgo y grosor íntima-media carotideo.Resultados: La prevalencia de la enfermedad fue de 12,5 x 100 000 habitantes, mayor en las mujeres (10,1) y en los mayores de 60 años (9,1). Se confirmó el diagnóstico en el 80,8 por ciento de las personas y más del 45 por ciento mostró estenosis no significativa en ambas carótidas. Predominaron las placas heterogénea (46,2 por ciento) localizadas fundamentalmente en: bifurcación carotidea y bulbo. El tabaquismo (80,8 por ciento), la hipertensión arterial (73,1 por ciento), y la enfermedad arterial periférica (65,1 por ciento) fueron los factores de riesgo más frecuentes; el 69,2 por ciento presentaba más de tres factores. Más del 75 por ciento de los mayores de 60 años, independiente del sexo, tenían valores patológicos del grosor íntima-media carotideo.Conclusiones: La prevalencia de enfermedad cerebrovascular extracraneal en el municipio es elevada. Alta incidencia de factores de riesgo. El diagnóstico precoz de dicha enfermedad en un alto porcentaje permitió remitir a sus áreas de salud a aquellas personas que requerían de un tratamiento oportuno(AU)

Introduction: Extracranial cerebrovascular disease is a problem of health internationally, in Cuba it is sub-diagnostician without knowing its prevalence in the general population.Objective: To determine the characteristics of the extracranial cerebrovascular disease in the Arroyo Naranjo municipality.Methods: a descriptive study was conducted in 26 people diagnosed with carotid disease of a sample of 200 people obtained by a simple random method of the 208 554 residents of the municipality of Arroyo Naranjo. The disease was confirmed by carotid doppler ultrasound. The variables were: age, sex, percent stenosis, location, type of plate, risk factors and thickness intima-media carotid.Results: The prevalence of the disease was 12,5 x 100 000 inhabitants, higher in women (10,1) and older adults (9,1). The diagnosis was confirmed in 80,8 percent of people, where more than 45 percent showed a non-significant stenosis in both carotid. The types of plates were the heterogeneous (46,2 percent) and the echogenic (38,6 percent), located in: carotid bifurcation, bulb, and internal carotid. Smoking (80,8 percent), hypertension (73,1 percent), and peripheral arterial disease (65,1 percent) were the most frequent risk factors, noting that 69,2 percent had more than three factors. More of the 75 percent of those older than 60 years, independent of sex, showed pathological values of the thickness intima-media carotid.Conclusions: The prevalence of the extracranial cerebrovascular disease in the municipality is high; and too the incidence of the risk factors. Early diagnosis of the disease in a high percentage allowed referring to their areas of health to those persons requiring prompt treatment(AU)

Características de la enfermedad cerebrovascular extracraneal en el municipio de arroyo naranjo / Characteristics of the extra-cranial cerebrovascular disease in Arroyo Naranjo municipality

Introducción: La enfermedad cerebrovascular extracraneal en Cuba está subdiagnosticada, de ahí que se desconoce su prevalencia en población general. Objetivo: Determinar las características de la enfermedad cerebrovascular extracraneal en el municipio de Arroyo Naranjo. Métodos: Se realizó estudio descriptivo en las 26 personas diagnosticadas con enfermedad carotidea de una muestra de 200 individuos obtenida por método aleatorio simple de los 208 554 residentes del municipio Arroyo Naranjo. La enfermedad se confirmó por eco-doppler carotideo. Las variables fueron: edad, sexo, porcentaje de estenosis, localización, tipo de placa, factores de riesgo y grosor íntima-media carotideo. Resultados: La prevalencia de la enfermedad fue de 12,5 x 100 000 habitantes, mayor en las mujeres (10,1) y en los mayores de 60 años (9,1). Se confirmó el diagnóstico en el 80,8 por ciento de las personas y más del 45 por ciento mostró estenosis no significativa en ambas carótidas. Predominaron las placas heterogénea (46,2 por ciento) localizadas fundamentalmente en: bifurcación carotidea y bulbo. El tabaquismo (80,8 por ciento), la hipertensión arterial (73,1 por ciento), y la enfermedad arterial periférica (65,1 por ciento) fueron los factores de riesgo más frecuentes; el 69,2 por ciento presentaba más de tres factores. Más del 75 por ciento de los mayores de 60 años, independiente del sexo, tenían valores patológicos del grosor íntima-media carotideo. Conclusiones: La prevalencia de enfermedad cerebrovascular extracraneal en el municipio es elevada. Alta incidencia de factores de riesgo. El diagnóstico precoz de dicha enfermedad en un alto porcentaje permitió remitir a sus áreas de salud a aquellas personas que requerían de un tratamiento oportuno(AU)

Introduction: Extracranial cerebrovascular disease is a problem of health internationally, in Cuba it is sub-diagnostician without knowing its prevalence in the general population. Objective: To determine the characteristics of the extracranial cerebrovascular disease in the Arroyo Naranjo municipality. Methods: a descriptive study was conducted in 26 people diagnosed with carotid disease of a sample of 200 people obtained by a simple random method of the 208 554 residents of the municipality of Arroyo Naranjo. The disease was confirmed by carotid doppler ultrasound. The variables were: age, sex, percent stenosis, location, type of plate, risk factors and thickness intima-media carotid. Results: The prevalence of the disease was 12,5 x 100 000 inhabitants, higher in women (10,1) and older adults (9,1). The diagnosis was confirmed in 80,8 percent of people, where more than 45 percent showed a non-significant stenosis in both carotid. The types of plates were the heterogeneous (46,2 percent) and the echogenic (38,6 percent), located in: carotid bifurcation, bulb, and internal carotid. Smoking (80,8 percent), hypertension (73,1 percent), and peripheral arterial disease (65,1 percent) were the most frequent risk factors, noting that 69,2 percent had more than three factors. More of the 75 percent of those older than 60 years, independent of sex, showed pathological values of the thickness intima-media carotid. Conclusions: The prevalence of the extracranial cerebrovascular disease in the municipality is high; and too the incidence of the risk factors. Early diagnosis of the disease in a high percentage allowed referring to their areas of health to those persons requiring prompt treatment(AU)

Lenticulostriate Vasculopathy and Neurodevelopmental Outcomes in Preterm Infants: A Systematic Review.

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.

Correlation Between the Reduction in Lenticulostriate Arteries Caused by Hypertension and Changes in Brain Metabolism Detected With MRI.

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.

Lenticulostriated vasculopathy is a high-risk marker for hearing loss in congenital cytomegalovirus infections.

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.

Spontaneous Dissecting Lenticulostriate Artery Aneurysm in Children: Radiologic Findings and Clinical Management.

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.

A case of cavernous sinus thrombosis with meningitis caused by community acquired methicillin resistant Staphylococcus aureus.

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.

[Why perform routine brain sonography in late preterm infants?].

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.

Carotid stent infection: a rare but potentially fatal complication of carotid artery stenting.

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.

High-resolution MRI findings in patients with capsular warning syndrome.

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.

Does lenticulostriate vasculopathy predipose to ischemic brain infarct? A case report.

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.

Rotational vertigo associated with putaminal infarction.

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.

Gamma Knife surgery for basal ganglia and thalamic arteriovenous malformations.

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.

Bilateral caudate nucleus infarction associated with a missing A1 segment.

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.

Three dimensional model-based analysis of the lenticulostriate arteries and identification of the vessels correlated to the infarct area: preliminary results.

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.