Mineralizing Lenticulostriate Vasculopathy: An Emerging Risk Factor for Basal Ganglia Stroke After Minor Head Trauma in Young Children.

Mineralizing lenticulostriate vasculopathy is a well-recognized risk factor for basal ganglia stroke after minor head trauma in infants and young children; it is diagnosed on head computed tomography by the presence of basal ganglia calcification, seen as punctate hyperdensities on axial and linear hyperdensities on reconstructed coronal and sagittal images. In children with anterior fontanel window, its presence is suggested by branching hyperechogenic stripes in the basal ganglia region on cranial ultrasound. Brain magnetic resonance imaging, including susceptibility-weighted sequences and brain magnetic resonance angiography, fail to detect calcification or vascular abnormalities. Although its etiology remains unknown, mineralizing lenticulostriate vasculopathy is considered to represent end-stage pathology of lenticulostriate vasculopathy, a neonatal radiographic condition detected during routine neonatal cranial ultrasonographic examination and represents nonspecific finding associated with a multitude of etiologies. The significance of mineralizing lenticulostriate vasculopathy lies in the fact that it has emerged as one of the most common risk factors for basal ganglia stroke in Indian children, accounting for one-fourth to one-half of all causes of stroke in some studies. The outcome of stroke in children with mineralizing lenticulostriate vasculopathy appears to be favorable with the majority achieving complete or nearly complete recovery of their motor functions. Stroke recurrence following repeat head trauma is seen in a small proportion of children despite aspirin treatment.

Placental pathology associated with lenticulostriate vasculopathy (LSV) in preterm infants.

OBJECTIVE: Our aim was to examine the frequency and type of placental abnormalities in neonates with LSV. STUDY DESIGN: We prospectively reviewed cranial ultrasounds (cUS) from neonates born at ≤32 weeks of gestation at Parkland Hospital between 2012 and 2014. Our cohort included neonates with LSV and gestational age and sex matched controls with normal cUS. We retrieved placental pathology reports retrospectively and compared placental abnormalities in both groups. RESULTS: We reviewed 1351 cUS from a total of 407 neonates. Placental pathology evaluations were complete for 64/65 (98%) neonates with LSV and 68/70 (97%) matched controls. There were no significant differences for any type of placental abnormities between LSV and control groups. However, infants with highest stage LSV were more likely to have large for gestational age (LGA) placentas (p = 0.01). CONCLUSION: The association between LSV and LGA placenta may indicate a shared vascular response to an adverse prenatal environment.

A rare cause of stroke in young children: minor head trauma associated with mineralising lenticulostriate angiopathy in three patients.

Acute basal ganglia infarct following minor head trauma in association with mineralisation of lenticulostriate arteries is an increasingly recognised entity in childhood stroke. Three cases with a classical history and phenotypical features of mineralising angiopathy are described. Case 1 was a 2-year-old girl who presented with acute onset hemiparesis with a same-side upper motor neuron (UMN)-type facial palsy following minor head trauma. Case 2 was a 14-month-old boy who presented with a left side hemiparesis and a left UMN-type facial nerve palsy following a minor fall. Case 3 was an 8-month-old boy who, following a fall from his cot, had a sudden-onset hemiparesis on the right side and deviation of the angle of the mouth to the left. In brain computed tomography, all three cases demonstrated characteristic basal ganglia calcification of the mineralising angiopathy. Magnetic resonance imaging of the brain demonstrated features supportive of acute infarcts in the lentiform nucleus, caudate nucleus and putamen. Two of the patients had iron deficiency anaemia with haemoglobin of 7.0 g/dL and 7.8 g/dL, respectively. On follow-up, Case 1 had mild residual weakness and the other two made a complete recovery. None of the patients had a recurrence of stroke. Basal ganglia stroke with mineralising angiopathy should be considered in toddlers presenting with sudden-onset focal neurological deficits preceded by minor head trauma.Abbreviations: ADC: apparent diffusion coefficient; CT: computed tomography; DWI: diffusion-weighted imaging; Hb: haemoglobin; IDA: iron deficiency anaemia; MRI: magnetic resonance imaging; SLV: sonographic lenticulostriate vasculopathy; SWI: susceptibility weighted imaging; UMN: upper motor neuron.

Lenticulostriate vasculopathy in routine brain ultrasonography in infants: next step?

Lenticulostriate vasculopathy (LSV) is a relatively common fi nding in routine cranial ultrasound examination that has been associated with many infectious and non-infectious conditions. The aim of this review was to provide a better understanding of LSV ultrasound fi nding, as well as the need for further laboratory and imaging examinations in infants. The most of the published studies represented small series, with few prospective long-term studies involving the control groups. Authors have mostly found an association between LSV, especially higher-grade (although there is no universally accepted classifi cation) with congenital cytomegalovirus (CMV) infection, classifying those children as at risk for sensorineural hearing loss. In contrast, some authors pointed out that LSV could be found relatively often, and believe that isolated LSV, especially lower-grade, is not predictive for an unfavourable outcome and a long-term prognosis. Therefore, although 35 years have passed since the first publication of LSV, there is still no consensus among experts on the clinical signifi cance of isolated LSV, but caution is certainly needed given the fact that most infants with congenital CMV are asymptomatic.

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.

Childhood ischaemic stroke in the basal ganglia can lead to fine motor and anxiety disorders: a retrospective analysis and follow-up of 109 cases.

BACKGROUND: Stroke in children easily causes long-term dysfunction. Whether the prognoses of motor and anxiety disorders are related to the affected stroke area has not been reported. METHODS: One hundred nine cases of children with ischaemic stroke were reviewed and divided into three groups: lenticular nucleus lesions only (lenticular nucleus group), lenticular nucleus and caudate head lesions (caudate head group), and lenticular nucleus and thalamus lesions (thalamus group). Overall prognosis was evaluated by the mRS score. The SCAS-P was used to evaluate anxiety in children aged ≥6 years. RESULTS: mRS scores were ≤ 2 points (mean: 0.62), no significant difference among groups. 3/21 (14.2%) patients in the caudate head group changed handedness, which is significantly higher than other groups. Patients with lesions in thalamus group had significantly higher SCAS-P scores. CONCLUSIONS: The overall prognosis of children with basal ganglia ischaemic stroke is good. However, hand preference changes and anxiety disorders may develop. Patients in the caudate head groups are more likely to suffer from fine motor disorders and changes in handedness. Patients within the thalamus group are more prone to anxiety than patients in the other groups. Anxiety disorders should be noted in children with basal ganglia stroke.

[Familial Mediterranean Fever-Associated Lenticulostriate Artery Aneurysm Presenting with Intracranial Hemorrhage and Disappearing Spontaneously: A Case Report].

Lenticulostriate artery (LSA) aneurysm is relatively rare, and the need for surgical intervention is controversial. Here, we report a case of ruptured LSA aneurysm which was accompanied by familial Mediterranean fever (FMF). A 45-year-old woman with a history of FMF presented with sudden onset of headache and vertigo. Computed tomography revealed hemorrhage in the right caudate nucleus and lateral ventricles. Digital subtraction angiography revealed a fusiform aneurysm (3mm) at the distal site of medial LSA. Observation was selected, and the aneurysm gradually decreased and eventually disappeared on the 105th day from the onset. This was the first report describing an LSA aneurysm associated with FMF. It suggests that such aneurysms could be treated conservatively with close radiological follow-up.

Aneurysms of the Lenticulostriate Artery: A Systematic Review.

BACKGROUND: Lenticulostriate artery aneurysms (LSAs) are rare vascular aberrations. Despite the potentially catastrophic sequelae of aneurysmal rupture, the optimal management strategy for LSA aneurysms has not been determined. The aim of the present review is to provide an overview of the clinical presentation and treatment strategies for LSA aneurysms. METHODS: Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a literature search was conducted in the PubMed, Cochrane, EBSCOhost, Scopus, Web of Science, and ProQuest search engines to identify reported studies of LSA aneurysms until July 1, 2020. A descriptive analysis was performed. RESULTS: A total of 71 studies with 112 cases of LSA aneurysms were included. Patient age ranged from 2 months to 83 years (median, 44.5 years). Male and female patients were affected similarly (49% and 51%, respectively). The most common presentation was aneurysmal rupture (78%), and headache was the most frequently reported symptom (36%). Overall, 48% of the patients had undergone underwent surgical treatment, 30% conservative management, 21% endovascular treatment, and 1% radiosurgery. Four patients died, all of whom had presented with aneurysmal rupture. CONCLUSION: We have summarized the reported cases of LSA aneurysms, with their clinical presentation, management, and outcomes, for physicians who may be confronted with this diagnosis. Future studies that use available classification systems and include as much detail as possible should be encouraged to fully elucidate the optimal management strategy for these patients.

Successful Mechanical Thrombectomy for Subocclusive Thrombus at the Origin of Lenticulostriate Artery.

Optimal treatment of patients with intracranial subocclusive thrombus remains unclear. Such a rare case successfully managed with endovascular mechanical thrombectomy is presented. A 71-year-old man experienced a sudden onset of dysarthria and motor deficits. At the time of admission his National Institutes of Health Stroke Scale (NIHSS) score was 4. DWI demonstrated incomplete infarction within the left lenticulostriate artery (LSA) territory, MRA showed partial flow defect in the distal left M1 segment and non-visualization of the LSA, and ECG revealed atrial fibrillation, thus ischemic stroke caused by cardiogenic embolism was diagnosed. Tissue plasminogen activator was administered, but symptoms progressed and NIHSS score increased up to 8. Diagnostic angiogrpahy confirmed presence of the subocclusive thrombus within the distal left M1 segment and complete occlusion of LSA at its origin. Since conservative therapy was ineffective, mechanical thrombectomy utilizing ADAPT (a direct aspiration first-pass thrombectomy) technique was performed resulting in compete recanalization of the LSA accompanied by the prompt regress of neurological symptoms. Eventally, the patient demonstrated nearly full recovery (modified Rankin Scale score 1). Thus, mechanical thrombectomy should be considered as a reasonable option in cases of acute cerebral stroke caused by subocclusive thrombus and progressive neurological deficits despite standard conservative therapy. J. Med. Invest. 67 : 372-374, August, 2020.

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.

Different subtypes of collateral vessels in hemorrhagic moyamoya disease with p.R4810K variant.

BACKGROUND: The aim of this study was to investigate the hemorrhgic sites and collateral vessels in hemorrhagic MMD with the p.R4810K variant. METHODS: Hemorrhage sites were classified as either anterior or posterior. Collateral vessels were classified into three subtypes according to origin: lenticulostriate anastomosis, thalamic anastomosis, and choroidal anastomosis. Hemorrhage sites and collateral vessels were compared between patients with wild-type p.R4810K variant (GG) and patients with heterozygous p.R4810K variant (GA) after 1:1 propensity score matching. RESULTS: A total of 130 hemorrhagic MMD patients were included in present study, 21 pairs (42 hemorrhagic hemispheres) were obtained after 1:1 propensity score. In GA group, 16 hemispheres (76.2%) presented anterior hemorrhage, and 5 hemispheres (23.8%) presented with posterior hemorrhage. In GG group, 13 hemispheres (61.9%) presented anterior hemorrhage, and 8 hemispheres (38.1%) presented with posterior hemorrhage. No significant differences were found in hemorrhagic sites between two matched groups (P > 0.05). Of 21 hemispheres in GA group, 10 (47.6%) exhibited lenticulostriate anastomosis, 6 (28.6%) thalamic anastomosis, and 6 (28.6%) choroidal anastomosis. Of 21 hemispheres in GG group, 3 (14.3%) exhibited lenticulostriate anastomosis, 5 (23.8%) thalamic anastomosis, and 9 (42.9%) choroidal anastomosis. There was significant difference in lenticulostriate anastomosis between two matched groups (P = 0.045). After adjustment the age, sex, and PCA involvement, we found that lenticulostriate anastomosis was associated with p.R4810K variant (OR, 5.995; 95% CI, 1.296-27.737; P = 0.022). CONCLUSION: Lenticulostriate anastomosis might be associated with p.R4810K variant. Whereas hemorrhagic sites, thalamic anastomosis, and choroidal anastomosis might not be associted withp.R4810K variant.

Ambulatory Blood Pressure Levels in the Prediction of Progression of Cerebral Small Vessel Disease.

OBJECTIVES: We aimed to study the value of ambulatory blood pressure monitoring (ABPM) in predicting the global progression of cerebral small vessel disease (cSVD). DESIGN: Longitudinal cohort study. SETTING: Data from the population-based Investigating Silent Strokes in Hypertensives study. PARTICIPANTS: Individuals with hypertension who were 50 to 70 years of age and stroke free at baseline. In baseline and follow-up visits, patients underwent magnetic resonance imaging and ABPM. MEASUREMENTS: Ambulatory systolic blood pressure (SBP) and diastolic blood pressure (DBP) levels were studied as continuous variables and dichotomized according to good or poor control on the basis of 125/75 (24 hours), 130/80 (day), and 110/65 (night) mm Hg cutoff values. Whole cSVD progression was qualitatively scored with 1 point when an incident lesion (incident lacunar infarcts, deep cerebral microbleeds, white matter hyperintensities, and basal ganglia enlarged perivascular spaces) was detected. The score ranged from 0 to 4. RESULTS: We followed up 233 participants with a median age of 65 years within 4 years. A total of 61 (26.2%) and 23 (9.9%) subjects showed cSVD progression in one and two or more markers, respectively. Baseline ambulatory SBP and DBP and nighttime pulse pressure (PP) values were positively correlated with the number of incident cSVD lesions. Interestingly, patients without incident lesions showed greater differences between office and ambulatory BP, thus suggesting an increased white coat effect. Poor DBP control, nighttime PP, and DBP white coat effect were independently associated with cSVD progression. The inclusion of these metrics in a clinical model resulted in a significant increase in the prediction of incident lesions (integrated discrimination improvement = 9.09%; P value <.001). CONCLUSION: ABPM may help assess cSVD risk of progression, especially by the identification of poor BP control, masked hypertension, and increased nighttime PP. J Am Geriatr Soc 68:2232-2239, 2020.

Patterns of motor recovery and structural neuroplasticity after basal ganglia infarcts.

OBJECTIVE: To elucidate the timeframe and spatial patterns of cortical reorganization after different stroke-induced basal ganglia lesions, we measured cortical thickness at 5 time points over a 6-month period. We hypothesized that cortical reorganization would occur very early and that, along with motor recovery, it would vary based on the stroke lesion site. METHODS: Thirty-three patients with unilateral basal ganglia stroke and 23 healthy control participants underwent MRI scanning and behavioral testing. To further decrease heterogeneity, we split patients into 2 groups according to whether or not the lesions mainly affect the striatal motor network as defined by resting-state functional connectivity. A priori measures included cortical thickness and motor outcome, as assessed with the Fugl-Meyer scale. RESULTS: Within 14 days poststroke, cortical thickness already increased in widespread brain areas (p = 0.001), mostly in the frontal and temporal cortices rather than in the motor cortex. Critically, the 2 groups differed in the severity of motor symptoms (p = 0.03) as well as in the cerebral reorganization they exhibited over a period of 6 months (Dice overlap index = 0.16). Specifically, the frontal and temporal regions demonstrating cortical thickening showed minimal overlap between these 2 groups, indicating different patterns of reorganization. CONCLUSIONS: Our findings underline the importance of assessing patients early and of considering individual differences, as patterns of cortical reorganization differ substantially depending on the precise location of damage and occur very soon after stroke. A better understanding of the macrostructural brain changes following stroke and their relationship with recovery may inform individualized treatment strategies.

Diagnostic and therapeutic challenge in intracerebral hemorrhage due to aneurysm of distal lenticulostriate artery.

OBJECTIVE: To present an atypical case of deep intracerebral hemorrhage caused by the rupture of an aneurysm of the distal lenticulostriate artery. A 42-year-old woman presenting with right-sided mild hemiparesis was diagnosed with an acute left-sided hemorrhage within the basal nuclei. Cerebral angiography revealed a microaneurysm of the distal portion of the left lenticulostriate artery. Conservative treatment under close neurological surveillance was recommended. After a few days of hospital stay, the patient's neurological symptoms improved, and she was discharged. The patient has remained asymptomatic for more than 6 months. Aneurysms of the distal lenticulostriate artery are extremely rare.

OBJETIVO: Presentar un caso atípico de hemorragia intracerebral profunda causada por la ruptura de un aneurisma de la arteria lenticuloestriada distal; se revisa la bibliografía y se analizan la fisiopatología y el tratamiento. Se presentó a la sala de emergencias una mujer de 42 años con hemiparesia leve del hemicuerpo derecho. Se la diagnosticó con una hemorragia aguda en los núcleos de la base izquierdos. La panangiografía cerebral reveló un microaneurisma de la porción distal de la arteria lenticuloestriada izquierda. Se recomendó tratamiento conservador bajo estrecha vigilancia neurológica. Después de unos cuantos días de hospitalización, los síntomas neurológicos de la paciente mejoraron y la paciente recibió el alta. La paciente ha permanecido asintomática durante más de seis meses. Los aneurismas de la arteria lenticuloestriada distal son extremadamente raros.

Basal ganglia infarction after mild head trauma in pediatric patients with basal ganglia calcification.

OBJECTIVES: Incidence rate of basal ganglia infarction (BGI) after mild head trauma was reported higher in children with basal ganglia calcification (BGC). We would like to review patients with BGC showed in head CT scan to see the incidence rate of stroke in these patients and the correlation of variables in these cases. PATIENTS AND METHODS: CT imaging data of cases with diagnosis of mild traumatic brain injury (mTBI) in a large tertiary pediatric center between Mar. 2014 and Mar. 2019 was retrospectively reviewed. Cases with findings of punctate calcification in the region of basal ganglion in CT scan were included. Correlation of variables of these cases (age, side and volume of basal ganglion calcification) with the diagnosis of BGI was the focus of this study. RESULTS: 37 patients (26 males, 9 females, median age: 3.88±3.54) were included in this study. 17 cases (45.9 %) were diagnosed of BGI and were admitted into the department of neurosurgery. Altogether 63 sides of BGC were categorized into two groups based on whether BGI happened and ROC curve was drawn. ROC curve showed when the cut-off point was 6.55 mm3, the sensitivity was 88.9 % and the specificity was 87.5 %; the area under curve was 0.849 (p<0.01). All the cases were divided into two groups according to whether basal ganglia infarction occurred or not. Mann-Whitney U test showed significant difference between these two groups in age (p=0.01). ROC curve of how age affect BGI after mTBI were drawn. The cut-off point was 3.25 years, and the sensitivity was 65.0 % and the specificity was 88.2 %; the area under curve was 0.746 (p=0.01). All patients received conservative treatment and recovered. CONCLUSION: Incidence rate is higher in children with BGC after mild head injury than that of other children. Larger BGC volume indicates higher risk of developing infarction after minor head injury. Older children with BGC are less-likely getting BGI after mTBI.

Size, shape and location of lacunar strokes and correlation with risk factors.

OBJECTIVE: We evaluated a prospective cohort of 150 patients under observation in our centre for lacunar strokes. The purpose of this study was to investigate if lacunar stroke of varying size, shape and locations had different risk factors between them and possibly different mechanisms and causes. PATIENTS AND METHODS: 150 patients with a lacunar stroke were included in the present study. Infarcts were classified by size, shape and location. We evaluated the correlation between several risk factors of stroke and the radiological characteristics of the infarcts. RESULTS: Older age was associated with the presence of a basal ganglia lesion (p < 0.001) and with the presence of a smaller lesion (trend to statistical significance, p = 0.07). Patients with infarcts >15 mm had higher NIHSS score at admission (p 0.01). CONCLUSION: The different subtypes of lacunar infarcts have distinct pathogenesis. Further studies, with a larger numbers of patients, are necessary to confirm our data.

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.