Introduction: Digital subtraction angiography (DSA) is commonly performed post pial synangiosis to establish the degree of neovascularization. However, angiography is invasive, and the risk of ionizing radiation is a concern in children. Our study aimed to identify whether arterial spin labelling (ASL) can predict post-operative angiogram grading. In addition, we aimed to identify whether patients who underwent ASL imaging without DSA had similar postoperative outcomes when compared to patients who received ASL imaging and post-operative DSA. Methods: The medical records of patients undergoing pial synangiosis at a single institution were reviewed during a 10-year period. Results: Twenty-two patients were identified. Twenty-one hemispheres of 13 patients with "ASL only" and 14 hemispheres of 9 patients with "ASL + DSA" were analyzed. Both groups had similar rates of MRI evidence of acute or chronic stroke preoperatively; 61.9% in the ASL only group and 64.3% in the ASL + DSA group. All symptoms improved in frequency after intervention except categories of hemorrhage and non-chronic headaches. We found a significant correlation between revascularization observed on the DSA and the ASL graded by 3 neuroradiologists. The ASL grades in patients with a postoperative Matsushima grade indicating robust neovascularization were significantly higher than the ASL grades of patients with a poor postoperative Matsushima grade. Conclusions: Non-invasive ASL perfusion imaging had an association with post-operative DSA neoangiogenesis following pial synangiosis surgery. There were no significant stroke-risk differences between ASL and ASL + DSA cohorts. Both cohorts show significant improvement in pre-operative symptoms after surgery. Providers may consider obtaining a postoperative ASL MRI imaging in lieu of a catheter angiogram.

Arterial-spin labeling imaging for assessment of neoangiogenesis after pial synangiosis in pediatric moyamoya patients

Background and Aims: Subpial hemorrhages (sPH) were first described in infants in 1972, but they remain underrecognized on neuroimaging and the clinical implications of sPH are poorly understood. The aim of this study is to describe the etiologies, risk factors, clinical presentations, and imaging findings of sPH in neonates. Methods: Institutional Neonatal Intensive Care Unit database was queried for presence of sPH. All infants recorded to have intracranial hemorrhage on brain MRI were reviewed by a pediatric neuroradiologist. Through retrospective chart review, data on pregnancy/birth history, comorbidities, and clinical concerns that led to imaging were collected. Results: Twenty-six infants with sPH were identified: 13 (50%) were preterm, 11 (42%) had low birthweight. Comorbidities included hypoxic-ischemic encephalopathy (n=1, 42%), birth trauma (n=6, 23%), cardiopulmonary disease (n=6, 23%), coagulopathy (n=6, 23%), ventricular shunt (n=3, 12%), severe congenital brain anomalies (n=1, 4%), and chromosomal anomaly (n=1, 4%). MRI acquisition was at a median of 5 days of life (range 1 to 41 days). sPH frequently accompanied intraparenchymal (n=17, 65%), subdural (n=16, 62%), intraventricular (n=13, 50%), and subarachnoid hemorrhage (n=8, 31%). In addition, 7 children (27%) had ischemic stroke. All children with sPH had either another form of hemorrhage, ischemic stroke, or both. In addition, 9 patients (34%) had follow-up MRIs. Development of adjacent encephalomalacia was consistently demonstrated in children with large subpial hemorrhages. Sixteen infants with sPH (62%) had clinical and/or electrographic seizures. Conclusions: sPH are associated with multiple comorbid medical conditions, other types of intracranial hemorrhage, and ischemic strokes. Further studies to evaluate long-term clinical implications of subpial hemorrhages are needed.

A new kind of bleed: Subpial hemorrhages in the neonatal intensive care unit

Case description 16 years old female with JD was admitted for stroke with symptoms of sudden onset right-sided hemiparesis, diplopia, dysarthria & difficulty word finding. On examination,was alert, oriented & neurologically intact except dysarthric speech, ataxic gait, bilateral dysmetria & dysdiadokinesia. MRI brain showed patchy asymmetric areas of T2/FLAIR hyperintensities without diffusion restriction involving pons, bilateral middle cerebellar peduncles & hemispheres. Initially started on immunosuppressive therapy (Steroids, Methotrexate & Cellcept) with concerns for cerebral vasculitis but due to worsening symptoms, imaging findings further investigation revealed positive JC Virus in her CSF fluid. Thus diagnosed with PML. Subsequently was treated with plasmapheresis. After 6 months of treatment & rehab she continues to show improvement. She is now able to sit without support & her speech is more clear. Recent JC virus results show 333 copies/ml (down from 2,900 at time of diagnosis). Recent MRI shows some improvement as well. Discussion: Active replication of JC virus in the brain leads to the development of PML, characterized by focal areas of demyelination and necrosis of glial cells(3). Diagnosis of PML is based clinically, neuroimaging & detection of JC virus in CSF. Most commonly reported neurological features are mono- and hemiparesis (50%), apathy (46%), confusion (38%), visual disturbance (29%), and seizures (21%)(2). In the pediatric population less than 50 cases of PML have been reported related to AIDS & less than 10 cases with inherited immunodeficiency. To our knowledge this is the first PML case reported in a pediatric patient with Juvenile Dermatomyositis (JD) and subsequent immunosuppression(1). References: 1.ReAngelini L, et al. Progressive multifocal leukoencephalopathy in a child with hyperimmunoglobulin E recurrent infection syndrome and review of the literature. Neuropediatrics. 2001;32(5):250-255. doi:10.1055/s-2001-19119 2.Shitrit D, et al. Progressive multifocal leukoencephalopathy in transplant recipients. Transpl Int. 2005;17(11):658-665. doi:10.1007/s00147-004-0779-3 3.Burke MT, et al. Progressive multifocal leukoencephalopathy with gastrointestinal disease in a pediatric kidney transplant recipient. Pediatr Transplant. 2013;17(5):E119-E124. doi:10.1111/petr.12107 Images: Asymmetric T2/FLAIR hyperintense signal within the right cerebellar peduncle the extensive cerebral hemisphere without evidence of diffusion restriction

Progressive multifocal leukoencephalopathy in a child with juvenile dermatomyositis presented as stroke

Introduction: Pediatric stroke is one of the leading causes of morbidity and mortality worldwide. Given the paucity of literature on etiology and presentation of childhood stroke in India, the clinico-etiological profile study would give better insights into understanding, treating and preventing childhood stroke among the Indian population. Methods: This is a Retro-prospective cohort study conducted at Narayana health city, Bangalore over a 6months period. 76 children in the age group of 0-18yrs diagnosed as arterial ischemic stroke clinically and radiologically, were included. Data was collected from hospital database and at OPD visits, analyzed and compared with available Indian and international data. Results: Our study found that most common causes for pediatric stroke were: cardio-embolic (28%), cryptogenic (27%), arteriopathy (17%) and infections (15%). There was a definite male preponderance (66%) and most of them presented at 0-5yrs age (52%). Clinically, common presenting features were hemiparesis (63%), seizures (53%), facial paresis (26%), encephalopathy (22%), fever (17%), headache (9%). 57% of patients had single cerebral hemispheric involvement. Multiple vascular territories were involved in 30%. Nine children had recurrence (11%) of which, four had Moya Moya Disease, two had cardioembolic stroke, one had vertebral artery dissection, one had propionic aciduria and one Prothrombin gene mutation. Most of them were managed conservatively (95%). Four children (7%) underwent interventions: STA-MCA bypass with EDAMS, vertebral artery coiling and 2 mechanical thrombectomy. Conclusions: Our study found the cardio-embolic phenomena as the commonest cause of AIS in India. Rarer causes of stroke were also identified. 27% remained cryptogenic despite exhaustive evaluation. A definite male preponderance was noted. Further multi-centric studies with larger sample size would confirm these findings and shed light on common causes, modes of diagnosis, preventive and curative treatment specific to Indian pediatric population.

A cohort study of clinical and etiological profile of arterial ischemic stroke among Indian pediatric population: A single center experience

The problem of a pediatric stroke has become relevant in recent decades. More than 50% of children with ischemic stroke are diagnosed with arteriopathy. Aim: assessment of the clinical features and course of arteriopathy in children diagnosed with arterial ischemic stroke (AIS). Materials: 72 patients with AIS, who were treated in Moscow primary pediatric stroke center from 2013 to 2018. Boys vs girls - 42 vs 30 (58,4%vs41,6%), the average age 7.6 years. The observation period was from 16 months to 5 years. Results: in 43 children out of 72 were diagnosed the arteriopathy. 30/43 showed signs of unilateral focal stenosis of the distal internal carotid artery (ICA) and / or the middle cerebral artery (CMA) and / or the anterior cerebral artery (PMA) with / without occlusion, The severity of stroke by PedNIHSS in average 7,3. CT was made: CT angiography 28 patients (from 7 days to 4 months from the onset of stroke (1 patient twice), 8 - direct catheter angiography. Arteriopathies in patients with focal cerebral artheropathy were divided: vasculitis- 3, progressive arteriopathy -4, dissection-3, transient cerebral arteriopathy (TCA)- 20. Dynamic MRI study (from 3 to 6 months) showed: a complete restoration of blood flow-5; increase of stenosis -4; decrease of stenosis - 11; without dynamics relatively acute period -3. MR-angiography after 9-12 months: full restoration of blood flow-9; reduction of stenosis (incomplete recovery) -7. Relapse of AIS was observed in 6 patients (11.6%)with artheriopathy during the follow-up period. Conclusion: Modern methods of neuroimaging have significantly improved the diagnosis of childhood stroke, clarifying the nature and extent of cerebral vascular lesions, including artheriopathy, which is very important in determining the management of children and adolescents.

Transient cerebral arteriopathy in children and adolescents, features of clinical manifestations and course

Objectives: The prevalence of cancer among children with ischemic stroke is unknown. This study sought to evaluate cancer-associated childhood ischemic stroke in a multinational pediatric stroke registry. Methods: Children between 29 days and 19 years of age with arterial ischemic stroke (AIS) and/or cerebral sinovenous thrombosis (CSVT) enrolled in the International Pediatric Stroke Study between January 2003 and June 2019 were included. Data including types of cancer, stroke etiology, and stroke treatment were compared between subjects with and without cancer using Wilcoxon rank sum and chi-square tests. Results: Remote or active cancer was present in 99 of 2968 children (3.3%) with AIS and 64 of 596 children (10.7%) with CSVT. Children with cancer were older than children without cancer in both the AIS (9.5 versus 5.7 years, p=0.0001) and CSVT groups (8.2 versus 6.3 years, p=0.014). Among children in whom cancer type was identified, 42 of 88 (48%) AIS cases had brain tumors and 35 (40%) had hematologic malignancies; 45 of 58 (78%) CSVT cases had hematologic malignancies and 8 (14%) had brain tumors. Primary causes most commonly reported in children with cancer-associated AIS included: arteriopathy (n=34, 62%), cardioembolic (n=9, 16%), prothrombotic state (n=4, 7%), and infection (n=4, 7%). The most frequently reported primary cause of CSVT in children with cancer was prothrombotic state (n=38, 83%). Children with cancer were less likely to receive any antithrombotic therapy for AIS (58 % v. 80%, p=0.007) and anticoagulation for CSVT (71% v. 87%, p=0.046), compared with children without cancer. Recurrent AIS (5% v. 2%, p=0.04) and CSVT (5% v. 1%, p=0.006) were more common among children with cancer. CONCLUSION: Cancer is an important risk factor for incident and recurrent childhood stroke. Although multiple risk factors for stroke were noted in children with cancer, presence of cerebral arteriopathy in children with AIS and prothrombotic state in children with CSVT appear to be the most significant underlying mechanisms contributing to increased risk for stroke in this population. Future research investigating the role of stroke prophylaxis in the high-risk subpopulations identified in this study are warranted.

Cancer-associated stroke in children: Results from the International Pediatric Stroke Study (IPSS)

Hemorrhage (HI) accounts for about half of all strokes in children. In total, vascular brain abnormalities accounted for 43.5% of all hemorrhages. Aim: to evaluate the incidence of HI in the primary pediatric stroke center of the Morozov Children City Clinical Hospital; revealed cerebral vascular anomalies without rupture, neurosurgical treatment options. Material: children who were treated in the primary pediatric stroke center from 2017 to 2019, with cerebrovascular diseases, and are included in the Moscow Pediatric Stroke Register. Results: 142 children were enrolled in the register in 2017: 62.2% of ischemic stroke and transient ischemic attacks (IS and TIA), 8.1% of sinus thrombosis (ST) and 29.7% of HI; in 2018, 282 children - 58% IS + TIA, 5.6% ST, 36.4% HI (7% cerebral vessel anomalies without rupture); in 2019, 235 children - 52.7% of IS and TIA, 12.8% of ST, 34.5% of HI (12.8% cerebral vessel anomalies without rupture). Cerebral angiography was performed in 90 patients, of which 45 (50%) patients did not have vascular pathology, 45 (50%) -pathology of cerebral vessels, of which: AVM-in 28 (62.2%) patients, arterial aneurysm - 6 (13.3%), venous angioma i- 4 (8.8%), arteriovenous fistula - 3 (6.6%) patients, ST - 2 (4.4%), Moya-Moya-2 (4.4%). Neurosurgical treatment of 14 patients (2017-2018): 11 (78.6%) with arteriovenous malformations, 2 (14.2%) with cavernomas, 1 (7.1%) aneurysm. Age ranged from 7 to 17 years. Ten of them (71.43%) were operated on: microsurgical -5 (50%), endovascular5 (50%). Conclusion: The neurosurgical service is an important part of the work of primary pediatric stroke centers. Improving diagnostic methods has improved the early detection of cerebral vascular anomalies with the optimal treatment tactics. According to the Moscow pediatric stroke Center, annually the number of first diagnosed cerebral vascular anomalies increases by 30-40%, with the predominance of AVM.

Neurosurgical service in the primary pediatric stroke center in Moscow

Introduction: Brainstem location of arteriovenous malformation occurs in 2-6% of all intracranial AVMs and more commonly presents as hemorrhage in adults with mean age of 25-38.5 years. This is the first local report of two cases presenting during the adolescent period with headaches. Case and Discussion The first case is a 13-year old male who had a one-year history of occipital headache eventually developing left hemiparesis, slurred speech and right-sided ataxia (mRS 3). Brain MRI revealed brainstem AVM extending to the right cerebellum (Figure 1) confirmed by angiography (Figure 2). After instituting medical supportive management, patient was sent home. Gradual improvement of the neurologic deficits (mRS 2) were noted three months after discharge. The second is an 18-year old female taking risperidone for Major Depressive Disorder and Post-Traumatic Stress Disorder who had recurrent episodes of migrainous-like headaches but consulted for galactorrhea. Cranial CT scan showed a midbrain AVM (Figure 3) confirmed by angiography (Figure 4). Endocrine work-up revealed risperidone-induced galactorrhea which resolved after drug withdrawal. Patient was discharged while waiting for definitive management. However, her AVM ruptured four months later manifesting as Weber syndrome (mRS 3). Patient was stabilized and eventually sent home. There is gradual improvement of the left hemiparesis (mRS 2) five months from the hemorrhagic stroke. Considering the poor prognosis of brainstem hemorrhage, these two cases illustrate the need for high-index of suspicion in seemingly benign headaches as early detection and management of this rare condition could spell the difference in their outcomes. Currently, treatment option for these patients is stereotactic radiosurgery. Keywords: pediatric hemorrhagic stroke, brainstem AVM.

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Arterial-spin labeling imaging for assessment of neoangiogenesis after pial synangiosis in pediatric moyamoya patients

A new kind of bleed: Subpial hemorrhages in the neonatal intensive care unit

Progressive multifocal leukoencephalopathy in a child with juvenile dermatomyositis presented as stroke

A cohort study of clinical and etiological profile of arterial ischemic stroke among Indian pediatric population: A single center experience

Transient cerebral arteriopathy in children and adolescents, features of clinical manifestations and course

Cancer-associated stroke in children: Results from the International Pediatric Stroke Study (IPSS)

Neurosurgical service in the primary pediatric stroke center in Moscow

Brainstem arteriovenous malformation in two adolescent Filipinos

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