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Neuroplacentology is an expanding field of interest that addresses the placental influence on fetal and neonatal brain lesions and on further neurodevelopment. The objective of this study was to clarify the link between placental pathology and perinatal arterial ischemic stroke (PAIS). Prior publications have reported different types of perinatal stroke with diverse methodologies precluding firm conclusions. We report here the histological placental findings in a series of 16 neonates with radiologically confirmed PAIS. Findings were grouped into 3 categories of lesions: (1) inflammation, (2) placental and fetal hypoxic lesions, and (3) placentas with a high birthweight/placenta weight ratio. Matched control placentas were compared to the pathological placentas when feasible. The eight term singleton placentas were compared to a series of 20 placentas from a highly controlled amniotic membrane donation program; in three twin pregnancies, the placental portions from the affected twin and unaffected co-twin were compared. Slightly more than half (9/16, 56%) had histopathological features belonging to more than one category, a feature shared by the singleton control placentas (13/20, 65%). More severe and extensive lesions were however observed in the pathological placentas. One case occurring in the context of SARS-CoV-2 placentitis further expands the spectrum of COVID-related perinatal disease. Our study supports the assumption that PAIS can result from various combinations and interplay of maternal and fetal factors and confirms the value of placenta examination. Yet, placental findings must be interpreted with caution given their prevalence in well-designed controls. [ABSTRACT FROM AUTHOR]

Purpose: Malignant stroke is a life-threatening emergency, with a high mortality rate (1–3). Despite strong evidence showing decreased morbidity and mortality in the adult population, decompressive hemicraniectomy (DCH) has been scarcely reported in the pediatric stroke population, and its indication remains controversial, while it could be a potential lifesaving option. Methods and results: We performed an extensive literature review on pediatric malignant arterial ischemic stroke (pmAIS) and selected 26 articles reporting 97 cases. Gathering the data together, a 67% mortality rate is observed without decompressive therapy, contrasting with a 95.4% survival rate with it. The median modified Rankin score (mRS) is 2.1 after surgery with a mean follow-up of 31.8 months. For the 33% of children who survived without surgery, the mRS is 3 at a mean follow-up of 19 months. As an illustrative case, we report on a 2-year-old girl who presented a cardioembolic right middle cerebral artery stroke with subsequent malignant edema and ongoing cerebral transtentorial herniation in the course of a severe myocarditis requiring ECMO support. A DCH was done 32 h after symptom onset. At the age of 5 years, she exhibits an mRS of 3. Conclusion: Pediatric stroke with malignant edema is a severe condition with high mortality rate if left untreated and often long-lasting consequences. DCH might minimize the vicious circle of cerebral swelling, increasing intracranial pressure and brain ischemia. Our literature review underscores DCH as an efficient therapeutic measure management of pmAIS even when performed after a significant delay; however, long-lasting morbidities remain high. [ABSTRACT FROM AUTHOR]

This document is a reply to a letter commenting on a previous study about a pediatric patient with a cerebellar peduncle pilocytic astrocytoma. The authors of the reply discuss the complex relationship between tumor location, trigeminal nerve involvement, and clinical manifestations in the management of trigeminal neuralgia. They highlight the importance of recognizing and addressing trigeminal nerve involvement in these cases, as it can lead to significant sensory and motor deficits. The authors also mention a recent condylar fracture in their patient, which they hypothesize may be related to secondary masticatory muscle atrophy. They emphasize the need for comprehensive multidisciplinary management strategies and long-term follow-up in these cases. The document concludes by suggesting that more research is needed to explore the long-term outcomes of managing pediatric pilocytic astrocytoma in the posterior fossa. [Extracted from the article]

Dear Editor, We would like to share a rare clinical experience in a child with trigeminal nerve chronic motor denervation caused by cerebellar peduncle pilocytic astrocytoma. The unilateral weakness of the masticatory muscles with temporal muscle atrophy was related to the long-standing compression of the proximal motor roots of the trigeminal nerve related to compressive mass effect of the tumor before diagnosis. [Extracted from the article]

Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution. Younger children also tended to have poorly defined and widespread lesions. The majority of patients with an isolated optic neuritis (n = 16) only presented small non-specific brain lesions or none at all. A longitudinally extensive transverse myelitis mainly affecting the cervical, and less often so the thoracic, lumbar, and conus regions, was detected in 31 children. The three children of our cohort who were then finally diagnosed with multiple sclerosis had at onset already demarcated white matter lesions as well as transverse myelitis. In conclusion, children with MOG seropositive ADS present disparate, yet characteristic imaging patterns. These patterns have been seen to correlate to the disease entity as well as to age of symptom onset. [ABSTRACT FROM AUTHOR]

Partial rhombencephalosynapsis (PRECS) has been recently reported in association with Chiari II (CII). However, its existence as a true malformation is challenged due to the anatomical changes potentially induced by CII. The aim of this report was to investigate the contribution of midbrain/hindbrain tractography in this setting. A 13-year-old boy with a known CII malformation and operated myelomeningocele was referred for brain imaging after a first complex partial seizure. In addition to the classical features of CII, MRI showed partially fused cerebellar hemispheres and multiple supratentorial abnormalities. Diffusion tensor imaging (DTI) color map and tractography showed absent transverse fibers on the midsection of the cerebellum, scarce fibers of the middle cerebellar peduncle (MCP), absence of the middle pontine crossing tract, and fibers running vertically in the medial part of the cerebellum. Vertical mediocerebellar fibers are a feature of classical RECS and the paucity or absence of MCP fibers is mainly described in CII. In our patient, DTI and FT therefore demonstrated structural characteristics of both RECS and CII confirming their potential coexistence and suggesting possible shared embryological pathway. [ABSTRACT FROM AUTHOR]

Background: Congenital ocular motor apraxia (COMA) occasionally shares with Joubert syndrome (JS) and related disorders (JSRDs) a peculiar malformation, the 'molar tooth sign' (MTS). In JSRDs, the absence of superior cerebellar peduncles (SCP) decussation is reported. Objective: To investigate whether COMA demonstrates similar abnormal axonal pathways. Materials and methods: Eight healthy age-matched controls, three children with clinical COMA and one child with clinical JSRD underwent examination with a 1.5-T MRI scanner. Diffusion-weighted imaging (DWI), colour-coded fractional anisotropy maps and three-dimensional diffusion tensor imaging (DTI) tractography of the cerebellorubral network were analyzed. Results: On DTI cartography, the 'red dot' originally supposed to represent the SCP decussation in the midbrain was present in controls as well in those with COMA but absent in the single case with JS. In none of the subjects including controls was 3-D FT able to depict the SCP decussation. When seeded, the red dot resulted in the ventral tegmental decussation (VTD). It was normal in controls and in patients with COMA but was absent in our single patient with JSRD. MTS was identified in alla patients with COMA and in the patient with JSRD. Conclusion: MTS can be present in both COMA and JSRD but the underlying anatomy depicted by fibre tracking is distinct. The main difference is the integrity of the VTD in COMA. [ABSTRACT FROM AUTHOR]

Introduction: Nephrotic children are prone to develop thromboembolic complications secondary to an acquired hypercoagulable state. Cerebral sinovenous thrombosis (CSVT) is increasingly recognised in this population, but clinical characteristics and outcome are not well documented.Patients and Methods: The database of the Canadian Pediatric Ischemic Stroke Registry (Toronto Site) containing prospectively enrolled children from 1992-2004 with CSVT identified four children with NS. A pooled literature analysis retrieved 17 additional cases reports.Results: CSVT presented in the majority of cases during the first flare or within 6 months after the onset of NS and was found to occur more often in SSNS/SDNS (n=13) than in SRNS (n=4). Clinical manifestations were non-specific and consisted primarily of seizures (n=8) and signs of raised intracranial pressure (n=16). Imaging studies revealed a predilection for superior sagittal sinus involvement (n=21) and rare parenchymal lesions (n=4). The most consistent biological risk factors were a severe hypoalbuminaemia (n=14) and, to a lesser extent, decreased antithrombin (AT) levels (n=9/16). Deficiency of other coagulation inhibitors (protein S, protein C) was not identified. Inherited thrombophilia was documented in a single case, suggesting that acquired, more than genetic, coagulation factors are involved. Anticoagulation was safe, and the outcome was good in most patients, and no recurrence of thrombotic event was reported.Discussion: In conclusion, CSVT is now a well-described complication of NS with potential morbidity. A high index of suspicion is required, especially in young children with NS presenting neurological symptoms. Reliable biological predictors of CSVT are lacking. [ABSTRACT FROM AUTHOR]