Midbrain infarction in inherited protein S deficiency: a rare association

Inherited thrombophilic disorders are well‐established predisposing factors for venous thromboembolism, but their role in arterial ischaemic stroke is uncertain. The exact mechanism of arterial thrombosis in thrombophilias remains elusive. Herein, we report a case of a 30-year-old woman who was admitted to our facility with sudden-onset right-sided ptosis and ophthalmoplegia. Detailed clinical features, neuroimaging and laboratory evaluation clinched the diagnosis of ischaemic stroke in midbrain due to microvascular obstruction associated with isolated protein S deficiency. She was treated with oral anticoagulant (warfarin) and physiotherapy; without any improvement of her symptoms at 2 months of follow-up. A high index of clinical suspicion is needed in any case of young ischaemic stroke in absence of common cardiac and vascular risk factors, to recognise the presence of inherited thrombophilia.

Incidence and predictors of epilepsy in children with congenital heart disease

Objective: Children with CHD may be at increased risk for epilepsy. While the incidence of perioperative seizures after surgical repair of CHD has been well-described, the incidence of epilepsy is less well-defined. We aim to determine the incidence and predictors of epilepsy in patients with CHD. Methods: Retrospective cohort study of patients with CHD who underwent cardiopulmonary bypass at <2 years of age between January, 2012 and December, 2013 and had at least 2 years of follow-up. Clinical variables were extracted from a cardiac surgery database and hospital records. Seizures were defined as acute if they occurred within 7 days after an inciting event. Epilepsy was defined based on the International League Against Epilepsy criteria. Results: Two-hundred and twenty-one patients were identified, 157 of whom were included in our analysis. Five patients (3.2%) developed epilepsy. Acute seizures occurred in 12 (7.7%) patients, only one of whom developed epilepsy. Predictors of epilepsy included an earlier gestational age, a lower birth weight, a greater number of cardiac surgeries, a need for extracorporeal membrane oxygenation or a left ventricular assist device, arterial ischaemic stroke, and a longer hospital length of stay. Conclusions: Epilepsy in children with CHD is rare. The mechanism of epileptogenesis in these patients may be the result of a complex interaction of patient-specific factors, some of which may be present even before surgery. Larger long-term follow-up studies are needed to identify risk factors associated with epilepsy in these patients.

Clinical Diversity of Cerebral Sinovenous Thrombosis and Arterial Ischaemic Stroke in the Neonate: A Surveillance Study

<b><i>Introduction:</i></b> Relative risk, risk factors, clinical presentation, onset of symptoms, and age at diagnosis differ between arterial ischaemic stroke (AIS) and cerebral sinovenous thrombosis (CSVT) in neonates. Distinguishing these 2 entities in time clinically can be of eminent importance. <b><i>Methods:</i></b> Active surveillance for AIS and CSVT was performed in 345 German paediatric hospitals. Only cases confirmed by cerebral MRI were included in our analysis. Patients with AIS were compared to CSVT cases with regard to age at diagnosis, pattern of clinical symptoms, and risk factors. <b><i>Results:</i></b> Data on 144 AIS and 51 CSVT neonatal cases were collected from 2015 to 2017. The relative risk of AIS was 2.8 (95% CI 2.1; 3.9) times higher than that of CSVT. CSVT patients were more likely to be born premature (CSVT 14/48, 29.2%; AIS 19/140, 13.2%; <i>p</i> = 0.02) and to have signs of perinatal acidosis (e.g., umbilical artery pH ≤ 7.1 30.2% CSVT vs. 13.5% AIS <i>p</i> = 0.01). Generalized seizures and lethargy were more likely to occur in infants with CSVT (<i>p</i> &#x3c; 0.0001). Age at onset of symptoms and at time of diagnoses was shifted to older ages in CSVT (<i>p</i> &#x3c; 0.0001). <b><i>Discussion/Conclusion:</i></b> The risk for AIS is about 3 times higher than that for CSVT in neonates. A higher proportion of critically ill infants in CSVT and a later onset of symptoms may indicate that perinatal and postnatal risk factors are more important for CSVT than for AIS. The data underline the need for an increased awareness of CSVT in critically ill infants.

Child with tuberculous meningitis and COVID-19 coinfection complicated by extensive cerebral sinus venous thrombosis

We herein report a case of a child with tuberculous meningitis and COVID-19 coinfection complicated by hydrocephalus, arterial ischaemic stroke and extensive cerebral sinus venous thrombosis. Both conditions induce a proinflammatory cytokine drive resulting, among others, in a prothrombotic state. The disruption of the coagulation system in this case was supported by elevated D-dimers, fibrinogen and ferritin levels, consistent with thrombotic complications reported in some adult patients infected with COVID-19. The child also exhibited prolonged viral shedding that suggests severe disease.

Childhood Arterial Ischaemic Stroke: Clinical Presentation, Risk Factors and Management

Childhood arterial ischaemic stroke (AIS) is a rare, but potentially life-threatening event which requires early diagnosis and adequate treatment. The reported significant time delay to childhood AIS diagnosis may be associated with low awareness, the more nonspecific clinical presentation as well as difficult clinical differentiation to more common “stroke mimics” and a less established “acute care structure” with delayed access to proper neuroimaging. Compared with adult stroke care, experiences with acute reperfusion therapies like thrombolysis and mechanical thrombectomy are promising but limited and not based on clinical trials. The etiological work-up is absolutely essential, as the child's individual risk profile determines acute management, secondary prevention, risk of recurrence and outcome. Follow-up care should be organized in a multidisciplinary setting covering all bio-psycho-social aspects to achieve the best integration of the child into its educational, later professional and social environments.