Béatrice Husson

Stroke in Childhood: Outcome and Recurrence Risk by Mechanism in 59 Patients:

This paper describes 59 patients, 3 months to 16 years of age, who were seen consecutively in the same center for cerebral arterial infarction. It focuses on the mechanism of stroke. The pathophysiologic process could be established for 78% of the children. Arteriopathic stroke (31 patients, or 53%) was the most common. The arteriopathies were either progressive (moyamoya in 4 patients, or 7%) or nonprogressive (27 patients, or 46%). The latter form occurred in two patterns: dissection of cervicocephalic arteries (12 patients, or 20%) and transient cerebral arteriopathy of unknown origin but probably angiitis (15 patients, or 25%). Cardiac or transcardiac embolic stroke occurred in 12% of the series and systemic diseases in 14%. There was a favorable outcome in 70% of patients having stroke due to nonprogressive arterial disease and stroke due to unidentified mechanisms. In contrast, only 26% of patients with embolic stroke, systemic disease, or moyamoya had a favorable outcome. Recurrences were more frequent and severe in this latter group. It is concluded that it is important to determine the mechanism of childhood stroke, because it strongly influences outcome, the recurrence risk, and treatment choice. (J Child Neurol 2000;15:290-294).

Magnetic Resonance Angiography in Childhood Arterial Brain Infarcts A Comparative Study With Contrast Angiography

Background and Purpose— Contrast angiography (CA) is the reference examination for the diagnosis of cerebral arterial abnormality, but this procedure is invasive. In childhood, ischemic strokes are being increasingly investigated by means of MRI, including MR angiography (MRA). Very few data are available about the accuracy of MRA compared with CA in the specific context of acute pediatric stroke. We sought to compare the results of MRA with those of CA for the study of cerebral arteries in children with arterial infarction in an arterial distribution. Methods— Twenty-four children presenting with 26 infarcts were studied. All were examined with cerebral MRI and MRA and with CA. The interval between CA and MRA was <3 days for most of the patients. Results— Arterial lesions were detected in all but 2 children. They were located in the major cerebral arteries, predominantly in the anterior circulation (85% of cases). All lesions shown by CA were present on MRA (19 cases). Patients with no lesion on MRA had normal CA (2 cases). Associated distal vascular lesions and degree of arterial stenosis were more accurately detected with CA. Conclusions— MRA is sensitive enough to provide an adequate initial evaluation of arterial brain disease in childhood.

New insights (and new interrogations) in perinatal arterial ischemic stroke

With an incidence of 1/2800 to 1/5000 live-births, perinatal arterial ischemic stroke is the most frequent form of cerebral infarction in children. About 40% of the children do not have specific symptoms in the neonatal period, and are only recognized later with the emergence of motor impairment, developmental delay, specific cognitive deficiency or seizures. In the remaining 60%, children present with early symptoms, mostly recurrent focal seizures in the first 3 days of life. The diagnosis is easily confirmed by cranial ultrasounds and MRI. Early MRI has both a key role in the diagnosis, dating the injury, but also an important prognostic value to predict the motor outcome of the child. Indeed, although the infarct does not recur, the majority of children show subsequent sequels: cerebral palsy, epilepsy, cognitive or behavioural problems. Finding predictors of outcome regarding these latter concerns (and the way to prevent or alleviate them) is of major interest. The main etiological hypothesis for perinatal AIS is a cerebral embolus, originating from the placenta through the foramen ovale. Most of the established risk factors are indeed either determinants or biomarkers of vasculo-placental pathology. Injury to the cervico-cerebral arteries, giving rise to thrombus/embolus during the birthing process is also suggested. Both placento-embolic and traumatic theories are supported by a few, but well-analysed pathological or arteriographic reports. Nevertheless, their relative frequency, the implication of other mechanisms, and their repercussions to evidence-based preventive strategies remain to be determined. Moreover, the mechanism of stroke in the different groups of newborns with stroke (term vs. preterm; symptomatic neonates vs. those with a delayed presentation) is likely to be different, and there is a need for future studies to assess all populations as different entities. Neonatal supportive care remains important for all infants while there is no evidence for preventive anticoagulant use at present. In an effort to reduce neurological dysfunction, and in adjunction with ongoing physical therapy and pharmacological treatment, new rehabilitative interventions, such as constraint-induced movement therapy and mirror therapy, are increasingly being used.

Acute transverse myelitis in children: Clinical course and prognostic factors

The objective of this study was to describe the clinical course of acute transverse myelitis in children, to identify prognostic factors, and to compare our findings with published data. Twenty-four children, aged 2 to 14 years and admitted with a diagnosis of acute transverse myelitis, were studied. Clinical features and results of investigations were collected at admission and during the course of the disease. Motor, sphincter, and global outcomes were compared with those in the main adult and pediatric series. During the initial phase, the most common presenting symptoms were pain (88%) and fever (58%). Motor loss preceded sphincter dysfunction in two thirds of patients and became bilateral in half of the patients. When maximal deficit was achieved (plateau), the patients presented a combination of sensory, motor, and sphincter dysfunctions without radicular involvement. The motor loss consistently involved the lower limbs but was inconsistent and moderate in the upper limbs. The mean duration of the plateau was 1 week. The recovery phase was characterized by a progressive improvement of all deficits. Sphincter dysfunction improved more slowly than did the other deficits. A full recovery was achieved by 31% of the patients; minimal sequelae were present in 25% and mild to severe sequelae in 44%. An unfavorable outcome was associated with complete paraplegia (P = .03) and/or a time to maximal deficit shorter than 24 hours (P = .005). A favorable outcome was associated with a plateau shorter than 8 days ( P = .03), the presence of supraspinal symptoms (P = .01), and a time to independent walking shorter than 1 month (P = .01). The course of acute transverse myelitis in children proceeds through three stages, an initial phase, a plateau, and a recovery phase, each characterized by specific clinical features. The global outcome was favorable in 56% of patients. Several prognostic factors were identified. (J Child Neurol 2003;18:401—406).

Ischaemic stroke from dissection of the craniocervical arteries in childhood: report of 12 patients.

Dissection of craniocervical arteries is the most common non-atherosclerotic cause of stroke in young adults. During childhood, it is described primarily as isolated reports. Among 59 patients with arterial ischaemic stroke seen consecutively in the same institution, 12 had a dissection of a cervical or cerebral artery. The diagnosis was established through imaging features. The dissection involved the cervical arteries in five patients and intracranial arteries in seven. A cervical or facial trauma preceded the onset of cerebral ischaemic symptoms in four patients with extracranial dissection by a few minutes to 10 days. For another six patients, the stroke occurred during physical exertion. The neurological deficit was preceded or associated with an intense headache or neck pain in nine patients. Initial treatment consisted of anticoagulation therapy in two patients with extracranial dissection, and aspirin in nine. There was only one recurrence of stroke after a mean follow-up of 3 years and 6 months. Four patients had persistent disabling neurological deficit. Dissection of cervical or cerebral arteries appears to be a common cause of stroke in childhood.

Expanding spectrum of encephalitis with NMDA receptor antibodies in young children.

The authors report here 2 cases of subacute-onset encephalitis with N-methyl-D-aspartate (NMDA) receptor antibodies. One had a paraneoplastic syndrome associated with a neuroblastoma, whereas the other had no primary tumor. This disease was originally described as a paraneoplastic syndrome in young women with ovarian teratoma. The clinical features of both children resembled the typical symptoms reported for older patients with this disease: psychomotor deterioration, movement disorders, and seizures. One of the reported cases is the first known case of paraneoplastic encephalitis with NMDA antibodies in a child with neuroblastoma. Both cases described here were younger than any of the previously reported cases. Consistent with recently published series, this report suggests that the spectrum of symptoms of encephalitis with NMDA receptor antibodies is probably wider than previously thought.

Motor outcomes after neonatal arterial ischemic stroke related to early MRI data in a prospective study.

OBJECTIVE: We aimed to correlate early imaging data with motor outcomes in a large, homogeneous, cohort of infants with neonatal (diagnosed before 29 days of life) arterial ischemic stroke (AIS). METHODS: From a prospective cohort of 100 children with neonatal AIS, we analyzed the MRI studies performed within the 28 first days of life for 80 infants evaluated at 2 years of age. The relationships between infarction location and corticospinal tract (CST) involvement and motor outcomes were studied RESULTS: Seventy-three infarctions involved the middle cerebral artery (MCA) territory. Of those, 50 were superficial infarctions, 5 deep infarctions, and 18 mixed infarctions. The CST was involved in 24 cases. Nineteen patients with MCA infarctions (26% [95% confidence interval: 16%–34%]) developed hemiplegia. Mixed infarctions (P < .0001) and CST involvement (P < .0001) were highly predictive of hemiplegia. In contrast, 88% of children with isolated superficial MCA infarctions did not exhibit impairment. CONCLUSIONS: Accurate prediction of motor outcomes can be obtained from early MRI scans after neonatal AIS. The absence of involvement of the CST resulted in normal motor development in 94% of cases. CST involvement resulted in congenital hemiplegia in 66% of cases.

Obstetrical and neonatal characteristics vary with birthweight in a cohort of 100 term newborns with symptomatic arterial ischemic stroke

OBJECTIVES Many questions remain regarding the mechanism of perinatal stroke. METHODS In a series of 100 prospectively enrolled term neonates with symptomatic arterial ischemic stroke, we explored family antecedents, pregnancy and delivery conditions and clinical presenting features and distinguished features of the 50 larger infants with the remainder. Cardiac and cervical arterial imaging were performed in 70 and 51 cases. RESULTS Previous fetal loss, first pregnancy, primiparity, twin-gestation, cesarean and traumatic delivery, neonatal distress, male sex and premature rupture of membranes were statistically more common than in the general population. Normal pregnancy proportion and mean birthweight were in the normal range, arguing against a vasculo-placental origin in the majority. Furthermore, there was an excess of large babies. The larger infants were more subject to suffer from acute perinatal events, with a trend for an excess of neonatal distress (p=0.065) and for more severe presenting features (p=0.027), while the lighter were more likely to have experienced longstanding obstetrical risk factors such as complicated pregnancy (p=0.047) and tobacco exposure (p=0.028). Cervical MR angiography showed an internal carotid occlusion in two babies, whereas echo-Doppler was always normal; in one case the two methods were discordant. Echocardiography was non-informative. INTERPRETATION The data from this prospective cohort of neonates with stroke confirm that many obstetrical and perinatal factors are risk determinants. They also suggest that birthweight and gender may be biomarkers of two populations of neonates with different pathological mechanisms. MR angiography appears more sensitive than echo-Doppler for the exploration of the neonatal cervical vasculature.

Radiological approach to disorders of arterial brain vessels associated with childhood arterial stroke—a comparison between MRA and contrast angiography

Recent studies of arterial ischaemic stroke in children have emphasised the higher frequency of cerebral arterial abnormalities than believed previously, explaining some of the strokes thought to have been idiopathic. Moreover, recurrent strokes are significantly more frequent in children with multiple risk factors, including those with demonstrable vascular abnormalities. Thus, cerebral arterial imaging is essential for the understanding and therapeutic approach to this pathology. Contrast angiography (CA) is the reference examination for the diagnosis of cerebral arterial abnormalities, but MR angiography (MRA) is a sensitive and non-invasive method to evaluate the vasculopathy of childhood stroke. In this article we highlight recent data concerning localisation and characterisation of arterial lesions and discuss the yield of MRA compared with CA.

Stroke due to lyme neuroborreliosis: changes in vessel wall contrast enhancement.

Neuroborreliosis is a rare cause of stroke in children. We aim here to demonstrate the diagnostic value of gadolinium‐enhanced magnetic resonance imaging (MRI) for demonstrating vessel wall abnormality in a child with brainstem stroke.