Mineralizing angiopathy with infantile basal ganglia stroke after minor trauma

Abstract

ABBREVIATIONS CMVCytomegalovirus SLV Sonographic lenticulostriate vasculopathy AIMS The objective of this study was to describe a cohort of infants with basal ganglia stroke associated with mineralization in the lenticulostriate arteries and their clinical outcomes.METHOD Subcortical strokes occurring in infants during the study period were categorized as arterial ischaemic, venous, or haemorrhagic. A cohort of infants with basal ganglia infarcts and associated mineralization of lenticulostriate arteries were identified. This group was analysed for possible aetiological factors, clinical course, and recurrence rate of the stroke. RESULTSOf 23 infants with basal ganglia arterial ischaemic stroke, 22 (16 males, six females; mean age 11mo [AESD 4.8mo]) were found to have lenticulostriate artery mineralization. Twenty infants presented with hemiparesis and two presented with recurrent episodes of hemidystonia. Eighteen infants had a history of minor trauma before onset of stroke. No other predisposing factors were identified in this cohort. There were no demonstrable causes for vascular and soft tissue calcification. The mean follow-up was 11 months, during which five infants experienced stroke recurrence. Of the 17 infants who did not experience a recurrent stroke, eight exhibited complete neurological recovery, and nine had mild residual hemiparesis.INTERPRETATION Acute basal ganglia stroke after minor trauma associated with mineralization of lenticulostriate arteries in infants is a distinct clinicoradiological entity. Investigations for prothrombotic states and vasculopathies are normal. Although neurological outcomes in most children are good, trauma is a risk factor for recurrence of stroke.Basal ganglia stroke has been reported to occur in infants in association with minor falls. Over the last decade, this association has been described in several case reports and small case series. [1][2][3][4] Affected infants present with facial paresis and hemiparesis soon after the injury and small infarcts in the basal ganglia are visible on neuroimaging. This condition has been reported to have a favourable prognosis. Investigations for the cause of stroke in these patients failed to provide an explanation. Although various aetiological factors have been postulated to explain this characteristic presentation and anatomical distribution of the lesions, there is no definitive evidence to prove any pathogenic mechanism. In a recent study, Yang et al. 5 described a series of 16 infants, less than 18 months of age, who developed cerebral infarction after mild head trauma. The authors reported that basal ganglia calcifications and cytomegalovirus (CMV) infection were the only potential risk factors in these infants. In 2008, we noticed the presence of mineralization of the lenticulostriate arteries in infants who had suffered basal ganglia strokes, with the onset of stroke occurring, in most cases, after minor trauma. In this paper, we report the demographic profile, clinical characteristics, imaging features, laboratory fin...

Acute encephalopathy: A novel presentation of mineralizing microangiopathy of childhood

“…[12] It typically presents with acute onset focal neuro deficit with or without seizure following minor trauma. Neuroimaging usually reveals unilateral infarcts in the basal ganglia with mineralization (calcification) in the corresponding lenticulostriate arteries.…”

Lingappa et al. reply

“…Mineralized vessels will, however, not only be rigid and likely to shear, but their contractility and barrier properties are likely to be affected as well. We do not as yet know which cells are involved in vascular calcification, but there is evidence to implicate pericytes 6 (including unpublished results from the present author) which derive from the mesoderm and have osteogenic properties. 7 Basal ganglia vascular calcification appears to involve adventitial or subendothelial cells, where pericytes are found, 8 and in adults basal ganglia calcification is associated with bone matrix proteins.…”

“…Mineralizing angiopathy with basal ganglia stroke in infants after minor trauma is one syndrome that clearly implicates lenticulostriate calcification as the pathological substrate. 6 Information on the platelet-derived growth factor receptor b gene (PDGF-Rb) mutations being associated with idiopathic basal ganglia calcification is also very important in understanding this entity. 7 Further pericyte dysfunction may predispose to breakage of the blood brain barrier and mineralization of lenticulostriate arteries.…”

Childhood Infections and Trauma as Risk Factors for Stroke

“…It has been postulated that the underlying mechanism is that of vasospasm of the lenticulostriate perforators for, as yet, unidentified reasons. A recent series has described a group of children with minor head trauma, basal ganglia infarction and evidence of a mineralizing angiopathy of the lenticulostriate perforators apparent on CT [80, 81]. It is intriguing to speculate why such cases have not been widely reported before; one possible explanation is that most children with AIS are now generally investigated with magnetic resonance imaging rather than CT and of childhood AIS cases overall, this group represents a small minority.…”

“…It is intriguing to speculate why such cases have not been widely reported before; one possible explanation is that most children with AIS are now generally investigated with magnetic resonance imaging rather than CT and of childhood AIS cases overall, this group represents a small minority. Several mechanisms have been postulated, for example mechanical stretching of the mineralized lenticulostriate arteries during trauma, a pre-existing neonatal lenticulostriate vasculopathy and congenital or very early childhood infections (CMV, EBV, Mycoplasma) as precursors of mineralization, suggesting that trauma may not be a cause, but a trigger for occlusion of predisposed vessels [81, 82]. …”