What isLenticulostriate Vasculopathy?
Lenticulostriate Vasculopathy is a common diagnostic finding on routine neonatal cranial ultrasound examinations. The term refers to hyperechogenic (bright) vessels in the basal ganglia and thalamus regions found on neonatal cranial ultrasonograms. The incidence range varies from 0.3% to 32%, depending on the population under study. It was first identified about three decades ago, after which the incidence rate has increased due to advancements in imaging techniques.
Some studies suggest Lenticulostriate Vasculopathy (LSV) is a benign phenomenon seen in otherwise normal (healthy) infants. LSV has great clinical significance, as many clinical conditions are linked to LSV findings.
It is also referred to as:
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Mineralizing vasculopathy.
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Thalamostriate vasculopathy.
Mineralizing vasculopathy is a known risk factor for basal ganglia stroke following a minor head injury in infants and young children.
What Are the Infectious Conditions Linked to LSV?
LSV has been linked to the following infectious etiologies:
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CMV (Cytomegalovirus).
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Amniotic infection.
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Rubella.
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Streptococcus sepsis.
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Toxoplasmosis.
Of all the above infections linked to LSV, cytomegalovirus infection has been studied the most. Congenital cytomegalovirus infection is reported in 0.5–2 percent of all live births. Congenital cytomegalovirus infection can result in neurological consequences like epilepsy, neurodevelopmental disability, and hearing loss. Other symptoms include microcephaly (the infant’s head is smaller), petechiae, seizures, jaundice, lethargy, poor sucking (inadequate sucking reflex), and migrational brain disorder (caused by the abnormal migration of nerve cells in the developing nervous system). Studies suggest that LSV was detected in 54.3% of neonates diagnosed with congenital CMV infection. Some studies suggest LSV as a possible positive marker for sensorineural hearing loss in neonates with congenital CMV.
What Are the Non-infectious Conditions Associated With LSV?
The perinatal and neonatal non-infectious conditions linked to LSV are:
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Down Syndrome (a genetic disorder characterized by an extra chromosome)
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Cardiac disease.
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Congenital malformations.
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Diabetic fetopathy (a severe complication of pre-existing maternal diabetes mellitus)
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Respiratory distress syndrome (a breathing disorder mostly seen in premature babies)
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Hypoxic ischemic state.
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Hydrops fetalis (a life-threatening condition characterized by abnormal fluid accumulation in the fetus)
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Trisomy 13 syndrome.
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Hyperbilirubinemia (an elevated bilirubin amount in the fetal blood resulting in yellowish discoloration of the skin and eyes).
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Neonatal lupus erythematosus (An uncommon acquired autoimmune condition due to placental transfer of maternal autoantibodies)
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Twin-to-twin transfusion (an uncommon pregnancy condition that affects identical twins).
The link between the above conditions and LSV is based on small, sample-sized studies. Better large sample studies are required to confirm the association.
What Is the Link Between LSV and Neurodevelopment?
Neonates identified with isolated LSV are usually found to have normal neurodevelopment and a favorable prognosis in the long term. Some studies suggest that premature babies with LSV were found to have lower neurodevelopment, especially in relation to behavioral and cognitive development. Such babies with developmental delay and LSV were found to have some other associated conditions like neonatal hypoxia, fetal alcohol exposure, CMV infection, systemic Streptococcus sepsis, and major malformations.
The basal ganglia and thalamus are important regions of the neonatal brain that help coordinate bodily movements, cognition, and emotions. The blood supply to these regions is provided by lateral striate or lenticulostriate arteries. In normal cases, the arteries that supply the thalamus and basal ganglia are not visible on the cranial ultrasound of the neonates. Therefore, the linear or branching echogenic stripes that appear on neonatal cranial ultrasound in the thalamus and basal ganglion region are referred to as LSV.
What Is the Link Between LSV and Neuropsychiatric Disorders?
Some studies have found LSV to be linked to the following neuropsychiatric disorders:
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Attention deficit hyperactivity disorder (a common neurodevelopmental disorder characterized by hyperactivity, impulsivity, and increased inattention).
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Neurological deficits.
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Obsessive-compulsive disorder.
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Developmental delay.
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Tics.
However, large-scale studies are needed on the topic.
What Is the Link Between Infantile Basal Ganglia Stroke and LSV?
Studies by Teele et al showed the presence of hypercellular lesions or mineralization within the arterial walls. This condition was referred to as mineralizing vasculopathy. Due to this mineralization, the lenticulostriate arteries turned more rigid and became more vulnerable to shear injury compared to the normal vessels. Due to this, even a mild head injury could result in an arterial injury.
This results in vasospasm and thrombosis, leading to an ischemic stroke. The infantile basal ganglia stroke, which occurs due to a mild head injury, is uncommon and points to lenticulostriate calcification as the pathological cause. Studies also suggest that all neonates with LSV may not have a mineralizing vasculopathy. Mineralizing vasculopathy is a known and recognized risk factor for basal ganglia stroke following a minor head injury in infants and young children.
It can be diagnosed by a head CT (computed tomography) and is characterized by basal ganglia calcification. These are punctuated hyperdensities on axial images and linear hyperdensities on sagittal and coronal images. Mineralizing lenticulostriate vasculopathy represents the end stage of lenticulostriate vasculopathy and has emerged as a common risk factor for basal ganglia stroke in children. The outcome of stroke in such children is mostly favorable, with most achieving a complete or nearly complete recovery of their motor functions.
Conclusion
LSV is a common radiological finding on routine neonatal cranial ultrasonograms. LSV is mostly linked to CMV infections. Infants and neonates with low-grade LSV were found to have normal neurodevelopment. Normal neurodevelopment is expected if an otherwise healthy neonate is detected with LSV, and an ultrasound follow-up for LSV is done regularly. In high-grade LSV cases, tests are conducted to detect congenital CMV infections.
If LSV is associated with other disease conditions, other required tests will be carried out. Though the occurrence of neonatal basal ganglia stroke is low, if neonates are found to have a high-grade LSV, then parents should be informed of the possible risks. Caution should be taken to prevent such affected neonates from falling from high places and beds.
