Echoencephalography of encephalopathy due to congenital lymphocytic choriomeningitis virus

Abstract

Congenital lymphocytic choriomeningitis virus (LCMV) infection is a transient intrauterine viral infection with selective teratogenicity depending on the timing of the primary maternal infection. LCMV is cleared from the body of the fetus after the acute phase of illness and, in most cases, has lost its virulence at the time of birth. LCMV competes with extracellular matrix proteins for its tropism toward heavily glycosylated alpha-dystroglycan. In the first trimester, while tropism toward heart myoblasts is high, intrauterine infection (IUI) can lead to miscarriage or fetal demise. Later in pregnancy, LCMV becomes neurotropic with tropism toward retina and brain. Most often systemic symptoms are subtle or absent at the time of birth. The timing of the IUI determines the neuroteratogenic phenotypes of congenital LCMV infection. IUI that occurs early during gestation can lead to isolated cerebellar dysgenesis and hypoplasia. When IUI occurs during neural radial migration, echoencephalography shows defective cerebral opercularization, intracranial calcifications at the gray-white matter junction, enlarged extraaxial subarachnoid space, and cortical dysplasia. When IUI occurs after the completion of insular opercularization (late second trimester), echoencephalography shows extensive cystic periventricular leukomalacia with bi-occipital porencephaly and mild defects in gyrogenesis. A late gestation IUI induces aseptic LCMV meningitis with occlusion of the aqueduct of Sylvius, leading to congenital obstructive hydrocephalus. Congenital LCMV remains underdiagnosed as clinical manifestations are predominantly neurologic.As echoencephalography remains the preferred screening strategy for detecting neonatal central nervous system pathology, prior knowledge of echoencephalography of congenital LCMV may facilitate its recognition, prompting a serological evaluation.

Cobblestone‐like brain malformation with a new bi‐allelic ADGRG1 (GPR‐56) mutation: Fetal imaging‐pathology correlation

“…They also noted that PMG is a group of disorders that spares the cerebellar cortex in most cases 13 . Cortical cerebellar dysplasia in association with PMG has been reported in intrauterine cytomegalovirus (CMV), Zika, or lymphocytic choriomeningitis virus (LCMV) infections 35,36 . PMG with cerebellar dysplasia can occur in metabolic disorders, such as peroxisomal disorders, 37 and can be also a feature of some tubulinopathies 38,39 and ciliopathies 40,41 …”

“…13 Cortical cerebellar dysplasia in association with PMG has been reported in intrauterine cytomegalovirus (CMV), Zika, or lymphocytic choriomeningitis virus (LCMV) infections. 35,36 PMG with cerebellar dysplasia can occur in metabolic disorders, such as peroxisomal disorders, 37 and can be also a feature of some tubulinopathies 38,39 and ciliopathies. 40,41 Because of the similarity of the brain pathology in patients with ADGRG1/GPR56 mutations with that reported in CMD, the original term BFPP is better replaced by a more appropriate one, such as "cobblestone/polymicrogyria-like cortical dysplasia" in order to pool together this spectrum of anomalies seen in both disorders.…”

Understanding the Neurotrophic Virus Mechanisms and Their Potential Effect on Systemic Lupus Erythematosus Development

“…Once LCMV infection of the CNS occurs, cells such as neurons and astrocytes are affected [222]. On the other hand, when a woman acquires LCMV infection during the second trimester of pregnancy, it has been observed that this infection can lead to the development of congenital disabilities in the fetus [110,111]. These defects may include hydrocephalus and chorioretinitis (Figure 1B) (Table 1) [110].…”