Child's Nervous System

, Volume 34, Issue 1, pp 95–99 | Cite as

Congenital Zika virus infection: a neuropathological review

  • L. Chimelli
  • E. Avvad-Portari
Focus Session



A relationship between Zika virus (ZikV) infection in pregnancy and the occurrence of microcephaly was established during the Zika outbreak in Brazil (2015–2016). Neuropathological findings in congenital Zika syndrome helped to understand its pathogenetic mechanisms.


The most relevant postmortem findings in the central nervous system (CNS) of fetuses and neonates infected with ZikV early in gestation are microcephaly with ex-vacuo ventriculomegaly and large head circumference associated with obstructive hydrocephalus due to severe midbrain and aqueduct distortion. Babies with severe brain lesions are born with arthrogryposis. Histologically, there is extensive destruction of the hemispheric parenchyma, calcifications, various disturbances of neuronal migration, reactive gliosis, microglial hyperplasia and occasional perivascular cuffs of lymphocytes, also in the meninges. Hypoplastic lesions secondary to the lack of descending nerve fibers include small basis pontis, pyramids and spinal corticospinal tracts. Cerebellar hypoplasia is also common. Severe nerve motor nerve cell loss is observed in the anterior horn of the spinal cord.


A spectrum of neuropathological changes, from severe microcephaly to obstructive hydrocephalus was observed. The severity of the lesions is directly related to the gestational age, the most severe occurring when the mother is infected in the first trimester. Infection of progenitor cells at the germinal matrix was demonstrated. The lack of spinal motor neurons is responsible for fetal acynesia and consequent arthrogryposis.


Congenital Zika virus infection Neuropathology Microcephaly Ventriculomegaly Calcifications Migration disturbances 


Compliance with ethical standards

Conflict of interest

I would also like to inform that neither Dr. Avvad Portari nor myself have any conflict of interest concerning the publication of this manuscript.


  1. 1.
    Oliveira Melo AS, Malinger G, Ximenes R, Szejnfeld PO, Alves Sampaio S, Bispo De Filippis AM (2016) Zika virus intrauterine infection causes fetal brain abnormality and microcephaly: tip of the iceberg? Ultrasound Obstet Gynecol 47:6–7. CrossRefPubMedGoogle Scholar
  2. 2.
    Soares de Oliveira-Szejnfeld P, Levine D, Melo ASO et al (2016) Congenital brain abnormalities and Zika virus: what the radiologist can expect to see? Radiology 281:203–218. CrossRefPubMedGoogle Scholar
  3. 3.
    Lucia de Noronha L, Zanluca C, Azevedo MLV et al (2016) Zika virus damages the human placental barrier and presents marked fetal neurotropism. Mem Inst Oswaldo Cruz 111:287–293. CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Acosta-Reyes J, Navarro E, Herrera MJ et al (2017) Severe neurologic disorders in 2 fetuses with Zika virus infection, Colombia. Emerg Infect Dis 23:982–984. CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Sousa AQ, Cavalcante DIM, Franco LM et al (2017) Postmortem findings for 7 neonates with congenital Zika vírus infection. Emerg Infect Dis 23:1164–1167. CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Driggers RW, Ho C-Y, Korhonen EM et al (2016) Zika virus infection with prolonged maternal viremia and fetal brain abnormalities. N Engl J Med 374:2142–2151. CrossRefPubMedGoogle Scholar
  7. 7.
    Martines RB, Bhatnagar J, de Oliveira Ramos AM et al (2016) Pathology of congenital Zika syndrome in Brazil: a case series. Lancet 388:898–904. CrossRefPubMedGoogle Scholar
  8. 8.
    Mlakar J, Korva M, Tul N et al (2016) Zika virus associated with microcephaly. N Engl J Med 374:951–958. CrossRefPubMedGoogle Scholar
  9. 9.
    Chimelli L, Melo ASO, Avvad-Portari E et al (2017) The spectrum of neuropathological changes associated with congenital Zika virus infection. Acta Neuropathol 133:983–999. CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.Laboratory of NeuropathologyState Institute of Brain Paulo Niemeyer and Federal University of Rio de Janeiro (UFRJ)Rio de JaneiroBrazil
  2. 2.Fernandes Figueira Institute-Oswaldo Cruz Foundation (Fiocruz) and State University of Rio de Janeiro (UERJ)Rio de JaneiroBrazil

Personalised recommendations