Steinlin M I, Nadal D, Eich G F, Martin E, Boltshauser E J
Department of Pediatrics, University Children's Hospital, Zurich, Switzerland.
Pediatr Neurol. 1996 Oct;15(3):249-53. doi: 10.1016/s0887-8994(96)00170-1.
Fetal Cytomegalovirus (CMV) infection in early pregnancy usually results in severe neurological handicap and sensorineural hearing loss with typical neuroradiological findings of calcification, migrational anomalies, disturbed myelination, and cerebellar hypoplasia. Infections acquired in late pregnancy have less prominent signs, such as microcephaly, hearing deficits, and minor neurological handicap. We report 7 children who presented with a similar clinical complex of signs: microcephaly, sensorineural hearing impairment, behavior problems with hyperactivity, reduced apprehension for pain in 5 of the 7, ataxia in 3, and hypotonia with clumsiness in 3 others. All manifested mild to severe developmental problems. Cranial CT revealed calcification in 4 of 6 patients. MRI in all 7 children showed patchy to confluent nonprogressive dysmyelination. Only 2 children had acute neonatal signs of congenital CMV infection. We assume that these children acquired CMV infection in the third trimester of gestation, leading to microcephaly, hearing loss, and neurological and developmental problems with typical neuroradiological signs.
妊娠早期的胎儿巨细胞病毒(CMV)感染通常会导致严重的神经功能障碍和感音神经性听力损失,并伴有钙化、迁移异常、髓鞘形成紊乱和小脑发育不全等典型的神经放射学表现。妊娠晚期获得的感染症状则不那么明显,如小头畸形、听力缺陷和轻度神经功能障碍。我们报告了7名具有相似临床症状的儿童:小头畸形、感音神经性听力障碍、多动行为问题、7名中有5名对疼痛的感知降低、3名共济失调、另外3名肌张力减退伴动作笨拙。所有患儿均表现出轻度至重度的发育问题。6名患者中有4名头颅CT显示钙化。所有7名儿童的MRI均显示片状至融合性的非进行性髓鞘形成异常。只有2名儿童有先天性CMV感染的急性新生儿体征。我们推测这些儿童在妊娠晚期获得CMV感染,导致小头畸形、听力损失以及具有典型神经放射学体征的神经和发育问题。