Inflammatory Diseases of the Brain in Childhood Charles R. Fitz1 From the Children's National Medical Center, Washington, DC Pediatric inflammatory disease may resemble found that the frequency of congenital involve­ adult disease or show remarkable, unique char­ ment increases with each trimester, being 17%, acteristics. This paper summarizes the current 25%, and 65%. However, infection severity de­ imaging of pediatric diseases with emphasis on creases in each trimester. The true frequency of those that are the most different from adult ill­ early cases may have been underestimated in nesses. their study, because spontaneous abortions were not included in the retrospective analysis. Congenital Infections Intracranial calcification is the most notable radiologic sign. Basal ganglial, periventricular, Most intrauterine infections are acquired and peripheral locations are all common (Fig. 1). through the placenta, although transvaginal bac­ Large basal ganglial calcifications are related to terial infections may also occur. The TORCH early infection, as is hydrocephalus. The hydro­ eponym remains a good reminder for these enti­ cephalus is invariably secondary to aqueductal ties, identifying toxoplasmosis, others, rubella, stenosis (2), and often has a characteristically cytomegalic virus, and herpes simplex. A second marked expansion of the atria and occipital horns H for HlV or perhaps the words A (AIDS) TORCH (Fig. 2), probably partly due to associated tissue should now be used, as AIDS becomes the most loss. This is associated with increased periventric­ common maternally transmitted infection. ular calcification in the author's experience. Mi­ crocephaly is common, and encephalomalacia is seen occasionally (2). Hydrencephaly has also Toxoplasmosis been reported (3). This infection is passed to humans from cats, Because calcifications are common and fairly since the oocyst of the Toxoplasma gondii para­ characteristic, computed tomography (CT) is site is excreted in cat feces. The incidence of preferable to magnetic resonance (MR) imaging seropositive adult human females is about 90% for diagnosis. Clinically, infants infected with T. in France and in some parts of the United States gondii most commonly show chorioretinitis, sei­ (1). Congenital infection occurs only when the zures, and developmental delay. mother becomes infected during pregnancy. Tox­ oplasmosis is said to affect 3% of pregnancies in Cytomegalovirus France (2), and between 1 in 1,000 to 1 in 8,000 pregnancies in the Unites States (1). The most Cytomegalic virus (CM V) is a herpes virus that complete recent radiologic report of the condition infects nearly every person at some time in his is Diebler et a!' s review of 31 infants (2). They life. Congenital infection occurs when maternal infection or reactivation of infection happens dur­ ing pregnancy (4). The incidence of infection is considerably higher in lower socioeconomic than 1 Address reprint requests to Dr Fitz at the Department of Radiology, middle-class women, with a seroconversion rate Children's Hospital of Pittsburgh, 3705 5th Ave, Pittsburgh, PA 15213. of 6% per year versus 2% per year in the two Index terms: Brain, infection; Encephalitis; Pediatric neuroradiology; Ac­ groups (4). As with toxoplasmosis, earlier mater­ quired immunodeficiency syndrome (AIDS) nal infection results in a more severe infant infec­ AJNR 13:551-567, Mar/ Apr 1992 0195-6108/ 92/ 1302-0551 tion (5). Up to 2% of newborns are said to be © American Society of Neuroradiology positive for the virus (6) . At the author's hospital, 551 552 AJNR: 13, March/April 1992 Fig. 1. Toxoplasmosis in a 1-month-old infant; noncontrast CT. There is extensive basal ganglia calcification plus peripheral calcification primarily in the subcortical white matter. Diffuse atrophy and ventricu­ lar enlargement are present. (Courtesy of Dr Carl Geyer, Walter Reed Army Medical Cen­ ter, Washington, DC.) Fig. 2. Toxoplasmosis with calcification and hydrocephalus; noncontrast CT in a 3 112- week-old infant. There is severe hydroceph­ alus, periventricular and peripheral calcifi­ cation, and intraventricular calcification, probably on inflammatory septations. 1 2 Fig. 3. CMV infection in a 11-week-old microcephalic retarded infant. Typical peri­ ventricular calcifications are present on CT. Although microcephalic, there is a superim­ posed hydrocephalus secondary to aque­ ductal stenosis. Fig. 4. CMV infection in a 10-month-old infant with migration anomalies, microce­ phaly and spastic quadriplegia. CT reveals peri ventricular calcifications on the right and pachygyria of the frontal lobes, more easily seen on the left. 4 AJNR: 13, March/April 1992 553 Fig. 7. AIDS; T2-weighted MR (2800/80/.75) in a 20-month­ old infant with developmental delay. Much of the subcortical Fig. 5. AIDS; 15-month-old child with AIDS and delayed de­ white matter still has a higher signal than gray matter, indicating velopment; noncontrast CT. Typical calcifications are seen in the delayed myelination, particularly in the frontal lobes. The overall lenticular nuclei, the heads of the caudata nuclei, and more white matter volume is thought to be mildly decreased. peripheral white matter in the frontal lobes. Generalized atrophy is also present. Fig. 6. AIDS in a 3-year-old child. A, Noncontrast CT shows dense calcifi­ cations in the basal ganglia and smaller but also dense calcifications in the frontal white matter. There is generalized atrophy. 8 , Three weeks later, during a preterminal bout of sepsis without identifiable brain in­ fection, CT shows striking further loss of brain tissue. The calcifications are un­ changed. imaging for CMV infection is 2.5 times more suggesting a propensity to affect the germinal common than for toxoplasmosis. Like toxoplas­ matrix. Calcification is also seen in the basal mosis, CMV causes destructive changes in the ganglia and other areas of the brain, so the pattern brain that lead to calcification and microcephaly. of calcification alone is not diagnostic. CMV has a higher predilection for periventricular Additional findings reported with CM V include: calcification (Fig. 3) that may be very thick in cerebellar hypoplasia (7), small subependymal severe infections. Migration anomalies are some­ cysts in the occipital horns that may be a fairly times associated with CMV infection (Fig. 4), specific finding on MR (8), and increased echo- 554 AJNR : 13, March/ April 1992 Fig. 8. Neonatal meningitis; 5-week-old infant with 1 week history of group B strep­ tococcal m eningitis. A, Contrast-enhanced CT shows en­ hancem ent along the path of the middle cerebral artery (arro w heads), and the edges of the temporal lobes (arro ws). B, More superi orl y, CT shows hydroceph­ alus and a right frontal infarction thought to be secondary to venous thrombosis. A 8 Fig. 9. Group B streptococcal meningitis; 6-week-old infant. A , Noncontrast CT shows a hem orrhagic right occipital lobe infarct. B, Contrast-enhanced CT shows faint en­ hancem ent of the ventricular wall (arro ws), indica ting ventriculitis. A 8 genicity of the thalamo-striate arteries causing a and the simpler examination to perform initially. candelabra-like pattern on ultrasound (9). How­ MR may be needed if a migration anomaly is ever, Ben Ami et al (10) found the same cande­ suspected but not clearly seen. labra-like pattern in a variety of congenital and Clinically, infants infected with CMV are often neonatal infections. Tassin et al diagnosed CMV premature with hepatosplenomegaly, jaundice, infection in the second trimester by using ultra­ thrombocytopenia, and chorioretinitis. Sensori­ sound to detect periventricular calcifications (11 ). neural hearing loss is common (4). Perinatal in­ As calcifications are far less likely to be visible fection is relatively common, but is rarely symp­ on MR than CT, CT is probably the more useful tomatic. AJNR: 13, March/ April 1992 555 Rubella Because of maternal screening and immuniza­ tion, congenital rubella has become a rarity in the developed world. Radiologic findings are similar to other viral infections. Microcephaly and brain calcification, especially basal ganglial and cortical calcification, are seen. Low density of the deep white matter has been described on CT (12). The first trimester infection is the most severe. The eyes are especially affected with cataracts, glau­ coma, and chorioretinitis all occurring. Deafness is also common. Cardiac anomalies are also seen (13). Herpes Herpes simplex is far more often transmitted at birth than in utero. Reports of radiologic find­ ings are rare. As with other placentally transmit­ ted infections, herpes infection commonly causes microcephaly. Periventricular calcifications with ventricular enlargement and cortical calcifications are usually present (14, 15) but exhibit no distin­ guishing features. Retinal dysplasia is common. Herpes zoster (chicken pox) is an uncommon intrauterine infection because of frequent child­ B hood exposure and immunization. No reports of CT findings are published, but one would expect abnormalities similar to the previously described infections. Microcephaly, cataracts, chorioretini­ tis, cortical atrophy, and limb atrophy are com­ mon clinical abnormalities (16). Others Human parvovirus B-19 has recently been dis­ covered to cause a variety of conditions including fetal hydrops and death (17). Its prevalence and severity are poorly studied, but perhaps it is an important "Other" in the TORCH spectrum. AIDS Seventy-eight percent of childhood HIV infec­