Nathan Watemberg

BACKGROUND AND PURPOSE: Congenital cytomegalovirus (CMV) infection can cause a wide range of brain anomalies. These changes have been well described postnatally, but descriptions of their in utero evolution are scarce. The purpose of this study was to analyze the sonographic spectrum of intracranial abnormalities in fetuses with proved CMV infection and to determine characteristic patterns of this infection. METHODS: We reviewed the transabdominal and transvaginal sonograms of eight fetuses with proved CMV infection. The sonographic analysis searched for signs of ventriculitis, leukomalacia, calcification, vasculitis, and periventricular cyst formation. The gyral pattern, corpus callosum, and cerebellar morphology also were examined. Fetal MR imaging was performed in two cases. RESULTS: The sonographic diagnosis of suspected CMV infection was made at a mean gestational age of 27.5 weeks (range, 22-37 weeks). An abnormal pattern of periventricular echogenicity was found in all fetuses. Echogenic intraparenchymal foci and ventriculomegaly were present in five fetuses. Other signs of fetal infection were intraventricular adhesions, periventricular pseudocysts, sulcation and gyral abnormal patterns, hypoplastic corpus callosum, cerebellar and cisterna magna abnormalities, and signs of striatal artery vasculopathy. For all fetuses, transvaginal sonography provided additional information. Fetal MR imaging provided additional information in one case. CONCLUSION: The presence of the described sonographic findings, particularly if two or more are present in the same fetus, is an indication for CMV investigation.

Although physical therapy (PT) is effective in improving motor function in children with developmental coordination disorder (DCD), insufficient data are available on the impact of this intervention in children with combined attention-deficit-hyperactivity disorder (ADHD) and DCD. This prospective study aimed to establish the prevalence of DCD among a cohort of patients with ADHD, characterize the motor impairment, identify additional comorbidities, and determine the role of PT intervention on these patients. DCD was detected in 55.2% of 96 consecutive children with ADHD (81 males, 15 females), mostly among patients with the inattentive type (64.3% compared with 11% of those with the hyperactive/impulsive type, p<0.05). Mean age was 8 years 4 months (SD 2 y). Individuals with both ADHD and DCD more often had specific learning disabilities (p=0.05) and expressive language deficits (p=0.03) than children with ADHD only. Twenty-eight patients with ADHD and DCD randomly received either intensive group PT (group A, mean age 9 y 3 mo, SD 2 y 3 mo) or no intervention (group B, mean age 9 y 3 mo, SD 2 y 2 mo). PT significantly improved motor performance (assessed by the Movement Assessment Battery for Children; p=0.001). In conclusion, DCD is common in children with ADHD, particularly of the inattentive type. Patients with both ADHD and DCD are more likely to exhibit specific learning disabilities and phonological (pronunciation) deficits. Intensive PT intervention has a marked impact on the motor performance of these children.

PURPOSE: Benign childhood epilepsy with centrotemporal spikes (BCECTS) is the most common epileptic syndrome in childhood. The outcome is usually excellent, but there are some atypical forms of BCECTS with less favorable outcomes. The aim of this study was to delineate the frequency of these atypical features among patients with BCECTS. METHODS: We conducted a retrospective chart study by retrieving the medical records of all consecutive patients with BCECTS who were evaluated in four pediatric neurology outpatient clinics in Israel between the years 1991 and 2008. KEY FINDINGS: A total of 196 patients with BCECTS were identified (78 female and 118 male; mean age at time of diagnosis 7.64 years, range 1.5-14). The mean duration of follow-up was 4.43 years (range 2-11). Nine patients (4.6%) developed electrical status epilepticus in slow waves sleep (ESES) during follow-up, four (2%) had Landau-Kleffner syndrome, three (1.5%) had BCECTS with frequent refractory seizures, two (1%) had BCECTS with falls at presentation, one (0.5%) had a "classic" atypical variant, and one (0.5%) had oromotor dysfunction. None had rolandic status epilepticus. Sixty-one patients (31%) had attention deficit hyperactivity disorder (ADHD), 43 (21.9%) had specific cognitive deficits, and 23 (11.7%) had behavioral abnormalities, including aggressiveness, anxiety disorders, depression, and pervasive developmental disorder (PDD). SIGNIFICANCE: The prevalence of most atypical forms of BCECTS other than ESES is low. There is, however, a high prevalence of ADHD and specific cognitive deficits among patients with BCECTS.

OBJECTIVE: To report the prenatal diagnosis and management of 34 fetuses with various intracranial structural pathologies diagnosed following a normal second-trimester ultrasound examination. METHODS: We retrospectively reviewed the images of 203 abnormal central nervous system ultrasound examinations performed between 13 and 37 weeks of gestation at our prenatal diagnosis unit. In 34 (16.7%) of them at least one previous second-trimester ultrasound examination had been performed and considered normal. These 34 fetuses represent the study group. RESULTS: The following intracranial pathologies were diagnosed: dysgenesis of the corpus callosum, ventriculomegaly, cerebral cysts or hemorrhage, migrational disorders, vermian dysgenesis, arachnoid cysts, macrocephaly, enlarged subarachnoid space, brain calcifications and microcephaly. CONCLUSION: A normal second-trimester ultrasound scan does not rule out significant intracranial anomalies. Parents and physicians should be informed about the limitations of second-trimester sonography as far as brain diagnosis is concerned. A repeat third-trimester scan may enable more accurate diagnosis and counseling.

OBJECTIVE: To determine the normal appearance and development of the fetal cerebellar vermis using mid-sagittal planes obtained by transvaginal sonography. SUBJECTS AND METHODS: A total of 101 fetuses were evaluated by transvaginal sonography between 21 and 39 weeks of gestation. The mid-sagittal antero-posterior and cranio-caudal vermian diameters, circumference and surface area were measured and calculated. Nomograms were produced. RESULTS: The vermis was observed in 96% of the cases and all the measurements were performed in 92%. The vermis grows in a linear fashion throughout pregnancy. The growth pattern correlates well with gestational age, biparietal diameter, head circumference and transverse cerebellar diameter. CONCLUSION: Transvaginal sonography is a valuable tool in the study of the fetal vermis during the second half of pregnancy. Knowledge of its normal appearance may help identify developmental anomalies and enable accurate prenatal counseling.