Fetal MR imaging.

Ultrasonography is the primary prenatal screening modality used in the evaluation of the fetus and the maternal pelvis. However, fetal MR imaging plays a complementary role to prenatal ultrasound in the evaluation of the fetus with suspected abnormalities. MR imaging's role includes confirming or excluding possible lesions, defining their full extent, aiding in their characterization, and demonstrating other associated abnormalities. As newer techniques such as diffusion imaging, MR spectroscopy, and functional studies are used more widely, it is hoped that additional information will be made available by this modality to physicians evaluating and taking care of fetuses.

Paralinguistic processing in children with callosal agenesis: emergence of neurolinguistic deficits.

Recent research revealed impaired processing of both nonliteral meaning and affective prosody in adults with agenesis of the corpus callosum (ACC) and normal intelligence. Since normal children have incomplete myelination of the corpus callosum, it was hypothesized that paralanguage deficits in children with ACC would be less apparent relative to their peers. The Familiar and Novel Language Comprehension Test (FANL-C) and Prosody Test were given to 18 children with ACC and normal intelligence and 17 controls matched for age (7-13 years), education, and IQ (83-122). When controlling for age, children with ACC were significantly poorer in comprehension of the precise meaning of both literal and nonliteral items on the FANL-C. Adults with ACC had previously been shown to have difficulty only on nonliteral items. The effect size for nonliteral comprehension in children with ACC was smaller than that seen in adults. There was only a trend for the child ACC group to perform worse on the recognition of affective prosody. Thus, while deficits in paralinguistic processing were apparent, children with ACC were not as clearly different from age peers as adults, and were equally deficient at comprehending literal and nonliteral expressions. The differences in results between adults and children with ACC are thought to reflect incomplete callosal development in normal children, and the importance of the corpus callosum in the early stages of the development of the ability to process literal language.

Intracranial meningeal disease: comparison of contrast-enhanced MR imaging with fluid-attenuated inversion recovery and fat-suppressed T1-weighted sequences.

BACKGROUND AND PURPOSE: Contrast-enhanced fluid-attenuated inversion recovery (FLAIR) imaging has been reported to have higher sensitivity for detecting leptomeningeal disease compared with contrast-enhanced T1-weighted MR imaging. The purpose of this study was to compare contrast-enhanced T1-weighted MR images with fat suppression to contrast-enhanced FLAIR images to determine which sequence was superior for depicting meningeal disease. METHODS: We reviewed MR images of 24 patients (35 studies) with a variety of meningeal diseases. The MR imaging protocol included contrast-enhanced T1-weighted MR images with fat suppression (FS) and contrast-enhanced fluid-attenuated inversion recovery (FLAIR) images that were reviewed by three neuroradiologists and were assigned a rating of positive, equivocal, or negative for abnormal meningeal enhancement. The two sequences were compared side by side to determine which better depicted meningeal disease. RESULTS: Abnormal meningeal enhancement was positive in 35 contrast-enhanced T1-weighted MR images with FS and in 33 contrast-enhanced FLAIR studies. In the first group, which had the T1-weighted sequence acquired first (21 of 33 studies), contrast-enhanced T1-weighted images with FS showed superior contrast enhancement in 11 studies (52%), inferior contrast enhancement in six studies (29%), and equal contrast enhancement in four studies (19%) compared with the contrast-enhanced FLAIR images. In the second group, which had the FLAIR sequence acquired first (12 of 33), contrast-enhanced T1-weighted images with FS showed superior contrast enhancement in seven studies (58%), inferior contrast enhancement in two studies (17%), and equal contrast enhancement in three studies (25%). CONCLUSION: Contrast-enhanced T1-weighted MR imaging with FS is superior to contrast-enhanced FLAIR imaging in most cases for depicting intracranial meningeal diseases.

Comprehension of humor in primary agenesis of the corpus callosum.

Individuals with agenesis of the corpus callosum (ACC) can, in some cases, perform normally on standardized intelligence tests. Nevertheless, recent studies suggest that individuals with ACC and normal IQ scores have deficits in domains of fluid and social intelligence. Anecdotal reports from families suggest diminished appreciation of the subtleties of social interactions, and deficits in the comprehension of jokes and stories. In this research, both the cartoon and narrative joke subtests of a humor test (developed by Brownell et al. [Brownell, H., Michel, D., Powelson, J., & Gardner, H. (1983). Surprise but not coherence: sensitivity to verbal humor in right-hemisphere patients. Brain and language, 18(1), 20-27] and Bihrle et al. [Bihrle, A. M., Brownell, H. H., Powelson, J. A., & Gardner, H. (1986). Comprehension of humorous and non-humorous materials by left and right brain-damaged patients. Brain and Cognition, 5(4), 399-411]) were given to 16 adults with complete ACC (all with IQs>80) and 31 controls of similar age and IQ. Individuals with ACC performed worse than controls on the narrative joke subtest (p<.025) when VIQ was controlled. However, on the cartoon subtest the two groups were not significantly different. Covarying age, forms of IQ, narrative memory, set-switching, and literal language comprehension did not substantially alter the group difference. However, covarying comprehension of nonliteral language and proverbs eliminated the difference, suggesting a common origin for the comprehension of jokes, nonliteral language, and proverbs, most likely related to capacity for understanding second-order meanings.

Role of proton magnetic resonance spectroscopy in the diagnosis of gliomatosis cerebri: a unique pattern of normal choline but elevated Myo-inositol metabolite levels.

A patient with histologically proven gliomatosis cerebri presented with a normal choline level but a markedly abnormal elevated myo-inositol level on magnetic resonance (MR) spectroscopy. We describe the case presentation, imaging findings (in particular, the unique MR spectroscopic pattern), and their significance regarding the diagnosis of this relatively rare neoplasm.

Neuroimaging of mitochondrial disorders.

Mitochondrial disease is frequently a multisystem disorder which often involves the central nervous system. Imaging finding although diverse are characterized by focal lesions with T2 hyperintensity, which may be most evident on FLAIR imaging and often progress to atrophy. Deep brain structures including brainstem and basal ganglia structures are particularly vulnerable though white matter and cortex may also be involved. In this paper we describe in detail the imaging features of the spectrum of mitochondrial diseases and suggest a scoring technique for recording severity and extent of brain involvement. Although there is overlap between the imaging features of disease phenotypes, magnetic resonance imaging may be useful in supporting the clinical diagnosis. There is little correlation between molecular defect and imaging findings with some noticeable exceptions such as the MELAS syndrome.

Communicative deficits in agenesis of the corpus callosum: nonliteral language and affective prosody.

While some individuals with agenesis of the corpus callosum can perform normally on standardized intelligence tests, clinical observations suggest that they nevertheless have deficits in the domains of fluid and social intelligence. Particularly important for social competence is adequate understanding and use of paralinguistic information. This study examined the impact of callosal absence on the processing of pragmatic and paralinguistic information. Young adult males with agenesis of the corpus callosum (ACC) were evaluated in the areas of nonliteral language comprehension, proverb recognition and interpretation, and perception of affective prosody. Ten ACC individuals with normal Wechsler IQ were compared to 14 sex, age, and IQ matched normal controls. The Formulaic and Novel Language Comprehension Test (FANL-C), Gorham Proverbs Test, and LA Prosody Test were administered. ACC subjects exhibited significant impairment on the nonliteral items of the FANL-C, but no significant difference from controls in comprehension of literal items. ACC subjects also exhibited significant deficits in both self-generated interpretation and recognition of proverb meaning, and in recognition of affective prosody. These results demonstrate that normally intelligent individuals with ACC are impaired in the understanding of nonliteral language and emotional-prosodic cues that are important in social communication. In all three tests, the performance of individuals with ACC was similar to patients with right hemisphere brain damage. Thus, persons with ACC appear to lack interhemispheric integration of critical aspects of language processed by the right hemisphere.

Factor XIII deficiency mistaken for battered child syndrome: case of "correct" test ordering negated by a commonly accepted qualitative test with limited negative predictive value.

We report herein a case of Factor XIII deficiency that remained undiagnosed until 2 years of age. Part of the delay in diagnosis was a consequence of testing that was performed on a blood sample obtained after plasma transfusion therapy for a life-threatening bleeding episode. Due to insufficient family follow-up after discharge from the hospital, the diagnosis was delayed 1 year until the child was rehospitalized and a pre-transfusion plasma sample was tested. The commonly accepted approach of using only a qualitative test for the diagnosis of factor XIII deficiency is challenged by this case report.