Acute and Chronic Kernicterus: MR Imaging Evolution of Globus Pallidus Signal Change during Childhood.

BACKGROUND AND PURPOSE: Despite its rarity in Western countries, kernicterus resulting from severe neonatal hyperbilirubinemia and its associated neurologic consequences still persists. Subtle MR imaging patterns may be overlooked, leading to diagnostic and prognostic uncertainties. The study systematically analyzes MR imaging pattern over time. MATERIALS AND METHODS: A retrospective MR imaging study was conducted in Departments of Pediatric Neurology at the University Children's Hospitals in Leipzig, Germany, or Tübingen, Germany, between 2012 and 2022 in patients who presented beyond the neonatal period suspected of having chronic kernicterus. RESULTS: Eight patients with a total of 15 MR images were identified. The clinical diagnosis of kernicterus was confirmed in all cases on the basis of typical MR imaging findings: Bilateral, diffuse hyperintensity of the globus pallidus was observed in the neonatal period on T1WI (1 MR imaging, at 2 weeks), in infancy on T2WI (4 MR images, at 9-26 months). In children 2 years of age and older, bilateral hyperintensity on T2WI was limited to the borders of the globus pallidus (8 MR images, at 20 months -13 years). Notably, 2 children exhibited normal initial MR imaging findings at 2 months of age. Hence, MR imaging depiction of kernicterus pathology evolves with time, first evident on T1WI, subsequently on T2WI, with a "blind window" during early infancy. The T2WI signal change initially involves the entire globus pallidus and later is limited to the borders. Kernicterus had not been diagnosed in any except 2 patients by previous investigators. CONCLUSIONS: All patients presented with a characteristic clinical history and signs and an evolving MR imaging pattern. Nonetheless, the diagnosis of kernicterus was frequently missed. Abnormalities on later MR images appear to be underrecognized.

Responses of antioxidant enzymes to cold and high light are not correlated to freezing tolerance in natural accessions of Arabidopsis thaliana.

Low temperatures and high light cause imbalances in primary and secondary reactions of photosynthesis, and thus can result in oxidative stress. Plants employ a range of low-molecular weight antioxidants and antioxidant enzymes to prevent oxidative damage, and antioxidant defence is considered an important component of stress tolerance. To figure out whether oxidative stress and antioxidant defence are key factors defining the different cold acclimation capacities of natural accessions of the model plant Arabidopsis thaliana, we investigated hydrogen peroxide (H2 O2 ) production, antioxidant enzyme activity and lipid peroxidation during a time course of cold treatment and exposure to high light in four differentially cold-tolerant natural accessions of Arabidopsis (C24, Nd, Rsch, Te) that span the European distribution range of the species. All accessions except Rsch (from Russia) had elevated H2 O2 in the cold, indicating that production of reactive oxygen species is part of the cold response in Arabidopsis. Glutathione reductase activity increased in all but Rsch, while ascorbate peroxidase and superoxide dismutase were unchanged and catalase decreased in all but Rsch. Under high light, the Scandinavian accession Te had elevated levels of H2 O2 . Te appeared most sensitive to oxidative stress, having higher malondialdehyde (MDA) levels in the cold and under high light, while only high light caused elevated MDA in the other accessions. Although the most freezing-tolerant, Te had the highest sensitivity to oxidative stress. No correlation was found between freezing tolerance and activity of antioxidant enzymes in the four accessions investigated, arguing against a key role for antioxidant defence in the differential cold acclimation capacities of Arabidopsis accessions.

[MR imaging in the diagnosis of primary and secondary cerebral infections]. / Wertigkeit der MRT in der Diagnostik primärer und sekundärer zerebraler Infektionen.

Cerebral infections may develop into a life-threatening condition. Fast and correct diagnosis is crucial for a differentiated therapy and MRI imaging is widely accepted as the method of choice. Both specific MR sequences and imaging characteristics of major cerebral infections are addressed in this overview. Furthermore, limitations and pitfalls of the method are discussed.

Juvenile metachromatic leukodystrophy 10 years post transplant compared with a non-transplanted cohort.

Metachromatic leukodystrophy (MLD) is a rare inborn error of metabolism leading to severe neurological symptoms and early death. Hematopoietic SCT (HSCT) is considered a treatment option, but results are inconsistent and comparison with natural history is practically missing. We compare a girl with juvenile MLD 10 years after allogeneic HSCT not only with her untreated sister, but also with a large cohort of untreated patients. The girl received HSCT at the age of 5 years when first motor signs appeared. Over 10 years she was stable with respect to her clinical course and gained cognitive abilities. Magnetic resonance imaging (MRI) showed clear regression of white matter changes and magnetic resonance spectroscopy (MRS) demonstrated a reversal of the initial choline increase and N-acetyl-aspartate (NAA) decrease. Only axonal demyelinating neuropathy showed some progression. Her gross motor function and MRI-scores were clearly better compared with her sister and the cohort of untreated patients. Difference to her sister became apparent only 4 years after HSCT. We conclude that HSCT, early in the course of disease, can lead to stabilization of juvenile MLD with a course clearly different from the natural history. HSCT may prevent disease progression, if performed sufficient time before loss of walking, which typically initiates rapid deterioration.

Common and uncommon imaging findings in progressive multifocal leukoencephalopathy (PML) with differential diagnostic considerations.

OBJECTIVE: To provide a practical review of the spectrum of possible imaging findings in patients with progressive multifocal leukoencephalopathy (PML) and to address differentials. CONCLUSION: PML manifests with a broad spectrum of imaging features. Besides knowledge of preferential location, extent, temporal course, enhancement, results of functional imaging and clinical setting, recognition of imaging findings reflecting active demyelination may help the clinician in appropriately narrowing down the differential diagnosis.

T2 and DWI in pilocytic and pilomyxoid astrocytoma with pathologic correlation.

OBJECTIVE: To quantify and compare T2 signal and apparent diffusion coefficient (ADC) in pilocytic and pilomyxoid astrocytoma (PA and PMA) and correlate results with myxoid content. METHODS: Echo-planar diffusion weighted images (DWI) and standard magnetic resonance imaging (MRI) findings were reviewed retrospectively in patients with PA (n=34) and PMA (n=8). Regions of interest (ROIs) were drawn on ADC maps within tumor parts with lowest ADC values. Apparent diffusion coefficient values in tumor were normalized to those in cerebrospinal fluid (ADC/CSF). The ratio of T2 signal intensity in solid tumor parts to CSF (T2/CSF) was registered. Myxoid matrix was histologically quantified retrospectively in 8 PMAs and 17 PAs and correlated with imaging findings. RESULTS: Mean ADC/CSF for PA and PMA was 0.53±0.10 and 0.69±0.10 (p<0.01). Mean T2/CSF for PA and PMA was 0.78±0.19 and 0.93±0.09 (p<0.01). Mean proportion of myxoid tumor matrix in PA was 50% (range, 10-100%) and 93% (range, 90-100%) in PMA (p=0.004). Eight patients (32%; all PA) had less than 50% myxoid content and 17 (68%; 8 PA; 9 PMA) had more. There was positive correlation of ADC/CSF, T2/CSF and ADC (r2=0.61, 0.65 and 0.60 respectively) and significant difference between the groups with more and less than 50% myxoid content (p=0.01 for ADC/CSF and T2/CSF and p=0.02 for ADC). CONCLUSIONS: General imaging features of PA and PMA are non-specific, ADC values and T2 signal intensity are generally higher in the latter, reflecting the proportion of myxoid matrix in these tumors.

Optimized 3D magnetization-prepared rapid acquisition of gradient echo: identification of thalamus substructures at 3T.

BACKGROUND AND PURPOSE: Because the substructures of the thalamus are not visible on standard T1- and T2-weighted MR images, planning of deep brain stimulation implantation relies on stereotactic atlas coordinates. The goal of the present work was to test whether an optimized 3D MPRAGE protocol can depict thalamus substructures. MATERIALS AND METHODS: After optimization of the TI to maximize contrast between gray matter and white matter, 6 healthy subjects were scanned at 3T with the optimized 3D MPRAGE. The results were compared with stereotactic atlases, and 2 expert readers trained in thalamic anatomy identified the 4 large thalamic nuclei groups. RESULTS: There was a high agreement between the different atlases and the resulting MR images. The 4 large thalamic nuclei groups (anterior, lateral, medial, posterior) could be detected reliably. The inter-reader consistency on the size and location was 75%-92%. CONCLUSIONS: The optimized 3D MPRAGE protocol improves contrast in the thalamus, and the 4 large thalamic nuclei groups can be identified with high inter-reader agreement.

Dual energy CTA of the carotid bifurcation: advantage of plaque subtraction for assessment of grade of the stenosis and morphology.

OBJECTIVES: Calcifications adjacent to the vessel lumen often limit the assessment of stenoses at the carotid bifurcation in 3D multi intensity projection images (3D-MIP) using conventional single energy CT. Aim of the study was to evaluate the diagnostic value of 3D-MIP images after subtraction of bone and calcified plaques (PBS) using dual energy CT for the assessment of carotid bifurcation stenoses. MATERIALS AND METHODS: 36 patients with a total of 46 stenoses at the carotid bifurcation were examined with a dual energy CT system. Grade of the stenoses and plaque morphology were assessed in axial multi planar projections (axMPR) and freely rotatable 3D-MIP images before and after PBS and compared with results from DSA. RESULTS: Grade of the stenosis could be evaluated in all 46 cases in DSA, axMPR and 3D-MIP after PBS. However, in 25 cases grade of the stenosis was not assessable prior to PBS. The average grade of the stenosis increased from DSA (81.4%) to axMPR (83.5%) to 3D-MIP before and after PBS (86.5% and 85.6%). The amount of pseudo-occlusions increased in concordance with the grade of the stenosis (0<9<16). Using 3D-MIP reconstructions, plaque morphology could be evaluated in 32/46 stenoses before PBS and in 44/46 cases after PBS. CONCLUSIONS: PBS facilitated the evaluation of grade of the stenosis in all cases. Nevertheless, after PBS stenoses were overrated in 3D-MIP in comparison to DSA and axMPR. Moreover, plaque morphology, as an independent risk factor for stroke, can be evaluated even in calcified plaques after PBS. Therefore dual energy CTA with plaque subtraction has the potential to identify patients with vulnerable plaques better than conventional CTA.

Spinal pilocytic astrocytoma: MR imaging findings at first presentation and following surgery.

OBJECTIVE: The objective of this article is to describe MR imaging findings of spinal cord pilocytic astrocytomas at first presentation and following neurosurgery and to discuss briefly some of the most common differential diagnoses. CONCLUSION: MR imaging findings in medullary pilocytic astrocytomas consist generally of focal or diffuse cord-enlarging masses that are irregularly shaped, accompanied by cystic elements and hydromyelia, present different degrees of contrast enhancement, high water diffusivity and a propensity for the thoracic and cervical cord.