Assessment of myelination in infants and young children by T1 relaxation time measurements using the magnetization-prepared 2 rapid acquisition gradient echoes sequence

Abstract Background Axonal myelination is an important maturation process in the developing brain. Increasing myelin content correlates with the longitudinal relaxation rate (R1=1/T1) in magnetic resonance imaging (MRI). Objective By using magnetization-prepared 2 rapid acquisition gradient echoes (MP2RAGE) on a 3-T MRI system, we provide R1 values and myelination rates for infants and young children. Materials and methods Average R1 values in white and grey matter regions in 94 children without pathological MRI findings (age range: 3 months to 6 years) were measured and fitted by a saturating-exponential growth model. For comparison, R1 values of 36 children with different brain pathologies are presented. The findings were related to a qualitative evaluation using T2, magnetization-prepared rapid acquisition gradient echo (MP-RAGE) and MP2RAGE. Results R1 changes rapidly in the first 16 months of life, then much slower thereafter. R1 is highest in pre-myelinated structures in the youngest subjects, such as the posterior limb of the internal capsule (0.74–0.76±0.04 s−1) and lowest for the corpus callosum (0.37–0.44±0.03 s−1). The myelination rate is fastest in the corpus callosum and slowest in the deep grey matter. R1 is decreased in hypo- and dysmyelination disorders. Myelin maturation is clearly visible on MP2RAGE, especially in the first year of life. Conclusion MP2RAGE permits a quantitative R1 mapping method with an examination time of approximately 6 min. The age-dependent R1 values for children without MRI-identified brain pathologies are well described by a saturating-exponential function with time constants depending on the investigated brain region. This model can serve as a reference for this age group and to search for indications of subtle pathologies. Moreover, the MP2RAGE sequence can also be used for the qualitative assessment of myelinated structures.

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Adult-onset Krabbe disease with homozygous T1853C mutation in the galactocerebrosidase gene

Neurology ◽

10.1212/wnl.49.5.1392 ◽

1997 ◽

Vol 49 (5) ◽

pp. 1392-1399 ◽

Cited By ~ 46

Author(s):

J.-I. Satoh ◽

H. Tokumoto ◽

K. Kurohara ◽

M. Yukitake ◽

M. Matsui ◽

...

Keyword(s):

White Matter ◽

Late Onset ◽

Spastic Paraparesis ◽

Internal Capsule ◽

Krabbe Disease ◽

High Signal ◽

Periventricular White Matter ◽

Mri Findings ◽

Posterior Limb ◽

Intellectual Capacity

A 51-year-old woman developed a slowly progressive spastic paraparesis and diminished vibration sense beginning at age 38. Intellectual capacity was normal. Krabbe disease was confirmed by markedly reduced leukocyte galactocerebrosidase (GALC) activity, typical inclusions in Schwann cell cytoplasm, and an identification of the homozygous point mutation T1835C(Leu618Ser) in the GALC gene. T2-weighted MRI of the brain showed symmetric high-signal-intensity lesions in the bilateral frontoparietal white matter, the centrum semiovale, and the posterior limb of the internal capsule with sparing of the periventricular white matter. This case is unusual because of the late onset, protracted clinical course, and MRI findings of demyelination confined to the corticospinal tracts.

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A Rare Cause of Fever of Unknown Origin: Reverse Shapiro’s Syndrome

Journal of Preventive Medicine And Care ◽

10.14302/issn.2474-3585.jpmc-19-2655 ◽

2019 ◽

Vol 2 (4) ◽

pp. 1-6

Author(s):

Huseyin Dag ◽

Yelda Turkmenoglu ◽

Tugce Aksu Uzunhan ◽

Edebali Erdogan ◽

Ozge Yapıcı Ugurlar ◽

...

Keyword(s):

Corpus Callosum ◽

Fever Of Unknown Origin ◽

Internal Capsule ◽

Unknown Origin ◽

Posterior Limb ◽

Methyl Prednisolone ◽

Cranial Mri ◽

Magnetic Resonance Imaging Mri ◽

Rate Of Response

Reverse Shapiro’s syndrome is described as unexplained hyperthermia coexisting with agenesis of the corpus callosum. Its pathophysiology dwells on the role of dopaminergic hypersensitivity caused by hypothalamic dysfunction. Until now, only 5 cases have been described in the literature as reverse Shapiro’s syndrome. We present a case of a 6-month-old girl who is now the sixth patient described in the literature. A 6-month-old female patient was admitted to the pediatrics unit for fever of unknown origin. Her fever occurred 2-3 times a day on average between 38°C and 39.5°C, and lasted for 1-2 hours. The fever was not diurnal, and antipyretics or staying in an air-conditioned room had no effect. She also had 2 convulsions during her hospital stay. Cranial magnetic resonance imaging (MRI) was requested owing to the patient’s convulsion history and retarded development. The cranial MRI showed diffuse hypoplasia of the corpus callosum in the midline sagittal T2-weighted image. T1-weighted imaging showed hypointensity due to delayed myelination of the genu of the corpus callosum (Figure 2, white arrow), which should normally appear hyperintense like the posterior limb of the internal capsule. Although dopamine agonists and serotonin agonists are recommended for the treatment, the rate of response to medical treatment is very low. Our patient did not benefit from cyproheptadine and methyl prednisolone.

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HOW ARE CARDIAC FUNCTIONS ALTERED IN PEDIATRIC PATIENTS RECEIVING ORAL IRON SUPPLEMENTATION DUE TO ANEMIA?

Journal of Scientific Perspectives ◽

10.26900/jsp.5.1.07 ◽

2021 ◽

pp. 81-92

Author(s):

Merve HİÇYILMAZ ◽

Ayhan PEKTAŞ ◽

Selçuk GÜREL ◽

Mehmet Bilgehan PEKTAŞ

Keyword(s):

White Matter ◽

Corpus Callosum ◽

Structural Difference ◽

Internal Capsule ◽

Control Group ◽

Posterior Limb ◽

Brain Functions ◽

Significant Difference ◽

Strongly Connected ◽

The Right

Migraine is a multifactorial neurovascular syndrome characterized by typical headache attacks that occur with internal and external triggering factors in individuals with genetic susceptibility. It affects more than 12% of the general population. The aim of the present study is to compare the morphometric measurements of the determined white matter structures with the control group to investigate whether there is a structural difference in white matter structures in female patients with migraine. The width of the internal capsule parts (anterior limb, posterior limb and genu) and genu angle was evaluated through MRI. Corpus callosum related measurements were determined in the sagittal section. It was manually traced following its edge on the midsagittal slice of T1 images, where its structure appeared most remarkable. The right and left internal capsule related measurements were compared with migraine and control groups. Except the genu angle, there were statistically significant difference between all measurements and widths in the migraine group were greater than controls. No significant difference was found between the corpus callosum related measurements in the comparison of both groups. Internal capsule consists of several essential white matter fiber bundles of the brain, and is strongly connected between a range of cortical and subcortical anatomical structures. It has a crucial importance for brain functions and it can be affected by a variety of pathologies. The corpus callosum is the main fiber tract connecting two hemispheres with extensive connections and is topographically organized. It has been investigated in several neurodegenerative diseases as a marker for cortical pathology. Knowing the white matter structure in migraine patients, determining its prevalence, and its correlation with the severity, type and duration of migraine can give an idea to clinicians.

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Diffusiontensor MRI tractography in patients with children’s cerebral palsy: assessment of structural lesions of conduction tracts

Российский педиатрический журнал ◽

10.18821/1560-9561-2016-19-2-68-74 ◽

2019 ◽

Vol 19 (2) ◽

pp. 68-74

Author(s):

Yulia V. Ermolina ◽

L. S. Namazova-Baranova ◽

A. M. Mamedyarov ◽

A. V. Anikin ◽

O. I. Maslova

Keyword(s):

Cerebral Palsy ◽

Corpus Callosum ◽

Diffusion Tensor ◽

Mean Diffusivity ◽

Internal Capsule ◽

Neurological Deficits ◽

Motor Deficit ◽

Diffusivity Coefficient ◽

Posterior Limb ◽

The Brain

Introduction. The application of diffusion-tensor (DT)-MRI tractography in patients with children S cerebral palsy (CP) allows to establish pathophysiological mechanisms of the development of motor and sensory impairments, to perform a clinical and neuroimaging comparison, as well as to determine correlations between lesions of the conduction tracts and neurological deficits. Aim. For the determination of changes in fractional anisotropy (FA) and mean diffusivity coefficient (MDC), obtained with DT-MRI in the corticospinal and sensory tracts, structures of the corpus callosum in spastic forms of CP. Materials and methods. There were examined 105 children with spastic forms of CP (spastic tetraparesis - 47, spastic diplegia - 38, hemiparetic form - 20) and 50 children without neurological deficit, at the age of 2-18 years. In all CP patients the severity of motor deficit was evaluated according to Gross Motor Function Classification System (GMFCS). DT-MRI was performed in all cases with the use of devices of General Electric (US), under magnetic field strength of 1.5T and 3T, with the calculation of the FA and the MDC in areas of interest - cerebral peduncles the brain stem, the posterior limb of the internal capsule, the genu and splenium of the corpus callosum, posterior thalamic radiation, postcentral gyrus, the thalamus. Results. There was established the distribution of children according to the GMFCS scale: I - 14 (13,4%), II - 19 (18,1%), III - 20 (19%), IV- 10 (9,5%), V- 42 (40%) cases. The severity of motor impairment on the GMFCS scale closely correlated with the values of FA and ADC in cerebral peduncles, posterior limb of the internal capsule, posterior thalamic radiance, genu of the corpus callosum. Conclusion. DT-MRI enables to assess adequately the changes in the white matter of the brain, structural andfunctional state of the motor and sensory conduction tracts, gives a possibility to predict the development of a child with CP.

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Megalencephalic leukoencephalopathy with subcortical cysts

Neurology ◽

10.1212/wnl.0000000000005334 ◽

2018 ◽

Vol 90 (16) ◽

pp. e1395-e1403 ◽

Cited By ~ 16

Author(s):

Eline M.C. Hamilton ◽

Pinar Tekturk ◽

Fia Cialdella ◽

Diane F. van Rappard ◽

Nicole I. Wolf ◽

...

Keyword(s):

White Matter ◽

Internal Capsule ◽

Disease Stage ◽

Mri Findings ◽

Cerebellar White Matter ◽

Cross Sectional ◽

Posterior Limb ◽

Mri Features ◽

Mri Characteristics ◽

Subcortical Cysts

ObjectiveTo provide an overview of clinical and MRI characteristics of the different variants of the leukodystrophy megalencephalic leukoencephalopathy with subcortical cysts (MLC) and identify possible differentiating features.MethodsWe performed an international multi-institutional, cross-sectional observational study of the clinical and MRI characteristics in patients with genetically confirmed MLC. Clinical information was obtained by questionnaires for physicians and retrospective chart review.ResultsWe included 204 patients with classic MLC, 187 of whom had recessive mutations in MLC1 (MLC1 variant) and 17 in GLIALCAM (MLC2A variant) and 38 patients with remitting MLC caused by dominant GLIALCAM mutations (MLC2B variant). We observed a relatively wide variability in neurologic disability among patients with classic MLC. No clinical differences could be identified between patients with MLC1 and MLC2A. Patients with MLC2B invariably had a milder phenotype with preservation of motor function, while intellectual disability and autism were relatively frequent. Systematic MRI review revealed no MRI features that distinguish between MLC1 and MLC2A. Radiologic improvement was observed in all patients with MLC2B and also in 2 patients with MLC1. In MRIs obtained in the early disease stage, absence of signal abnormalities of the posterior limb of the internal capsule and cerebellar white matter and presence of only rarefied subcortical white matter instead of true subcortical cysts were suggestive of MLC2B.ConclusionClinical and MRI features did not distinguish between classic MLC with MLC1 or GLIALCAM mutations. Absence of signal abnormalities of the internal capsule and cerebellar white matter are MRI findings that point to the remitting phenotype.

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CARD8 and IL1B Polymorphisms Influence MRI Brain Patterns in Newborns with Hypoxic-Ischemic Encephalopathy Treated with Hypothermia

Antioxidants ◽

10.3390/antiox10010096 ◽

2021 ◽

Vol 10 (1) ◽

pp. 96

Author(s):

Katarina Esih ◽

Katja Goričar ◽

Zvonka Rener-Primec ◽

Vita Dolžan ◽

Aneta Soltirovska-Šalamon

Keyword(s):

Oxidative Stress ◽

Brain Injury ◽

Genetic Variability ◽

Internal Capsule ◽

Nucleotide Polymorphisms ◽

Mri Findings ◽

Posterior Limb ◽

Buccal Swabs ◽

Magnetic Resonance Imaging Mri ◽

The Impact

Inflammation and oxidative stress are recognized as important contributors of brain injury in newborns due to a perinatal hypoxic-ischemic (HI) insult. Genetic variability in these pathways could influence the response to HI and the outcome of brain injury. The aim of our study was to evaluate the impact of common single-nucleotide polymorphisms in the genes involved in inflammation and response to oxidative stress on brain injury in newborns after perinatal HI insult based on the severity and pattern of magnetic resonance imaging (MRI) findings. The DNA of 44 subjects was isolated from buccal swabs. Genotyping was performed for NLRP3 rs35829419, CARD8 rs2043211, IL1B rs16944, IL1B rs1143623, IL1B rs1071676, TNF rs1800629, CAT rs1001179, SOD2 rs4880, and GPX1 rs1050450. Polymorphism in CARD8 was found to be protective against HI brain injury detected by MRI overall findings. Polymorphisms in IL1B were associated with posterior limb of internal capsule, basal ganglia, and white matter brain patterns determined by MRI. Our results suggest a possible association between genetic variability in inflammation- and antioxidant-related pathways and the severity of brain injury after HI insult in newborns.

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