Past research has suggested that white matter volume increases from childhood to adulthood; however, during adolescence, there is somewhat limited data to support this finding. In the present study, 65 typically developing adolescents underwent structural magnetic resonance imaging. Using magnetic resonance imaging, prefrontal white matter volumes were examined in relation ...

We performed a detailed neuroimaging study in a patient with Parry-Romberg syndrome. Proton MR spectroscopy demonstrated normal spectral patterns, though conventional MR imaging revealed high-intensity areas in the entire white matter in the left hemisphere. Single-photon emission tomography showed increased perfusion in the cortex of the affected hemisphere. Pyramidal tracts ...

Vanishing white matter disease (VWM; MIM #603896), also known as childhood ataxia with central nervous system hypomyelination (CACH) syndrome, is an autosomal recessive transmitted leukoencephalopathy related to mutations in each of the 5 genes (EIF2B1, EIF2B2, EIF2B3, EIF2B4 and EIF2B5) encoding for the 5 subunits of eukaryotic translation initiation factor ...

Hypoxic ischemic encephalopathy may cause early deep white matter abnormalities on MR imaging that usually progress to include gray matter and basal ganglia change. Toxic leukoencephalopathy due to heroin inhalation predominantly causes cerebellar and posterior cerebral radiologic change. Both conditions rarely present clinically and radiologically in a delayed manner with ...

To delineate the frequency, severity, and characteristics of the brain injury occurring in children surviving extremely premature birth, we reviewed brain magnetic resonance images (MRIs) of children with cerebral palsy whose birthweight was less than 1000 g and whose gestational age was less than 28 weeks. The patients were all ...

OBJECTIVE: The purpose of our study was to show how, despite pathognomonic signs of cerebral involvement in chronic progressive external ophthalmoplegia (CPEO), mitochondrial respiratory chain insufficiency is associated with increased lactate and reduced N-acetylaspartate. CPEO and mitochondrial myopathy are caused by mitochondrial DNA mutations leading to impaired oxidative phosphorylation. Cortical ...

BACKGROUND: Minor physical anomalies are more prevalent among people with psychosis. This supports a neurodevelopmental aetiology for psychotic disorders, since these anomalies and the brain are both ectodermally derived. However, little is understood about the brain regions implicated in this association. AIMS: To examine the relationship between minor physical anomalies ...

We report the serial magnetic resonance imaging (MRI) findings in a case of methanol intoxication. A 50-year-old man who had accidentally ingested methanol was admitted to our institution. The patient was almost blind, and became comatose. The MRI showed bilateral putaminal lesions with restricted diffusion. The MRI, which was performed ...

Two patients with multifocal pilocytic astrocytoma diagnosed by magnetic resonance imaging (MRI) and confirmed by histopathological examination are reported. They presented distinct sites and mechanisms of metastasis: to distant ventricles through the cerebral spinal fluid (CSF) in patient 1 and to contralateral parenchyma, possibly through white matter tracts, in patient ...

Numerous studies have shown gender differences in the brain volumes of elderly adults. Some evidence shows that higher estrogen levels may be neuroprotective, suggesting that hormone therapy (HT) may in part be responsible for these gender differences; however, few studies have examined the relation between HT and brain volumes. Brain ...

In this pediatric case of vanishing white matter disease with early onset, rapidly progressive course, and fatal outcome, the white matter vanishing process in patient was for the first time documented morphologically in detail: An initial magnetic resonance imaging documented a normal appearing brain maturation. Rapid progressive brain lesions initiated ...

The central nervous system undergoes profound and predictable developmental changes during the first few years of life that provide the structural and functional elements necessary for normal neurological development. The establishment and maturation of white matter pathways is a critical component of the developing nervous system. Diffusion tensor imaging (DTI) ...

3-Hydroxy-3-methylglutaryl coenzyme A lyase deficiency, an inborn error of ketone body synthesis and leucine degradation, is a rare disorder. There are few reports demonstrating clinical and neuroradiologic findings of this condition. This report describes a 3.5-year-old previously healthy male who was admitted with complex partial seizures and was diagnosed as ...

This review provides the reader with an overview of the magnetic resonance spectroscopy technique and the clinical, pathological, imaging, and metabolic features for select white matter disorders of interest. With this composite summary, the reader should find it easier to implement and interpret spectroscopy in the clinical setting for the ...

OBJECTIVES: The purpose of this article is to explore the magnetic resonance (MR) imaging features and the advanced MR techniques for differential diagnosis of white matter diseases of the brain. RESULTS: Magnetic resonance imaging is exquisitely sensitive for detecting brain abnormalities. Particularly in the evaluation of white matter diseases, MR ...

OBJECTIVE: The authors examined the ethical and practical management issues resulting from the detection of incidental abnormal findings on magnetic resonance imaging (MRI) research studies in healthy pediatric volunteers. METHOD: A retrospective examination of the findings from 60 clinical reports of research MRI scans from a cohort of healthy pediatric ...

Disseminated necrotizing leukoencephalopathy (DNL) is a potentially fatal complication of treatment involving intrathecal administration of chemotherapeutic agents such as methotrexate (MTX) alone or in combination with cranial radiotherapy (RT). We describe a case of acute lymphoblastic leukemia (ALL) treated with high-dose intravenous and intrathecal methotrexate combined with craniospinal RT resulting ...

To evaluate cerebral metabolism and intergroup differences in closely matched patients with myotonic dystrophy type 2 (DM2, n = 15) and type 1 (DM1, n = 14), we performed (1)H magnetic resonance spectroscopic (MRS) analyses of the occipital and temporoparietal cortical regions as well as of subcortical frontal white matter. ...

Even though previous neuropsychological studies and clinical case reports have suggested an association between pedophilia and frontocortical dysfunction, our knowledge about the neurobiological mechanisms underlying pedophilia is still fragmentary. Specifically, the brain morphology of such disorders has not yet been investigated using MR imaging techniques. Whole brain structural T1-weighted MR ...

OBJECTIVES: Gray and white matter volume deficits have been reported in a number of studies of children with attention-deficit/hyperactivity disorder (ADHD); however, there is a paucity of structural magnetic resonance imaging (MRI) studies of adults with ADHD. This structural MRI study used an a priori region of interest approach. METHODS: ...

MRI at high magnetic field strength potentially allows for an increase in resolution and image contrast. The gains are particularly dramatic for T(2)(*)-weighted imaging, which is sensitive to susceptibility effects caused by a variety of sources, including deoxyhemoglobin, iron concentration, and tissue microstructure. On the other hand, the acquisition of ...

Multifractal analysis based on generalized concepts of fractals has been applied to evaluate biological tissues composed of complex structures. This type of analysis can provide a precise quantitative description of a broad range of heterogeneous phenomena. Previously, we applied multifractal analysis to describe heterogeneity in white matter signal fluctuation on ...

To determine the rate of progression of white matter lesions and hemorrhages in a cohort with cerebral amyloid angiopathy (CAA). The authors analyzed data from 26 patients with possible (3) or probable (23) CAA, diagnosed by the Boston Criteria. Brain maps of white matter hyperintensities, normalized to head size (nWMH), ...

Serial T2-weighted magnetic resonance imaging in a 29-year-old woman with juvenile type dentatorubral-pallidoluysian atrophy (DRPLA) demonstrated that a cerebral white matter hyperintensity appeared within 2 months after status epilepticus and persisted for more than 20 months. The patient had rapidly progressive mental regression and became akinetic after status epilepticus. The ...

Magnetic resonance (MR) imaging features of white matter lesions, often seen in the elderly, are correlated with histologic findings. Dilatation of perivascular spaces is seen, especially in the frontal and/or parietal subcortical white matter; the spaces are less than 3 mm in diameter and have sharp margins with no perifocal ...

This work demonstrates that intraventricular microinjections of a low dose of potassium dichromate (0.4 microL of 10 mM solution) yield a specific contrast enhancement of white matter (WM) tracts in T1-weighted 3D MRI of mouse brain in vivo. Pronounced and persistent signal increases (40-100% at 24 hr after injection) were ...

The authors report a case of systemic lupus erythematosus with posterior leukoencephalopathy who presented with headache, tonic-clonic seizure, loss of consciousness and bilateral loss of vision, after taking azathioprine for three weeks. The patient had hypertension with normal eye grounds. The brain CT showed a hypodensity lesion at both bilateral ...

General paresis (parenchymatous neurosyphilis) is a rare disease, and in recent years the number of papers published on the magnetic resonance imaging findings has been limited. The findings are as follows: cerebral atrophy; mesiotemporal T2 hyperintensity; ventriculomegaly; pathological T2 hypointensity of the globus pallidus, putamen, the head of the caudate ...

Temporal lobe and limbic structures may be abnormal in bipolar disorder. T2-weighted magnetic resonance imaging (MRI) scans frequently show deep white matter lesions. MRI was performed on 50 young (19-39 years) euthymic patients with bipolar disorder and 26 controls. Mean temporal lobe volumes were reduced in patients (right, 9.42 cm3; ...

Voxel based morphometry (VBM) studies of Williams syndrome (WS) have demonstrated remarkably consistent findings of reduced posterior parietal gray matter compared to typical controls. Other WS VBM findings have been inconsistent, however. In particular, different findings have been reported for hypothalamus and orbitofrontal gray matter regions. We examined a sample ...

OBJECTIVE: To detect white matter abnormalities in patients with mild Alzheimer disease (AD) by diffusion tensor imaging and to determine their topographic relationship with gray matter atrophy. METHODS: Thirteen patients with mild AD and 16 normal age-matched volunteers underwent diffusion tensor imaging and three-dimensional spoiled gradient-recalled sequence scanning. Voxel-based morphometry ...

BACKGROUND: Aicardi-Goutierés syndrome (AGS) is a severe and progressive familial encephalopathy that is characterized by acquired microcephaly, intracranial calcification, white matter lesions, and chronic lymphocytosis with elevated levels of interferon-alpha in the cerebrospinal fluid. Although the degree of calcification and the severity of brain atrophy are variable, typically, the brain ...

The onset of multiple sclerosis is relapsing remitting or primary progressive. An improved understanding of the causes of early progressive disability in primary progressive multiple sclerosis (PPMS) could provide mechanistic targets for therapeutic intervention. Five magnetic resonance imaging (MRI) parameters that could potentially cause progressive disability were investigated in 43 ...

Sjögren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. Patients present the classical triad of congenital ichthyosis, mental retardation and spastic di- or tetraplegia. Magnetic resonance imaging (MRI) of the brain usually shows hypomyelination involving the periventricular white matter. ...

Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal neurological disorder of childhood and early adolescence. In this report, we present brain magnetic resonance spectroscopy (MRS) and conventional magnetic resonance MR imaging findings of early-stage (stage II) SSPE in an 8-year-old child. MRS demonstrated increased myo-inositol/creatine and choline/creatine ratios, and a ...

RATIONALE AND OBJECTIVES: Segmented inversion recovery (IR) ratio imaging (SIRRIM) has been established as a sensitive tool to assess neurodegeneration of the substantia nigra pars compacta (SN(C)) in patients with idiopathic Parkinson disease (IPD). The obtained results suggest the possibility of magnetic resonance imaging (MRI) as a biological marker for ...

The purpose of the study was to evaluate brain myelination by measuring the magnetization transfer ratio (MTR) and to measure grey (GMV) and white matter volume (WMV) in macrocephalic children with neurofibromatosis type 1 (NF1). Seven NF1 patients (aged 0.65-16.67 years) and seven age- and gender-matched controls were studied. A ...

OBJECTIVE: To elucidate the phenotype, genotype, and MRI findings of Korean patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and mutation carriers. METHODS: The authors studied 40 members of nine unrelated Korean CADASIL families. After genetic analysis of Notch3, clinical and MRI findings were correlated in ...

BACKGROUND: Primary blepharospasm is a focal dystonia characterised by excessive involuntary closure of the eyelids. The pathophysiology of primary blepharospasm is unresolved. AIM: To pinpoint grey-matter changes that are associated with primary blepharospasm. METHODS: 16 right-handed patients with primary blepharospasm (mean age 67.4 (SD 4.3) years; 12 women) were compared ...

Recently, several reports describing patients with a nonprogressive clinical course, increased signal in the cerebral white matter, and cystic changes in the anterior temporal lobes on magnetic resonance imaging (MRI) have appeared. To date, 25 patients with this very distinctive condition have been described. We report four new cases of ...

CT and MR imaging showed diffuse changes of the frontal white matter and genu of the corpus callosum with minimal atrophy and no contrast enhancement in a 41-year-old woman with progressive dementia. Brain biopsy disclosed axonal spheroids and gliosis in the white matter without macrophage or inflammatory infiltration or vessel ...

BK virus infection is most often associated with urologic disease in patients who have undergone renal or bone marrow transplantation. We report a rare case of biopsy-confirmed BK virus encephalitis in an immunocompromised patient with hemorrhagic cystitis, in whom dramatic imaging findings were present despite relatively mild clinical symptoms. MR ...

BACKGROUND AND PURPOSE: "Incidental" MRI white matter (WM) lesions, comprising periventricular lesions (PVLs) and deep subcortical lesions (DSCLs), are common in the aging brain. Direct evidence of ischemia associated with incidental WM lesions (WMLs) has been lacking, and their pathogenesis is unresolved. METHODS: A population-based, postmortem cohort (n=456) of donated ...

Structural and functional asymmetry of the human brain has been well documented using techniques such as magnetic resonance imaging (MRI). However, asymmetry of underlying white matter connections is less well understood. We applied an MRI technique known as diffusion tensor tractography to reveal the morphology of the white matter in ...

Subcortical vascular cognitive impairment is caused by lacunes and widespread ischemic white matter damage which closely resembles white matter abnormalities seen in multiple sclerosis. Recent evidence suggests that the progression rate of ischemic white matter lesions on MRI is very similar to that observed in multiple sclerosis. Consequently, it has ...

Recognized magnetic resonance imaging (MRI) abnormalities in the brains of patients with Fabry disease include the consequences of infarction and haemorrhage, non-specific white and grey matter lesions, vascular anomalies, in particular dolicho-ectasia, and a characteristic appearance of the posterior thalamus. A preliminary analysis of MRI findings in patients registered in ...

A 41-year-old man suspected of having lead poisoning was evaluated with MR imaging before and after British antilewisite therapy. The MR imaging findings showed bilateral symmetric involvement of the occipital lobe, affecting predominantly the gray-white matter junction and the subcortical white matter. A right cerebellar lesion was noted, with focal ...

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder consisting of external ophthalmoplegia, retinitis pigmentosa, ataxia and heart block. Magnetic resonance imaging (MRI) shows abnormal T2 high signal intensity in the deep gray matter nuclei, the cerebellar and the subcortical white matter. We report an unusual MR pattern of KSS, where the ...

We hypothesized that a combination of quantitative magnetic resonance imaging (MRI) sequences would detect a differential evolution of hypoxic-ischemic changes in white matter compared with gray matter in a recently developed model of unilateral mild cerebral hypoxia-ischemia in the 7-d-old rat. Using this model, which involved unilateral carotid artery occlusion ...

The objective of this study was to study genetic and phenotypic features of a family with X-linked Charcot-Marie-Tooth consisting of a healthy father, affected mother, two affected sons and one healthy one. A detailed electrophysiological and neuroimaging study, along with sequencing of the Cx32 gene, was performed in all family ...