Signal Intensity In White Matter Research Articles

Clinical, molecular pathological and genetic analyses of a Chinese family with congenital muscular dystrophy type 1A

To analyze and characterize the clinical, molecular pathological and genetic features of a Chinese family with congenital muscular dystrophy type 1A (MDC1A). Clinical data of the proband and her family members were collected. Immunohistochemistry staining was performed on muscular biopsy tissues with anti-merosin, alpha-dystroglycan, beta-dystroglycan and dystrophin antibodies. Genomic DNAs from the patient and her parents were extracted using standard procedures from the peripheral blood leukocytes. PCR and DNA direct sequencing were employed to analyze all of the 65 exons of the LAMA2 gene to determine the gene mutation, and the relationships between genotype and phenotype were analyzed. The proband presented with delayed motor development and a myopathic face. Her midrange elevated serum creatine kinase (CK) levels and white matter signal intensity changes are consistent with MDC1A, and was clinically diagnosed as MDC1A. The immunohistochemistry analysis for the proband exhibited complete loss of merosin staining. Further test with PCR detected a homozygous mutation of c.817A>T in exon 5, while her parents were heterozygotes for the mutation. The authors have defined the clinical manifestation of the Chinese family with MDC1A. The proband carried a homozygous nonsense mutation c.817A>T, and her parents were heterozygous carriers, consistent with autosomal recessive inheritance.

Chronological Changes of the Signal Intensities of White Matter on the FLAIR Images of Infants

Purpose: To assess the pattern of chronological change for the signal intensities of white matter on the FLAIR images of infants. Materials and Methods: FLAIR, T1- and T2-weighted images of 119 infants (newborn to 24 months age) were obtained by using a 1.5 T MRI machine. From these images, the signal intensities of 9 different white matter regions were compared to those of the adjacent gray matter and the signal intensities of each image were scored from 1 to 5. Results: The FLAIR images show high signal intensity for the cerebellar peduncle and posterior limb of the internal capsule at birth, but changed to low signal intensities in 2 to 3 months. The low signal intensities of the occipital-, parietal-, and frontal deep white matter and subcortical white matter changed to high signal intensities in 2 months, and they returned to low signal intensities in 10, 11, 12 and 19 months, respectively. Conclusion: The signal intensity of the white matter of infants showed peculiar chronologic changes on the FLAIR images. This knowledge could be helpful for the differentiation of normal white matter development and the lesions that mimic white matter disease in infants.

A case of hippocampal sclerosis diagnosed as cortical dysplasia due to preoperative brain MRI finding

= Abstract = Hippocampal sclerosis (HS) is one of the most common features of intractable temporal lobe epilepsy. Generally it can be identified through brain magnetic resonance imaging (MRI) with high degree of sensitivity and specificity. Typical brain MRI findings of HS are hippocampal atrophy with hyperintense signal confined to the lesion. On the other hand cortical dysplasia exhibits blurring of the gray-white matter junction and abnormal white matter signal intensity. We present a case where preoperative brain MRI strongly suggested the presence of diffuse cortical dysplasia in the left temporal lobe but postoperative pathology revealed the temporal lesion to be unremarkable except for hippocampal sclerosis. (Korean J Pediatr 2010;53:106-110)

Fukutin gene retrotransposal insertion in a non‐Japanese Fukuyama congenital muscular dystrophy (FCMD) patient

Fukuyama-type congenital muscular dystrophy (FCMD) is an autosomal recessive disorder, characterized by severe muscular dystrophy associated with brain malformation. FCMD is the second most common form of muscular dystrophy and one of the most common autosomal recessive diseases among the Japanese population; however, no typical FCMD cases have been reported in any other population. In this study, we report on the first identification of a Chinese FCMD patient; our findings are supported by clinical, histological, and magnetic resonance imaging (MRI) evidence, as well as fukutin gene mutational analyses. The patient presented with neonatal hypotonia, seizures, and delayed motor and speech development. Additional testing revealed cerebral and cerebellar gyrus abnormalities with white matter signal intensity changes, elevated serum creatine kinase (CK) levels, and dystrophic skeletal muscle with alpha-dystroglycan hypoglycosylation, and normal beta-dystroglycan and merosin expression. Genetic analysis of the fukutin gene showed one copy with a Japanese founder 3-kilobase (kb) retrotransposal insertion in the 3'-non-coding region and the other copy with a known c.139C>T mutation. This is the first FCMD case reported in the Chinese population and the first case in which the 3-kb insertion has been found outside of the Japanese population. This report emphasizes the importance of considering the fukutin founder mutation for diagnostic purposes outside of Japan.

Exercise-induced acute kidney injury with reversible posterior leukoencephalopathy syndrome

We report a case of a 17-year-old boy suffering from severe loin pain and oliguric acute kidney injury after strenuous exercise, with slightly elevated serum myoglobin and creatinine phosphokinase. Exercise-induced acute kidney injury (AKI) was diagnosed. We started intermittent hemodialysis thrice a week from the admission day. Four days later, he temporally lost consciousness, followed by visual agnosia and general clonic seizure. T2-weighted brain magnetic resonance images (MRI) showed multiple areas of increased signal intensity in the subcortical white matter, predominantly in the cerebrum of the posterior and parietal lobes and in the cerebellum. Clinical symptoms improved without sequelae. Follow-up MRI 1 month later showed complete resolution of the signals, and he was diagnosed with reversible posterior leukoencephalopathy syndrome (RPLS). This is the first reported case of exercise-induced AKI associated with RPLS. Vasoconstriction and endothelial dysfunction are considered as the common etiology of these diseases.

Variations of brain magnetic resonance imaging among manganese-exposed workers

Variations of the signal intensities in the magnetic resonance (MR) T(1)-weighted image (T(1)WI) of globus pallidus among manganese(Mn)-exposed workers were explored to provide a scientific basis for exposed biomarker of manganese-injured central nervous system (CNS). The brain MR T(1) and T(2) WI in eighteen male asymptomatic Mn-exposed, eight manganism and nine healthy control workers were examined routinely by adopting a 1.5 Tesla signal superconducting system. The SIGP and the signal intensity in frontal white matter (SIFWM) in the same side were determined, then pallidal index (PI) was calculated. Concentration of MnO(2) in workplaces and content of manganese in red blood cell (MnRBC) among workers were respectively determined by flame atomic absorption spectrometer (AAS) and inductively coupled plasma-atomic emission spectrophotometry (ICP-AES). The follow-up investigation in the eight high Mn-exposed workers was made one year later. The results showed that the median of air MnO(2) in smelting workplace was 0.64 mg/m(3)(0.07 - 5.40 mg/m(3)), which were respective 0.56 mg/m(3)(0.09 - 1.71 mg/m(3)) in power distribution room (low Mn-exposure) and 0.89 mg/m(3) (0.07 - 5.40 mg/m(3)) in furnace (high Mn-exposure). PI in the Mn-exposed and high Mn-exposed workers were both higher than those of the manganism and control workers(116.4 +/- 8.2, 119.0 +/- 7.9, 105.3 +/- 8.4 and 102.2 +/- 1.5, respectively. Mn vs control, t' = 7.146, P = 0.000; Mn vs manganism, t = 3.181, P = 0.004. High Mn-exposure vs control, t' = 7.446, P = 0.000; high Mn-exposure vs manganism, t = 3.763, P = 0.001). The increased signal in T(1)WI of globus pallidus was observed in Mn-exposed workers, especially in high Mn-exposed workers. The content of manganese in red blood cell of Mn-exposed and control workers was significantly higher than those of the manganism workers [(151.6 +/- 40.5) ng/ml, (149.2 +/- 21.3) ng/ml, (154.5 +/- 46.6) ng/ml, (144.4 +/- 14.2) ng/ml, (20.8 +/- 7.4) ng/ml respectively. The difference was significant in statistics. Manganism vs control, t = 20.206, P = 0.000; manganism vs Mn, t' = 13.144, P = 0.000; manganism vs low and high Mn, t' = 12.964, 9.957, respectively, P = 0.000]. Only a decreased median of air MnO(2) in furnace was found one year later (0.89, 0.31 mg/m(3), Z = -2.142, P = 0.032). The difference was significant in statistics. Our data suggests that SIGP of MR T(1)WI among workers was obviously increased by manganese-exposure. PI may be taken as the signal of CNS injury which was induced by manganese-exposure.

Dynamic Contrast-Enhanced Perfusion MR Imaging With SPIO

To investigate the feasibility of dynamic susceptibility-weighted contrast material-enhanced (DSC) perfusion imaging with ferucarbotran at 3.0 T, and to assess the impact of the different characteristics of gadolinium (Gd)- and ferucarbotran-based agent in rCBV measurement under pathologic condition with BBB disruption. Nine patients with suspected meningioma (4 men and 5 women; age range, 27-79 years; mean age, 61.6 +/- 17.9 years) were eligible for the study. Approval was obtained from the institutional review board. All participants provided signed informed consent prior to the study. DSC perfusion images were acquired with a T2*-weighted echo-planar imaging sequence during the first pass of a standard-dose of Gd-based agent, and of 4, 8, or 16 micromol Fe/kg of ferucarbotran-based agent in 3 patients for each dose respectively. Pre- and postenhanced T1-weighted images were obtained in all. Ferucarbotran decreased the signal intensity in both gray and white matter in a dose-dependent fashion. Differences regarding the DeltaSI between the 4 and 8 or 8 and 16 micromol Fe/kg groups were statistically significant (P < 0.05, 1-way analysis of variance, Tukey-Kramer method) in any region. No effect of leakage of ferucarbotran was demonstrated in the intensity-time curves or in the postenhanced T1-weighted images. Relative CBV in tumor area obtained with Gd was reduced to 37.6%-86.5% compared with that obtained with ferucarbotran. T2*-weighted DSC perfusion imaging with ferucarbotran is feasible at 3.0 T. Compared with Gd-based agent, larger particle size of ferucarbotran can diminish the effect of contrast leakage during the first pass under pathologic conditions with BBB disruption.

Neurosarcoidosis presenting as spontaneously remitting hypersomnia

A 38-year-old woman was first admitted to our hospital in November 2007 because of excessive daytime somnolence and short-term memory disturbance. She reported that her speech had gradually become slower starting in April 2007. Severe fatigue and somnolence had developed at the beginning of August 2007. She felt very sleepy and had to take long naps, generally sleeping all day except for at meal times, and she was therefore unable to do housework. Hypersomnia and chronic fatigue persisted after she was admitted to the hospital. On admission, her body temperature was 35.8 C, heart rate was 99 beats/min, and blood pressure was 118/56 mm Hg. Physical and neurological findings were uninformative with the exception of mild cognitive dysfunction; her MiniMental State Score was 23/30. She had daytime somnolence and her Epworth Sleepiness Score [4] was 18 points. Blood cell counts and serum levels of sodium, potassium, creatinine, and urea nitrogen as well as liver enzymes, glucose, angiotensin-converting enzyme (ACE), and adenosine deaminase (ADA) were all within normal ranges. However, cerebrospinal fluid (CSF) cell count (17/ll) and protein level (139 mg/dl) were elevated. Activity of ACE (0.2 U/l) and ADA (2.2 IU/l) in the CSF were within the normal range, and CSF culture and cytology were negative. CSF hypocretin-1 (orexin A) concentration was decreased (82.8 pg/ml: lower limit of normal = 110 pg/ml) [5]. T2-weighted magnetic resonance imaging (MRI) showed high-intensity signals in the brainstem, hypothalamus, basal ganglia, and white matter surrounding the lateral ventricle (Fig. 1a). Gadolinium-enhanced T1weighted MRI (Gd-MRI) demonstrated numerous small spots of high intensity in the bilateral thalamus, hypothalamus, and basal ganglia (Fig. 1b). Bilateral hilar lymphadenopathy (BHL) was absent on chest X-ray. Neither chest computed tomography (CT) nor whole body gallium scanning revealed abnormal lesions. The patient was clinically diagnosed with central nervous system (CNS) sarcoidosis associated with secondary hypersomnia, although the diagnosis was not confirmed by laboratory data at that time. The hypersomnia spontaneously and gradually improved. Therefore, no medication was initiated, and she was discharged from the hospital in December 2007. Six months later, the hypersomnia and memory disturbance had disappeared completely. The high-intensity areas in the bilateral thalamus and basal ganglia on MRI T2-weighted imaging, as well as the numerous small spots on gadolinium-enhanced T1-weighted imaging, had disappeared by May 2008 (Fig. 1c, d). Mild memory disturbance recurred in September 2008, and new bilateral cerebral white matter lesions were seen on brain MRI, which demonstrated foci of abnormal signal intensity in the bilateral white matter; these showed decreased signal Y. Nakazato (&) S. Kondo A. Ohkuma Y. Ito N. Tamura N. Araki Department of Neurology, Faculty of Medicine, Saitama Medical University, 38 Morohongo, Moroyama, Iruma-gun, Saitama 350-0495, Japan e-mail: nakachan@saitama-med.ac.jp

Age-associated alterations in cortical gray and white matter signal intensity and gray to white matter contrast

Prior studies have focused on patterns of brain atrophy with aging and age-associated cognitive decline. It is possible that changes in neural tissue properties could provide an important marker of more subtle changes compared to gross morphometry. However, little is known about how MRI tissue parameters are altered in aging. We created cortical surface models of 148 individuals and mapped regional gray and white matter T1-weighted signal intensities from 3D MPRAGE images to examine patterns of age-associated signal alterations. Gray matter intensity was decreased with aging with strongest effects in medial frontal, anterior cingulate, and inferior temporal regions. White matter signal intensity decreased with aging in superior and medial frontal, cingulum, and medial and lateral temporal regions. The gray/white ratio (GWR) was altered throughout a large portion of the cortical mantle, with strong changes in superior and inferior frontal, lateral parietal, and superior temporal and precuneus regions demonstrating decreased overall contrast. Statistical effects of contrast changes were stronger than those of cortical thinning. These results demonstrate that there are strong regional changes in neural tissue properties with aging and tissue intensity measures may serve as an important biomarker of degeneration.

Age-Associated Alterations in Cortical Gray Matter and Subjacent White Matter Signal Intensity

Age-Associated Alterations in Cortical Gray Matter and Subjacent White Matter Signal Intensity

T2 at MR Imaging Is an Objective Quantitative Measure of Cerebral White Matter Signal Intensity Abnormality in Preterm Infants at Term-equivalent Age

To compare quantitative T2 relaxometry of cerebral white matter (WM) with qualitative assessment of conventional T2-weighted magnetic resonance (MR) images, to assess the relationship between cerebral WM T2 and region-specific apparent diffusion coefficient (ADC), and to examine WM T2 regional variation in preterm infants at term. The local ethical committee granted ethical permission for this study; informed parental consent was obtained for each infant. Sixty-two preterm infants born at less than 32 weeks gestation and nine control infants were examined at 1.5 T; T2-weighted fast spin-echo MR images, T2 relaxometry data, and diffusion-weighted MR images were acquired. Conventional T2-weighted MR images were assessed by a pediatric neuroradiologist for diffuse excessive high signal intensity (DEHSI) in WM. Regions of interest were positioned in frontal WM, central WM, and posterior WM at the level of the centrum semiovale. In preterm infants at term, T2 was longer in all WM regions than in control infants; in infants with DEHSI, T2 was longer than in infants without DEHSI and control infants, with posterior WM T2 being longer than central or frontal WM T2. In control infants, T2 was similar in all WM regions. Frontal and posterior WM ADCs were higher in preterm infants at term than in control infants. Cerebral WM T2 is an objective quantitative measurement that can easily and rapidly be obtained during clinical MR imaging in preterm infants at term.

Brain Diffusion-Weighted and Diffusion Tensor Imaging Findings in an Infant with Biotinidase Deficiency: Fig 1.

A previously healthy 7-week-old male infant was admitted because of clonic movements of the upper limbs. Findings of a neurologic examination were normal. His psychomotor development was appropriate for his age. Brain MR imaging showed supra- and infratentorial white matter signal-intensity

Magnetic resonance imaging evaluation of radiation: Cranial radiation therapy-induced diffuse white matter injury

e13033 Background: To study the value of MRI in detection and grading of diffuse white matter injury induced by cranial radiation therapy for brain tumors and to evaluate the influence of various risk factors for its development. Methods: This study included 60 patients who had primary brain tumors and received external beam conventionally fractionated radiotherapy. Twenty-three out of 60 patients received chemotherapy after radiotherapy. Post radiotherapy follow-up MRI examination was performed for all patients at 6 weeks, 3, 6, 9, 12 months, and at more than 1 year from completion of radiotherapy. MR imaging was performed with T2-weighted turbo spin echo, fast fluid-attenuated inversion- recovery (FLAIR), T1-weighted spin-echo, and T1-weighted post-contrast spin-echo sequences. Results: Twenty-one of 60 patients (35%) developed diffuse white matter injury at a mean of 15.3 + 7.6 months from the completion of radiotherapy (range, 5–36 months). Radiation-related diffuse white matter changes were characterized as diffuse high signal intensity in the periventricular deep white matter on T2WI and FLAIR sequences. The MRI grading system of these periventricular hyperintensity (PVH) were as follow: 39 patients (65%) had no WMI (grade 0), four had grade 1 changes, three patients had grade 2 changes, nine patients had grade 3, and five patients had grade 4. They were symmetrical in 13 of 21 patients (61.9%), and asymmetrical in eight out of 21 patients (38.1%). The prevalence of diffuse matter injury found to be significantly related to the volume of irradiation (p = 0.02), fraction size (p = 0.008), and concomitant use of chemotherapy (p &gt; 0.02)Conclusions: Diffuse white matter injury is a known consequence of radiation therapy, and the capability of detecting and grading such damage is optimally provided by MRI. No significant financial relationships to disclose.

Occurrence of ankylosing spondylitis and multiple sclerosis-like syndrome in a HLA-B27 positive patient

Occurrence of multiple sclerosis (MS) in patients with ankylosing spondylitis (AS) has been reported in isolated cases. We describe a white 33-year-old male with a definite familial HLAB27 positive AS and MS-like syndrome. The patient developed acute onset of gait difficulty, postural unsteadiness, dysarthria and right side weakness that resolved within 1 month; after 6 months he presented right-sided face sensory loss, disappeared after 2 weeks. Brain and cervical MRI was performed twice and showed disseminated lesions in space (multiple foci of increased signal intensity in the periventricular white matter, in the corpus callosum, in the hypothalamus, in the brainstem and in the cervical spinal cord) and in time (a new enhancing lesion >3 months after the onset of the clinical event). Visual evoked potentials were markedly altered. Cerebrospinal fluid examination was negative for intrathecal production of oligoclonal bands. Differential diagnosis was considered and other pathologies were excluded.

MRI of acute experimental allergic encephalomyelitis: correlation with pathology

Objective To investigate the MR imaging findings of acute experimental allergic encephalomyelitis(EAE) in correlation with pathology. Methods An EAE model was induced by intradermal inoculation with guinea pig CNS homogenate in 6 female Lewis rats.Another 6 rats served as control.The clinical presentation and body weight of the animals were recorded daily. Routine MRI,Gd-enhanced MRI were performed when EAE animals showed the initial symptoms. Uhrasmall superparamagnetic iron oxide(USPIO) colloid solution was also administrated intravenously and MRI was performed again after 24 hours. The brain was removed instantly after the second MR imaging. The pathological exams including HE staining,myelin sheath staining and prussian staining were performed.The imaging findings were observed in correlation with pathological results. Results The EAE rats showed decrease of body weight on the 6th to 7th day after inoculation,and the clinical symptoms appeared on the 10th to 11th day after inoculation.Routine MRI did not show any definite abnormalities.The Gd-enhanced MRI found the diffuse thickening and enhancement of brain meninges.The USPIO-enhanced MRI showedareas of low signal intensity at white matter of medulla oblongata on T2WI,and high signal intensity was observed at the corresponding area on T1 WI. Gradient T2 * WI found more foci of low signal intensity in eerebellar white matter besides the lesions in the brain stem.The range of abnormal signal intensity was larger in animal with higher clinical scores than that with lower score.There were no abnormal findings in control animaL The pathological exam found perivascular cuff in the brain white matter in EAE animals,some accompanied with adjacent demyelinatian. The prussian staining found blue particles within the cytoplasm of the macrophages around the lesion,which corresponded to the area of low signal intensity on T2WI.Conclusion USPIO-enhanced MRI could reveal acute EAE lesions which were not capable of being shown on routine MRI and Gd-enhanced MRI.It can image the macrophages around the lesions in vivo.USPIO is important for future research and application in MS patients. Key words: Encephalomyelitis,autoimmuc,experimental; Magnetic resonance imaging; Pathology

SPG11 mutations cause Kjellin syndrome, a hereditary spastic paraplegia with thin corpus callosum and central retinal degeneration

Autosomal recessive hereditary spastic paraplegia (ARHSP) with thin corpus callosum (TCC) is genetically heterogenous and approximately 35% of patients carry mutations in either of the SPG11 or SPG15 genes. Disease onset is during the first three decades of life with spastic paraplegia and mental impairment. Peripheral neuropathy and amyotrophy may occur. Kjellin syndrome is characterized by central retinal degeneration in addition to ARHSP-TCC and the disease is associated with mutations in the SPG15 gene. We identified five patients in four unrelated kindreds with spastic paraplegia and mental impairment. Magnetic resonance imaging revealed TCC, atrophy elsewhere in the brain and increased T2 signal intensity in the periventricular white matter. Probands from the four kindreds were screened for mutations in the SPG11 gene. All patients were found homozygous or compound heterozygous for truncating SPG11 mutations of which four are reported for the first time. Ophthalmological investigations revealed that the four index cases have central retinal degeneration consistent with Kjellin syndrome. PET examinations with N-[11C-methyl]-L-deuterodeprenyl (DED) and fluor-18 2-fluorodeoxyglucose (FDG) were performed in two patients with Kjellin syndrome. We observed a reduced glucose uptake in the thalami, anterior cingulum, and sensorimotor cortex indicating neuronal loss, and an increased DED binding in the thalami and pons which suggests astrogliosis. From our results we extend the SPG11 associated phenotype to comprise also Kjellin syndrome, previously found to be associated with mutations in the SPG15 gene. We anticipate that degeneration of the central retina is a common and previously unrecognized feature in SPG11 related disease.

Magnetic Resonance Imaging, Magnetic Resonance Spectroscopy, and Facial Dysmorphism in a Case of Lowe Syndrome with Novel OCRL1 Gene Mutation

Lowe syndrome is a multisystem disorder characterized by anomalies of the eye, the nervous system, and the kidney. It is an uncommon, X-linked disease. Bilateral cataract and severe hypotonia are present at birth. Psychomotor retardation is evident in childhood, while renal complications arise in adolescence. The mutation of the gene OCRL1 localized at Xq26.1 is responsible for the disease. The authors report on a 12-year-old male with mental retardation, facial dysmorphism as prominent forehead, long and slender-shaped face, prominent eyebrows, epicanthus, microphthalmia, low-posterior set ears with prominent helix and antihelix, long philtrum, and mild prognathia. He also had history of neonatal hypotonia and congenital cataracts. His cranial magnetic resonance imaging showed increased signal intensity in white matter on T2-weighted images, and magnetic resonance spectroscopy revealed elevation of the myoinositol peak at 3.56 ppm. Molecular analysis of OCRL1 gene revealed novel N574K mutation on 17th exon.

Magnetic resonance imagining findings of the white matter abnormalities in the brain of very-low-birth-weight infants

Purpose : To observe the abnormal white matter findings on the magnetic resonance imaging (MRI) scans of very-lowbirth-weight (VLBW) infant brains at term-equivalent age and to determine the clinical risk factors for the development of periventricular leukomalacia (PVL). Methods : In all, MRI was performed in 98 VLBW infants and the white matter abnormalities were observed. Clinical risk factors for cystic and noncystic PVL were determined. Results : MRI scans of 74 infants (75.5%) showed diffuse excessive high signal intensity (DEHSI) in the periventricular white matter, 17 (17.3%) lateral ventricle dilation, 5 (5.1%) and 11 (11.2%) focal punctate lesions and cystic changes in the periventricular white matter, respectively, 9 (9.1%), germinal layer hemorrhage (GLH) or subependymal cysts 3 (3.1%) intraventricular hemorrhage (>grade 2) 2 (2.0%) posthemorrhagic hydrocephalus and 2 (2.0%) periventricular hemorrhagic infarct. Gestational age (GA), 1-minute Apgar score, Clinical Risk Index for Babies-II (CRIB-II) score, and inotrope use, and GA, CRIB-II score, postnatal steroid administration, inotrope use, and abnormal white blood cell (WBC) count at admission were related to cystic PVL and noncystic PVL development, respectively (P<0.05). However, in logistic regression analysis, CRIB-II (odds ratio, 1.63, 295% confidence interval, 1.15–2.30 P=0.006) for cystic PVL, and GA (odds ratio 0.90, 95% confidence interval, 0.82–0.99 P=0.036) for noncystic PVL were only significant independently. Conclusion : White matter abnormalities could be observed on MRI scans of the VLBW infant brains at term-equivalent age, and CRIB-II and GA were only independently significant for cystic and noncystic PVL development, respectively. (Korean J Pediatr 2009;52:1127-1135)

MR Imaging Detection of Cerebral Microbleeds: Effect of Susceptibility-Weighted Imaging, Section Thickness, and Field Strength

The emergence of cerebral microbleeds (CMB) as common MR imaging findings raises the question of how MR imaging parameters influence CMB detection. To evaluate the effects of modified gradient recalled-echo (GRE) MR imaging methods, we performed an analysis of sequence, section thickness, and field strength on CMB imaging properties and detection in subjects with cerebral amyloid angiopathy (CAA), a condition associated with microhemorrhage. Multiple MR images were obtained from subjects with probable CAA, with varying sequences (GRE versus susceptibility-weighted imaging [SWI]), section thicknesses (1.2-1.5 versus 5 mm), and magnetic field strengths (1.5T versus 3T). Individual CMB were manually identified and analyzed for contrast index (lesion intensity normalized to normal-appearing white matter signal intensity) and diameter. CMB counts were compared between 1.5T thick-section GRE and thin-section SWI for 3 subjects who underwent both protocols in the same scanning session. With other parameters constant, use of SWI, thinner sections, and a higher field strength yielded medium-to-large gains in CMB contrast index (CI; Cohen d 0.71-1.87). SWI was also associated with small increases in CMB diameter (Cohen d <0.3). Conventional thick-section GRE identified only 33% of CMB (103 of 310) seen on thin-section SWI. Lesions prospectively identified on GRE had significantly greater CI and diameter measured on the GRE image than those not prospectively identified. The examined alternatives to conventional GRE MR imaging yield substantially improved CMB contrast and sensitivity for detection. Future studies based on these techniques will most likely yield even higher prevalence estimates for CMB.

MR Imaging Characteristics and Neuropathology of the Spinal Cord in Adult-Onset Autosomal Dominant Leukodystrophy with Autonomic Symptoms

MR imaging findings in adult-onset autosomal dominant leukodystrophy (ADLD) with autonomic symptoms have been described in the brain, but no descriptions of MR imaging findings in the spinal cord have been published. Here, we describe MR imaging findings in the spinal cord in adult-onset ADLD with autonomic symptoms and histopathologic investigations of the spinal cord. Twelve subjects from 2 families with adult-onset ADLD with autonomic symptoms identified by clinical investigation underwent MR imaging examination of the spinal cord. Sagittal and transverse sections were obtained. MR imaging examination of the brain was performed in 11 patients. One of the patients underwent postmortem examination, and the spinal cord was subjected to histopathologic analysis. In all family members with adult-onset ADLD with autonomic symptoms, even in the asymptomatic person, the spinal cord was thin. All examined family members also had a slight general white matter signal intensity (SI) increase in the whole spinal cord, mainly visible in T2-weighted transverse images. The pathologic examination revealed a discrete demyelination in the spinal cord. Brain MR imaging also showed increased T2 SI in the white matter. The spinal cord is affected in adult-onset ADLD with autonomic symptoms. Findings consist of atrophy and a diffuse T2 SI increase in the white matter. Transverse images are needed to assess these findings. The typical SI changes of the spinal cord are also present in subjects without clinical symptoms of the disease and with very limited changes in the brain.