Biomedical subjects

Petra J W Pouwels

Publications and source records attributed to Petra J W Pouwels.

17 recordsLinked to original sources

PubMed2006

Human gray matter: feasibility of single-slab 3D double inversion-recovery high-spatial-resolution MR imaging.

The purpose of this study was to develop and prospectively evaluate the feasibility of a single-slab three-dimensional (3D) double inversion-recovery, or DIR, sequence for magnetic resonance imaging at 1.5 T. The study was approved by the local ethics committee, and informed consent was obtained from six healthy control subjects (one woman, five men; age range, 26-47 years) and two patients with multiple sclerosis (one woman, aged 39; one man, aged 56). Gray matter (GM)-only images were obtained by selectively suppressing cerebrospinal fluid (CSF) and white matter (WM) signals. Whole-brain high-spatial-resolution 3D images (1.2 x 1.2 x 1.3 mm) were acquired within 10 minutes. Cortical and deep GM structures were clearly delineated from WM and CSF, and there were regional differences in GM signal intensity. No flow artifacts from blood or CSF were observed. These GM images with high spatial resolution are suitable to identify cortical pathologic conditions and can potentially be used for segmentation purposes to determine cortical thickness or volume.

Adult↗

PubMed2006

Whole-brain T1 mapping in multiple sclerosis: global changes of normal-appearing gray and white matter.

PURPOSE: To prospectively investigate whether T1 changes in normal-appearing white matter (WM) and normal-appearing gray matter (GM) in multiple sclerosis (MS) are global or regional and their relationship to disease type. MATERIALS AND METHODS: The institutional ethics review board approved study; written informed consent was obtained. Whole-brain T1 maps were obtained in 67 patients with MS and 24 healthy control subjects with three-dimensional fast low-angle shot flip angle-array method, with correction for B(1) imperfections. Analysis of variance was performed on T1 histogram parameters of global normal-appearing WM and GM. Regional mean T1 values were analyzed with a multilevel approach. Multiple linear regression analysis was performed to investigate associations with clinical disability and overall atrophy. For patients, T2 lesion load was determined. RESULTS: T1 histograms of normal-appearing WM had significantly higher peak positions for patients with MS (792 msec +/- 36 in secondary progressive [SP] MS) than for control subjects (746 msec +/- 23) and were significantly broader and lower (all P < .001). Histograms for cortical normal-appearing GM were significantly shifted (peak positions, 1263 msec +/- 44 in control subjects and 1355 msec +/- 62 in patients with SP MS) (P < .001). Histogram peak positions were significantly higher in SP MS than in relapsing-remitting (RR) and primary progressive MS (P < .05). In SP disease, at least 31% of normal-appearing WM and 20% of cortical normal-appearing GM were affected. In MS, T1 was significantly elevated in all normal-appearing WM and cortical normal-appearing GM regions (all P < .01) but was elevated only in the thalamus in deep GM (P < .05). Cortical T1 histogram peak position was associated with clinical disability; T2 lesion load was not. CONCLUSION: Results suggest that a global disease process affects large parts of both normal-appearing WM and GM in MS and effects are worse for SP MS than for RR MS.

Adult↗

PubMed2006

Altered diffusion tensor in multiple sclerosis normal-appearing brain tissue: cortical diffusion changes seem related to clinical deterioration.

PURPOSE: To investigate normal-appearing white (NAWM) and cortical gray (NAGM) matter separately in multiple sclerosis (MS) in vivo using diffusion tensor imaging (DTI). MATERIALS AND METHODS: In 64 MS patients (12 primary progressive [PP], 38 relapsing remitting [RR], 14 secondary progressive [SP]) and 20 healthy controls, whole-brain apparent diffusion coefficient (ADC) and fractional anisotropy (FA) maps were acquired. A stimulated echo acquisition mode (STEAM) DTI sequence was used with minimal geometrical distortion in comparison to echo-planar imaging (EPI). NAWM and NAGM were identified using conventional magnetic resonance (MR) images, allowing a cautious assessment of FA in cortex. RESULTS: Histogram analyses showed significant global FA decreases and ADC increases in MS NAWM compared to control WM. MS cortical NAGM had no significant global ADC increase, but FA was decreased significantly. In regional analyses, nearly all NAWM regions-of-interest (ROIs) had significantly increased ADC compared to controls, but FA was not changed. In nearly all cortical NAGM ROIs, ADC was significantly increased and FA significantly reduced. In multiple linear regression analyses in RR/SPMS patients, NAGM-ADC histogram peak height was associated more strongly with clinical disability than T2 lesion load. CONCLUSION: Tissue damage occurs in both NAWM and cortical NAGM. The cortical damage appears to have more clinical impact than T2 lesions.

Adult↗

PubMed2006

MR spectroscopic evidence for thalamic and hippocampal, but not cortical, damage in multiple sclerosis.

Gray matter (GM) damage is an important pathophysiological feature in Multiple Sclerosis (MS), and may be related to clinical, including cognitive, deficits. Quantitative single-voxel (1)H-Magnetic Resonance Spectroscopy (TR/TE 6000/20 ms) was performed in 33 MS patients (11 per disease subtype; mean age 48 years, 16 females) and 10 healthy controls (mean age 43 years, 7 females). No overall spectroscopic changes were found in MS cortex. In MS thalamus, a 9% decrease of N-acetyl aspartate (NAA; P=0.005) and a 31% increase of myo-inositol (Ins; P=0.002) were found. A 21% Ins increase was observed (P=0.02) in MS hippocampus. Reduced NAA and increased Ins concentrations are thought to reflect neuro-axonal damage or loss and gliosis, respectively. Significant correlations between Ins concentrations and total-brain T(2) lesion load were found for MS thalamus (r=0.65, P<0.001) and hippocampus (r=0.57, P=0.001). MS thalamic and hippocampal Ins concentrations also correlated with each other (r=0.68; P<0.001). Cortical Gln correlated with thalamic NAA (r=-0.38; P=0.03) in MS. Thalamic and hippocampal Ins increases were most prominent in secondary-progressive (SP) patients (37% and 34%, respectively), whereas the largest thalamic NAA decrease (14%) was found in primary-progressive (PP) patients. In conclusion, thalamic and hippocampal GM pathology are important features of (progressive) MS.

Adult↗

PubMed2006

Childhood white matter disorders: quantitative MR imaging and spectroscopy.

PURPOSE: To prospectively investigate whether quantitative magnetic resonance (MR) parameters, including magnetization transfer ratio (MTR), apparent diffusion coefficient (ADC), fractional anisotropy (FA), and MR spectroscopic metabolite concentrations, allow for discrimination between different types of pathologic conditions that underlie signal intensity abnormalities in white matter. MATERIALS AND METHODS: Institutional review board approval and informed consent were obtained. Forty-one patients (19 male, 22 female; mean age, 15.4 years) and 41 control subjects (25 male, 16 female; mean age, 11.3 years) were included. Twelve patients had a hypomyelinating disorder; 14, a demyelinating disorder; five, a disorder characterized by myelin vacuolation; and 10, a disorder characterized by cystic degeneration. Regions of interest were selected within the parietal white matter and were transferred to the corresponding sections of the generated ADC, FA, and MTR maps to extract quantitative measurements. Linear discriminant analysis and univariate analysis of covariance were used for statistical evaluation. RESULTS: Linear discriminant analysis showed that 95% of patients were correctly classified by using total creatine, choline-containing compounds, myo-inositol, MTR, and ADC. In the hypomyelination group, all MR parameters were close to normal, with the exception of elevated total creatine (P = .03) and myo-inositol (P < .001) levels and decreased MTR values (P < .001). In the demyelination group, the levels of choline-containing compounds (P = .02) and myo-inositol (P < .001) were highly elevated. In the myelin vacuolation and cystic degeneration groups, high ADC values (P < .001) and variable decreases in all MR spectroscopic metabolites were seen. MTR was significantly reduced (P < .001) in the cystic degeneration group. CONCLUSION: Quantitative MR techniques can be used to discriminate between different types of white matter disorders and to classify white matter lesions of unknown origin with respect to underlying pathologic conditions.

Adolescent↗

PubMed2005

Intracortical lesions in multiple sclerosis: improved detection with 3D double inversion-recovery MR imaging.

PURPOSE: To prospectively compare the depiction of intracortical lesions by using multislab three-dimensional (3D) double inversion-recovery (DIR), multislab 3D fluid-attenuated inversion-recovery (FLAIR), and T2-weighted spin-echo (SE) magnetic resonance (MR) imaging in patients with multiple sclerosis. MATERIALS AND METHODS: Local ethics review board approval and informed consent were obtained. Conventional T2-weighted SE and multislab 3D FLAIR and DIR images were acquired in 10 patients with multiple sclerosis (five women, five men) and 11 age-matched healthy control subjects (seven women, four men). Mean age was 40 years (range, 25-54 years) in patients and 34 years (range, 24-55 years) in control subjects. Lesions were classified according to seven anatomic regions: intracortical, mixed white matter-gray matter, juxtacortical, deep gray matter, periventricular white matter, deep white matter, and infratentorial lesions. The numbers of lesions per category were compared between techniques (Dunnett-corrected analysis of variance). Gain or loss (with 95% confidence intervals [CIs]) of numbers of lesions detected at 3D DIR imaging was calculated in comparison with those detected at T2-weighted SE and 3D FLAIR imaging. RESULTS: Total number of lesions did not differ between 3D DIR and 3D FLAIR sequences, but the 3D DIR sequence showed a gain of 21% (95% CI: 4%, 41%) in comparison with the T2-weighted SE sequence. Because of high gray matter-white matter contrast, DIR images depicted more intracortical lesions (80 lesions in 10 patients) than both SE (10 lesions) and FLAIR (31 lesions) images; gains with DIR were 538% (95% CI: 191%, 1297%) and 152% (95% CI: 15%, 453%) compared with SE and FLAIR, respectively. Only four intracortical lesions were detected in control subjects. Also, DIR imaging enabled a better definition of mixed white matter-gray matter lesions because of greater contrast between the lesion and its surroundings. CONCLUSION: MR imaging with 3D DIR enables increased intracortical lesion detection in the multiple sclerosis brain, as well as improved distinction between juxtacortical and white matter-gray matter lesions.

Adult↗

PubMed2005

Histopathologic correlates of radial stripes on MR images in lysosomal storage disorders.

BACKGROUND AND PURPOSE: Radially oriented hypointense stripes in hyperintense cerebral white matter are recognized on T2-weighted images of certain lysosomal storage disorders. We compared in vivo and postmortem MR imaging with histopathologic findings in three patients with metachromatic leukodystrophy (MLD), globoid cell leukodystrophy (GLD), and infantile GM1 gangliosidosis (GM1) to understand this characteristic MR imaging pattern. METHODS: The in vivo MR imaging protocol comprised T1- and T2-weighted spin-echo and fluid-attenuated inversion recovery imaging. Postmortem MR imaging, including coronal 1-cm-thick brain sections, was performed after at least 5 weeks of formalin fixation and included T2-weighted spin-echo images and 3D fluid-attenuated inversion recovery images with high spatial resolution. Afterward, the sections were embedded in paraffin, whole-mount sections were made, and neuropathologic stains were applied. RESULTS: Similar imaging features were found in the three patients on in vivo and postmortem images, with more prominent stripes in GLD and MLD than in GM1. Neuropathologic examination revealed that the stripes were related to relative sparing of myelin in the perivenular regions in GM1 and MLD, but lipid-containing glial cells were also present in these areas in MLD. Perivenular clusters of globoid cells containing lipid material in absence of any myelin corresponded to the stripes in GLD. CONCLUSION: Results of our postmortem study showed that radial stripes of white matter on MR images represented relative myelin sparing in some lysosomal storage disorders, but they may also represent lipid storage.

Adolescent↗

PubMed2005

Cortical lesions in multiple sclerosis: combined postmortem MR imaging and histopathology.

BACKGROUND AND PURPOSE: Cortical lesions constitute a substantial part of the total lesion load in multiple sclerosis (MS) brain. They have been related to neuropsychological deficits, epilepsy, and depression. However, the proportion of purely cortical lesions visible on MR images is unknown. The aim of this study was to determine the proportion of intracortical and mixed gray matter (GM)-white matter (WM) lesions that can be visualized with postmortem MR imaging. METHODS: We studied 49 brain samples from nine cases of chronic MS. Tissue sections were matched to dual-echo T2-weighted spin-echo (T2SE) MR images. MS lesions were identified by means of myelin basic protein immunostaining, and lesions were classified as intracortical, mixed GM-WM, deep GM, or WM. Investigators blinded to the histopathologic results scored postmortem T2SE and 3D fluid-attenuated inversion recovery (FLAIR) images. RESULTS: Immunohistochemistry confirmed 70 WM, eight deep GM, 27 mixed GM-WM, and 63 purely cortical lesions. T2SE images depicted only 3% of the intracortical lesions, and 3D FLAIR imaging showed 5%. Mixed GM-WM lesions were most frequently detectable on T2SE and 3D FLAIR images (22% and 41%, respectively). T2SE imaging showed 13% of deep GM lesions versus 38% on 3D FLAIR. T2SE images depicted 63% of the WM lesions, whereas 3D FLAIR images depicted 71%. Even after side-by-side review of the MR imaging and histopathologic results, many of the intracortical lesions could not be identified retrospectively. CONCLUSION: In contrast to WM lesions and mixed GM-WM lesions, intracortical lesions remain largely undetected with current MR imaging resolution.

Adult↗

PubMed2004

The use of high-resolution magnetic resonance imaging for monitoring interbody fusion and bioabsorbable cages: an ex vivo pilot study.

OBJECT: Interbody fusion is a gradual process of graft resorption and tissue formation, ideally resulting in a bone bridge between two adjacent vertebral bodies. Initially, fibrous tissue and cartilage are formed, which subsequently are replaced by bone through the process of endochondral ossification. When cages and/or their contents are made of resorbable polymers like lactic or glycolic acids, there is a simultaneous process of implant degradation, which is eventually accompanied by reactions in the surrounding tissues. The purpose of this study was to explore the use of highresolution magnetic resonance (MR) imaging for monitoring tissue differentiation, spinal fusion, cage degradation, and eventually tissue reactions as a function of time. METHODS: Lumbar vertebral segments obtained in 14 goats with 3, 6, and 12 months of follow up (three, four, and seven animals, respectively) were available from a study of the feasibility of poly(L,D-lactic acid) cages for spinal fusion. Plain x-ray films, MR images, and histological sections were used to evaluate spinal fusion and cage resorption. The first follow-up tests revealed that MR imaging noninvasively provided three-dimensional information on cage placement, cage degradation and bone formation, and that it has potential to differentiate between the various soft tissues. CONCLUSIONS: Although the magnetic field strength and thus the resolution used were higher than normal in clinical practice, MR imaging appears to be a promising modality for the noninvasive clinical follow up of patients who undergo fusion with resorbable cages. Tissue reactions were not encountered in this study, and thus could not be evaluated.

Absorbable Implants↗

PubMed2004

Quantitative 1H-MRS of healthy human cortex, hippocampus, and thalamus: metabolite concentrations, quantification precision, and reproducibility.

PURPOSE: To evaluate metabolite concentrations in cortical gray matter, hippocampus, and thalamus of healthy adults, and to investigate precision and reproducibility of quantitative proton magnetic resonance spectroscopy (1H-MRS) in these gray matter regions. MATERIALS AND METHODS: Quantitative single-voxel short echo-time spectra were obtained from healthy human cortex, hippocampus, and thalamus. Subjects were examined twice. Metabolite concentrations, quantification precision, and reproducibility were determined. RESULTS: There were no significant differences between test and retest measurements. Regional differences were observed with respect to metabolite concentrations, quantification precision, and reproducibility. Quantification precision and reproducibility of N-acetylaspartate and N-acetyl aspartylglutamate (tNAA), creatine and phosphocreatine (tCr), choline-containing compounds (Cho), and myo-inositol (myo-Ins), were better than those of glutamate (Glu) and glutamine (Gln). Generally, precision and reproducibility were better in cortex than in hippocampus or thalamus. The quantification precision was shown to correlate both with reproducibility and spectral linewidth. CONCLUSION: The reliability of quantitative MRS depends on the metabolite concerned, its concentration, and on the brain area studied. Moreover, the quantification precision of a metabolite in a single spectrum appears to be a reliable measure for its reproducibility in a longitudinal study.

Adult↗

PubMed2003

A new leukoencephalopathy with brainstem and spinal cord involvement and high lactate.

We identified eight patients with a distinct magnetic resonance imaging pattern of inhomogeneous cerebral white matter abnormalities and selective involvement of brainstem and spinal tracts. Proton magnetic resonance imaging showed increased lactate in the abnormal white matter. Clinically, the patients had slowly progressive pyramidal, cerebellar, and dorsal column dysfunction. The uniform, highly characteristic magnetic resonance imaging pattern and the similarities in clinical and magnetic resonance spectroscopy findings provide evidence for a new disease entity. Autosomal recessive inheritance is likely.

Adolescent↗

PubMed2003

Multislice T1 relaxation time measurements in the brain using IR-EPI: reproducibility, normal values, and histogram analysis in patients with multiple sclerosis.

PURPOSE: To perform T(1) measurements using inversion recovery (IR) echoplanar imaging (EPI) to evaluate reproducibility, normal values, and T(1) histogram analysis as a measure of disease progression in multiple sclerosis (MS) patients. MATERIALS AND METHODS: Multislice IR-EPI was performed in 10 controls and 36 MS patients. Region-of-interest (ROI) and T(1) histogram analysis were performed on T(1) maps and compared to hypointense T(1) lesions and brain atrophy in MS patients. RESULTS: Coefficient of variation (COV) varied from 1.6% to 4.9%. Callosal normal (appearing) white matter (N(A)WM) showed the lowest and cortical gray matter the highest T(1) values. T(1) histogram analysis in controls showed a sharp WM peak centered on a T(1) value of 729 msec (range = 679-765) with extension into a shoulder of higher T(1) values. In MS patients, a shift toward higher T(1) values (mean = 788 msec, range = 700-957) with a lower relative peak amplitude was present, predominantly resulting from T(1) prolongation in NAWM. T(1) histogram parameters strongly related to hypointense T(1) lesion volume and brain atrophy in MS patients. CONCLUSIONS: IR-EPI provides a reproducible method to obtain T(1) values in the brain. Regional variation in T(1) values is present in N(A)WM of volunteers and MS patients. Since T(1) histogram parameters reflect changes in NAWM and correlate with conventional measures of disease burden in MS patients, T(1) histogram analysis may provide a global measure of disease progression in MS.

Adult↗

PubMed2003

Cerebral proton magnetic resonance spectroscopy of a patient with giant axonal neuropathy.

Magnetic resonance imaging of a girl with giant axonal neuropathy revealed a progressive white matter disease. In close agreement with histopathological features reported previously, localized proton magnetic resonance spectroscopy at 9 and 12 years of age indicated a specific damage or loss of axons (reduced N-acetylaspartate and N-acetylaspartylglutamate) accompanied by acute demyelination (elevated choline-containing compounds, myo-inositol, and lactate) in white matter as well as a generalized proliferation of glial cells (elevated choline-containing compounds and myo-inositol) in both gray and white matter.

Axons↗

PubMed2002

Magnetic resonance image registration in multiple sclerosis: comparison with repositioning error and observer-based variability.

PURPOSE: To study the use of image registration in the analysis of multiple sclerosis (MS) lesion volume and compare this with repositioning error and observer-based variability. MATERIALS AND METHODS: The normalized mutual information (NMI) algorithm is evaluated in an accuracy study using a phantom, followed by a validation study on magnetic resonance (MR) data of MS patients. Further, using scan-rescan MR data, the effect of registration on MS lesion volume compared with repositioning error and observer-based variability is assessed. RESULTS: The registration accuracy was near perfect in the phantom study, while the in vivo validation study demonstrated an accuracy on the order of 0.2-0.3 mm. In the scan-rescan study, quantification accounted for 15.6% of the relative variance, repositioning for 44.4%, and registration for 40.0%. CONCLUSION: NMI resulted in robust and accurate alignment of MR brain images of MS patients. Its use in the detection of changes in MS using large serial MR imaging (MRI) data warrants future evaluation.

Adult↗

PubMed2002

Image registration and subtraction to detect active T(2) lesions in MS: an interobserver study.

Serial MRI studies are used to analyse change in multiple sclerosis (MS) lesion volume in clinical trials. As such an evaluation is very time consuming and subject to quantification errors, one might assess only the change in number or size of lesions using subtracted images. The advantage of subtracted images is that both new and/or enlarging and resolving and/or shrinking lesions can be evaluated, resulting in a more precise volume change than a net volume change. We studied the interobserver agreement in the detection of active MS lesions using paired dual-echo T(2)-weighted spin-echo studies (3-mm slices) of 30 MS patients with a range of MS disease activity on MRI from treatment trials. Using an automatic matching algorithm based on mutual information, the follow-up scan was registered to baseline, after which subtracted images were obtained. After a training session with formulation of guidelines, six observers identified new, enlarging, resolving and shrinking lesions on subtracted images. Weighted kappa (kappa) values were calculated to assess interobserver agreement. Good agreement was found for new lesions (kappa 0.69 +/- 0.08), while moderate agreement was found for enlarging lesions (kappa 0.52 +/- 0.06). When new and enlarging lesions were combined, good agreement was found for "positive" activity (kappa 0.71 +/-0.06). The interobserver agreement was poor for resolving lesions (kappa 0.31 +/- 0.07), and moderate for shrinking lesions (kappa 0.53 +/- 0.08). In conclusion, the use of subtracted images in the visual detection of new T(2) lesions resulted in a good level of interobserver agreement for "positive" disease activity. Subtraction of registered images is a reliable, time efficient method to assess disease progression in MS.

Adult↗

PubMed2002

Serial isotropic three-dimensional fast FLAIR imaging: using image registration and subtraction to reveal active multiple sclerosis lesions.

OBJECTIVE: Image registration and subtraction to detect the change of disease burden in multiple sclerosis on serial MR images should benefit from the use of high-resolution isotropic voxels. We compared 1.2-mm isotropic three-dimensional (3D) fast fluid-attenuated inversion recovery (FLAIR) images with standard 3-mm two-dimensional spin-echo images for the detection of new or enlarging lesions in longitudinal studies. SUBJECTS AND METHODS: Serial MR images were obtained at baseline, month 6 (n = 20), and month 7 (n = 16). For the half-yearly intervals, subtracted 3D FLAIR images and T2-weighted spin-echo images were compared. For the monthly intervals, subtracted 3D FLAIR images were compared with triple-dose contrast-enhanced T1-weighted spin-echo images. New, enlarging, and enhancing lesions were marked in consensus by two radiologists. RESULTS: At the half-yearly intervals, 3D FLAIR imaging detected more new or enlarging lesions than T2-weighted spin-echo imaging, both at the initial interpretation (80 vs 52; p < 0.001) and after a side-by-side comparison of the lesions (88 vs 65; p < 0.001). Post hoc analyses showed the largest benefit for new (rather than enlarging), for small, and for temporal lesions. At the monthly intervals, 32 enhancing lesions were detected on contrast-enhanced T1-weighted spin-echo images versus 20 new or enlarging lesions detected on 3D FLAIR images (p < 0.05). After a side-by-side comparison of the lesions, seven additional lesions were identified on 3D FLAIR images, making the difference with contrast-enhanced T1-weighted spin-echo images insignificant (27 vs 32; p > 0.05). CONCLUSION: Isotropic 3D FLAIR imaging holds great promise for the detection of new or enlarging lesions in multiple sclerosis using registration and subtraction techniques certainly at longer intervals.

Adult↗

PubMed2002

New syndrome characterized by hypomyelination with atrophy of the basal ganglia and cerebellum.

BACKGROUND AND PURPOSE: Leukoencephalopathies of unknown origin constitute a considerable problem in child neurology. The purpose of our ongoing study of the subject was to define new disease entities among them by using primarily MR imaging pattern recognition. METHODS: We identified seven unrelated patients with a distinct MR imaging pattern consisting of hypomyelination and atrophy of the basal ganglia (neostriatum) and cerebellum (H-ABC). We reviewed the clinical, MR imaging, MR spectroscopic, and laboratory data. RESULTS: Clinically, the patients' diseases were characterized by variably disturbed early development followed by increasing extrapyramidal movement abnormalities, ataxia, and spasticity. Mental capacity was variably affected, but it appeared to be relatively preserved. Parents were not related, and none of their siblings were affected. No metabolic defect was found. Follow-up MR imaging demonstrated atrophy of the cerebral white matter, neostriatum, and cerebellum, which was most pronounced in the most clinically severe cases. Single-voxel proton MR spectroscopic results were normal in the parietal cortex. In the cerebral white matter, myo-inositol and creatine levels were elevated; this finding was compatible with gliosis. N-acetylaspartate and choline levels were normal, suggesting that neither axonal loss nor active demyelination occurred. Proton MR spectroscopic imaging revealed relatively decreased N-acetylaspartate levels in the frontal region. CONCLUSION: The uniform and highly characteristic MR imaging findings, in combination with the similarities in the clinical findings, provide evidence of a distinct nosologic entity. The acronym H-ABC is offered to indicate patients sharing these clinical and MR imaging features.

Atrophy↗