Computed tomography in late‐infantile metachromatic leukodystrophy

Buonanno, Ferdinando S.; Ball, M. R.; Laster, Dan W; Moody, Dixon M.; McLean, William T.

doi:10.1002/ana.410040108

Cited by 18 publications

(4 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In metachromatic leucodystrophy there was abnormal signal, particularly affecting the periventricular and deep white-matter in all lobes and accompanied by some degree of ventricular enlargement. A similar distribution of low attenuation is seen with CT (Robertson et al 1977, Buonanno et al 1978, Heinz et al 1979.…”

Section: (Sezm/ao) From Five-year-old Boy With Subacute Sclerosing Pasupporting

confidence: 55%

Magnetic Resonance Imaging of Inflammatory and Demyelinating White‐matter Diseases of Childhood

Miller¹,

Robb

Ormerod³

et al. 1990

Develop Med Child Neuro

View full text Add to dashboard Cite

SUMMARY Magnetic resonance imaging (MRI) was performed on 36 children and two adults (with clinical presentation during childhood) with white‐matter disease of the central nervous system. Abnormalities were readily demonstrated in patients with multiple sclerosis, acute disseminated encephalomyelitis, leucodystrophies and subacute sclerosing panencephalitis: MRI demonstrated the extent and distribution of abnormalities more clearly than computed tomography for all these disorders. The abnormalities tended to be asymmetrical and multifocal in multiple sclerosis and acute disseminated encephalomyelitis, and more confluent and symmetrical in the leucodystrophies. Children with clinically isolated optic neuritis had a significantly lower frequency of MRI brain‐lesions than adults with the same disorder. MRI should be regarded as the radiological investigation of choice when white‐matter disease is suspected in children. RÉSUMÉ Imagerie de résonance magnétique des affections inflammatoires et démyélinisantes de la substance blanche de l'enfance Une imagerie de résonance magnétique (IRM) a été effectuée chez 36 enfants et deux adultes (avec manifestations cliniques durant l'enfance), ayant présenté une affection de la substance blanche du système nerveux central. L'existence d'anomalies a été facilement démontrée en cas de sclérose en plaques, d'encéphalite aiguë disséminée, de leucodystrophie et de panencéphalite sclérosante subaiguë: l'IRM révélait l'étendue et la distribution des anomalies plus clairment que le scanner dans toutes ces affections. Les anomalies tendaient à être assymétriques et multifocals dans la sclérose en plaques et l'encéphalomyélite aiguë disséminée, plus regroupées et symétriques dans les leucodystrophies. Les enfants porteurs d'une névrite optique isolée avaient une fréquence de lésions cérébrales à l'IRM plus faible que les adultes atteints du même trouble. L'IRM doit être considérée comme l'exploration radiologique de choix lorsqu'une affection de la substance blanche est suspectée chez l'enfant. ZUSAMMENFASSUNG Kernspinntomographie bei entzüdlichen und demyelinisierenden Erkrankungen der weißen Substanz in Kindesalter Bei 36 Kindern und zwei Erwachsensen mit einer Erkrankung der weißen Substanz des Zentralnervensystems wurde eine Kernspinntomographie durchgeführt (MRI). Die beiden Erwachsenen hatten schon während des Kindesalters erste klinische Symptome gezeigt. Bei Patienten mit Multipler Sklerose, akuter Encephalomyelitis disseminata, Leukodystrophien und subakuter sklerosierender Panencephalitis wurden abnorme Befunde nachgewiesen: Im MRI wurden Aussdehnung und Verteilung der abnormen Befunde für all diese Erkrankungen klarer dargestellt als im Computertomogramm. Die abnormen Befunde waren bei der Multiplen Sklerose und der akuten Encephalitis disseminata eher asymmetrisch und multifokal und bei den Leukodystrophien mehr konfluierend und symmetrisch. Kinder mit einer klinisch isolierten Opticusneuritis hatten signifikant seltener Hirnläsionen im MRI als Erwachsene mit derselben Erkrank...

show abstract

Section: (Sezm/ao) From Five-year-old Boy With Subacute Sclerosing Pasupporting

confidence: 55%

Magnetic Resonance Imaging of Inflammatory and Demyelinating White‐matter Diseases of Childhood

Miller¹,

Robb

Ormerod³

et al. 1990

Develop Med Child Neuro

View full text Add to dashboard Cite

show abstract

“…Bilateral, symmetrical, decreased attenuation of the cerebral white matter has been the usual finding in the late infantile form of MLD (2,3). Decreased attenuation of the cortical grey matter of the occipital lobe has been described (4).…”

Section: Discussionmentioning

confidence: 99%

“…Schilder's diseases, adrenoleucodystrophy and multiple sclerosis are characterised by asymmetric focal areas of decreased white matter attenuation with enhancement of the advancing edge of the lesion (5)(6)(7). The decrease in attenuation of the white matter on CT in demyelinating diseases, has been ascribed to loss of myelin (2). Increased water content, accumulation of simple lipids and alterations in protein and mineral content in the affected areas are some of the factors implicated in diseases other than MLD (5).…”

Section: Discussionmentioning

confidence: 99%

Computed tomography (CT) in late infantile metachromatic leucodystrophy

Jayakumar

Aroor

Jha

et al. 1989

Acta Neurologica Scandinavica

View full text Add to dashboard Cite

Computed tomographic features of 7 cases of the late infantile form of MLD confirmed by sural nerve biopsy are presented. Diffuse symmetrical white matter, low attenuation of the cerebral parenchyma was the common feature. Hypodensity of the temporal lobes and the cerebellar hemispheres are 2 features which have not been reported earlier. Three patients had brainstem atrophy without evidence of cerebral atrophy. Awareness of the various CT features of MLD may help in more definitive radiological diagnosis of the disease and to differentiate it from other dysmyelinating diseases of the central nervous system.

show abstract

“…Computed tomography (CT) scanning often shows focal or diffuse low radiodensity in the cerebral white matter of leukodystrophies including metachromatic leukodystrophy (MLD) [1], adrenoleukodystrophy (ALD) [2][3][4], Pelizaeus Merzbacher disease (PMD) [3,5,6], Alexander's disease (AD) [7,8], and Canavan's disease (CD) [5,7].…”

mentioning

confidence: 99%

Computed tomography in Krabbe's disease: Comparison with neuropathology

et al. 1983

View full text Add to dashboard Cite

We report computed tomography (CT) appearance of two patients with Krabbe's disease. The most common findings included severe brain atrophy: an enlarged frontal extracerebral space, dilatation of ventricles, enlarged cisterns and enlarged cortical sulci. There was low attenuation in the corpus medullaris of the cerebellum, and symmetrical focal hypodensity in the central periventricular white matter. CT at the terminal stage (16 months) showed marked cerebral atrophy including flattening of the heads of caudate nuclei and widening of the third ventricles confirmed by a neuropathological study. There was relatively less low density on the white matter of Krabbe's disease compared with that of other leukodystrophies. These CT findings may be useful in the diagnosis. The relative lack of low density in the white matter of Krabbe's disease might be related to severe gliosis and reduced total lipid contents.

show abstract

Computed tomography in late‐infantile metachromatic leukodystrophy

Cited by 18 publications

References 12 publications

Magnetic Resonance Imaging of Inflammatory and Demyelinating White‐matter Diseases of Childhood

Magnetic Resonance Imaging of Inflammatory and Demyelinating White‐matter Diseases of Childhood

Computed tomography (CT) in late infantile metachromatic leucodystrophy

Computed tomography in Krabbe's disease: Comparison with neuropathology

Contact Info

Product

Resources

About