Computed tomography in Pelizaeus-Merzbacher disease

Statz, A; Boltshauser, Eugen; Schinzel, Albert; Spiess, H

doi:10.1007/bf00344782

Cited by 25 publications

(9 citation statements)

References 10 publications

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“…The MRI changes seen in our case are compatible but not specific: indeed, similar changes were seen in the two unclassified cases, whose clinical picture was totally different. There is evidence that CT is often normal during the first 15 years of life in Pelizaeus-Merzbacher disease (Niakan et al 1979, Statz et al 1981 and the MRI may be abnormal at an earlier age (Journel et al 1987), and thus provide earlier support for what is essentially a clinical diagnosis.…”

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

confidence: 99%

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

Miller¹,

Robb

Ormerod³

et al. 1990

Develop Med Child Neuro

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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...

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

confidence: 99%

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

Miller¹,

Robb

Ormerod³

et al. 1990

Develop Med Child Neuro

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“…In PMD, brain CT shows normal or mild atrophy or dilatation of ventricles [11]. Although disappearance of myelination from the entire central nervous system was reported in pathological studies [4], myelination of the peripheral nervous systems was preserved.…”

Section: Discussionmentioning

confidence: 92%

Three young adult patients with Pelizaeus–Merzbacher disease who showed only waves I and II in auditory brainstem responses but had good auditory perception

Kaga

Tamai²,

Kodama³

et al. 2005

Acta Oto-Laryngologica

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Three young adult males with Pelizaeus-Merzbacher disease have been followed up since childhood. This disease is thought to be a dysmyelinating disorder of the brain during the prenatal period caused by gene mutations. The patients manifested horizontal nystagmus and severe rigidity of the extremities. Although the patients showed only waves I and II in auditory brainstem responses, they had relatively good hearing ability at approximately equal to dB. They could not speak words at all but could hear well and enjoy listening to conversation and music. One of them had a normal hearing threshold in pure-tone audiometry and a normal speech discrimination rate in speech audiometry. This can be explained by a nerve conduction blockade through dysmyelinated axons or the desynchronization of neurons and nerves responsible for the waves following waves I and II. At present, all three patients are living with their families. We report their present hearing, speech and language abilities.

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“…At the terminal stage, the CT scan of case 2 ( Fig.2b) showed flattening of the heads of caudate nuclei, widening of the third ventricles and patchy low density area in the cerebral white matter, but, the low attenuation in Krabbe's disease was not nearly as obvious as in ALD, MLD, AD, or CD. The CT findings of only three previous cases of Krabbe's disease [4,6,9] have been reported. Two were almost the same as ours.…”

Section: Discussionmentioning

confidence: 99%

“…There are only three brief reports of CT in Krabbe's disease [4,6,9]. Ih order to provide some insight into this disorder, we report CT scanning of two patients with Krabbe's disease and discuss the relationship between radiodensity and the pathological process involving the cerebral white matter of leukodystrophies.…”

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Computed tomography in Krabbe's disease: Comparison with neuropathology

et al. 1983

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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.

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Computed tomography in Pelizaeus-Merzbacher disease

Cited by 25 publications

References 10 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

Three young adult patients with Pelizaeus–Merzbacher disease who showed only waves I and II in auditory brainstem responses but had good auditory perception

Computed tomography in Krabbe's disease: Comparison with neuropathology

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