File:U fibres big.JPG

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Summary

[edit]
Description Desmyelinating Disorder
Date
Source Radiology picture of the day
Author Dr. Laughlin Dawes
Permission
(Reusing this file)

Explanation

[edit]
English: In Dr. Laughlin's site you can read this explanation of the picture (also under Creative Commons).
"This 18-month old female presented with developmental delay. By this age myelination should be essentially

complete, yet there is residual T2 high signal in the subcortical white matter due to disruption of normal myelin formation. The differential diagnosis is depends on the head size.

With megalencephaly the possibilities are:

Without megalencephaly: - in utero infection including Toxoplasma, Rubella, cytomegalovirus and herpes simplex virus. - mitochondrial cytopathy - Pelizeus-Merzbacher disease (but patient is female and PMD is X-linked recessive) - 18q deletion syndrome (the karyotype was normal) - Tuberous sclerosis (there were no other stigmata)

The more common leukodystrophies, metachromatic leukodystrophy and adrenoleukodystrophy, typically spare the subcortical U-fibres.

Credit: Dr Laughlin Dawes"
Español: En la web del Dr. Laughlin se puede leer la siguiente explicación de la imagen (La explicación también está bajo licencia Creative Commons).

Esta niña de 18 meses presenta retraso en el desarrollo. A esta edad la mielinización debería, en esencia, estar completa, aunque aún hay una alta señal de T2 en la materia blanca subcortical debido a la disrupción de la formación normal de mielina. El diagnóstico diferencial depende del volumen cefálico:

Con macrocefalia las posibilidades son:

Sin macrocefalia:

  • Infecciones intrauterinas como toxoplasmosis, rubella, citomegalovirus y virus herpes simplex.
  • Citopatía mitocondrial
  • Enfermedad de Pelizeus-Merzbacher (Pero el paciente es niña y la enfermedad está ligada al X de forma recesiva)
  • Sindrome de deleción del cromosoma 18q (El cariotipo era normal)
  • Esclerosis tuberosa (no habia otras llagas)
Las leucodistrofias más comunes, la metacromática y la adrenoleucodistrofia, típicamente estan libres de fibras U subcorticales.

Autor Dr Laughlin Dawes

Licensing

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Date/TimeThumbnailDimensionsUserComment
current16:49, 10 March 2008Thumbnail for version as of 16:49, 10 March 20081,638 × 1,458 (185 KB)Gustavocarra (talk | contribs){{Information |Description=Desmyelinating Disorder |Source=[http://www.radpod.org/2006/11/19/dysmyelinating-disorder/ Radiology picture of the day] |Date=19/11/2006 |Author=Dr. Laughlin Dawes |Permission=Creative Commons |other_versions= }} ==Explanation

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