Thickening of the cauda equina roots: a common finding in Krabbe disease.
Objectives: Evaluation of Krabbe disease burden and eligibility for hematopoietic stem cell transplantation are often based on neuroimaging findings using the modified Loes scoring system, which encompasses central but not peripheral nervous system changes. We show that quantitative evaluation of th...
| Publicado en: | European Radiology Vol. 26; no. 10; pp. 3377 - 3383 |
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| Autores principales: | , , , , , , , |
| Formato: | research Journal Article |
| Publicado: |
Springer Nature
Oct2016
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=118060245&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 118060245 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 09387994 NPH jtl: European Radiology issn: 09387994 maglogo: N pubinfo: dt: Oct2016 vid: 26 iid: 10 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 118060245 118060245 NLM27137647 118060245 10.1007/s00330-016-4233-6 NLM27137647 118060245 ppf: 3377 ppct: 6 formats: fmt: @attributes: type: P tig: atl: Thickening of the cauda equina roots: a common finding in Krabbe disease. aug: au: Hwang, Misun Zuccoli, Giulio Panigrahy, Ashok Rodriguez, David Poe, Michele Escolar, Maria Poe, Michele D Escolar, Maria L affil: Department of Radiology of University of Pittsburgh Medical Center , 3950 Presby South Tower Pittsburgh 15213 USA sug: subj: Cauda Equina Pathology Leukodystrophy, Globoid Cell Pathology Male Leukodystrophy, Globoid Cell Infant Human Lumbar Vertebrae Cauda Equina Child, Preschool Magnetic Resonance Imaging Methods Retrospective Design Female Validation Studies Comparative Studies Evaluation Research Multicenter Studies Infant: 1-23 months Child, Preschool: 2-5 years Male Female ab: Objectives: Evaluation of Krabbe disease burden and eligibility for hematopoietic stem cell transplantation are often based on neuroimaging findings using the modified Loes scoring system, which encompasses central but not peripheral nervous system changes. We show that quantitative evaluation of thickened cauda equina nerve roots may improve the evaluation of Krabbe disease and therapeutic guidance.Methods: Lumbar spine MRI scans of patients obtained between March 2013 and September 2013 were retrospectively evaluated and compared to those of controls. Quantitative evaluation of cauda equina roots was performed on the axial plane obtained approximately 5 mm below the conus medullaris. The largest nerves in the right and left anterior quadrants of the spinal canal were acquired.Results: Fifteen symptomatic patients with Krabbe disease (5-44 months old) and eleven age-matched controls were evaluated. The average areas (mm(2)) of anterior right and left nerves were 1.40 and 1.23, respectively, for patients and 0.61 and 0.60 for controls (differences: 0.79 and 0.63; p < 0.001).Conclusions: Cauda equina nerve root thickening is associated with Krabbe disease in both treated and untreated patients. Adding lumbar spine MRI to the current neurodiagnostic protocols, which fails to account for peripheral nerve abnormalities, will likely facilitate the diagnosis of Krabbe disease.Key Points: • Neuroimaging is valuable for evaluating cauda equina nerve abnormality in Krabbe disease • MRI can be used to quantitatively evaluate cauda equina nerve thickening • Lumbar MRI could be useful for diagnosis and treatment monitoring of Krabbe disease. pubtype: Academic Journal doctype: research Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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